Rheumatology

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Rheumatology

• Debbie King FNP
• Nursing 8800

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Scleroderma

• Derived from the Greek words "sklerosis," meaning
  hardness, and "derma," meaning skin, scleroderma
  literally means hard skin. Though it is often
  referred to as if it were a single disease,
  scleroderma is really a symptom of a group of
  diseases that involve the abnormal growth of
  connective tissue, which supports the skin and
  internal organs. It is sometimes used, therefore,
  as an umbrella term for these disorders. In some
  forms of scleroderma, hard, tight skin is the
  extent of this abnormal process. In other forms,
  however, the problem goes much deeper, affecting
  blood vessels and internal organs, such as the
  heart, lungs, and kidneys.
• Scleroderma is called both a rheumatic
  (roo-MA-tik) disease and a connective tissue
  disease. The term rheumatic disease refers to a
  group of conditions characterized by inflammation
  and/or pain in the muscles, joints, or fibrous
  tissue. A connective tissue disease is one that
  affects tissues such as skin, tendons, and
  cartilage.

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Patho

• Damage to cells lining the wall of small arteries
• Build up of tough scar like fibrous tissue
• Overproduction of collagen in connective tissue
• Can prove degenerative and life threatening

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Patho

• FYI
• Scleroderma is NOT
• Contagious
• Infectious
• Cancerous
• Malignant
• And probably not genetic
• Research indicates that there is a susceptibility
  gene which raises the likelihood of getting
  scleroderma, but by itself does not cause the
  disease

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Types of Scleroderma

• Localized
• Morphia
• Linear
• Systemic
• Limited (CREST)
• Diffuse

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Impact

• How serious
• Varies
• Depends on affected area
• Depends on extent of affection
• A mild case can become more serious if not
  diagnosed and properly treated by a qualified
  provider!!!
• Prompt treatment may minimize the symptoms and
  lesson the chance of irreversible damage!!!

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Course of disease

• Highly individualized disease
• Never the same presentation
• Is life long chronic disease
• First few years are rocky
• Will quiet down after first few years
• May have periods of remission

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Limited (CREST)

• Systemic
• Effects wide spread
• Usually does not affect internal organs
• Has a positive outlook
• CREST- term not used much anymore, referred to as
  limited cutaneous systemic sclerosis
• C-calcinosis, R-Raynaud's, E-Esophageal
  dysfunction, S-Sclerodactyly, T-Telangiectasia

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Diffuse Scleroderma

• Systemic
• Affects skin, connective tissue, organs
• May be slow or rapid
• May overlap with other disease
• e.g.. Mixed connective tissue disease

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Signs and Symptoms

• Skin
• Pronounced puffiness (usually first)
• Tightening
• Discoloration
• Thickening
• Numbness
• Tingling
• Telangiectasis
• Calcinosis
• Loss of hair
• Decreased sweat glands

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Vascular Changes

• Hands or feet change color- blanching
• Reddish spots-- telangiectasis
• Ulcers on fingertips, knuckles, elbows
• Visible capillaries around nails

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Bone and Muscle Changes

• Arthritis
• Brittle bones
• Bone loss
• Decreased mobility of fingers
• Muscles near shoulders and hips weaken
• General weakness

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GI Changes and Effects

• 80 of patients with Scleroderma are affected in
  this body system
• Decreased esophageal mobility
• Increased heartburn
• Difficulty swallowing
• Constipation
• Mal-absorption
• Watery diarrhea

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Respiratory Changes

• This system can be the most severely affected
• Dry cough
• SOB
• Difficulty taking deep breaths
• SEEN ANY PATIENTS LIKE THIS???

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Other General Signs and Complications

• Weight loss
• Fatigue
• Pain
• Unexplained swelling
• Internal organ problems
• Sjögren's syndrome
• chronic autoimmune disease in which peoples
  white blood cells attack their moisture-producing
  glands

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Other General Signs and Complications

• Auto-immune disorder of liver
• Thyroid dysfunction
• Nerve damage
• Impotence- first sign for many men
• Brain changes in late disease

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Causes

• - most commonly accepted theory
• Unknown inciting event triggers injury probably
  to cells lining the blood vessels. Autoimmune
  reaction leads to stimulation of fibroblasts
  (cells that make collagen). Net result is
  accumulation of collagen and other connective
  tissue components in parts of the body such as
  skin, lungs ECT

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Epidemiology

• Who gets scleroderma
• 300,000 cases in US, 80-100,000 are systemic form
• overall incidence rate of SSc in the adult
  population of the United States is approximately
  20 per million per year
• estimated the prevalence of systemic sclerosis in
  the United States to be around 240 cases per 1
  million adults
• Develops between ages 25-55 (or any age)
• Women 3-4 times more than men
• Prognosis worse in men
• Regional outbreaks
• Incidence unknown around the world
• European decent more common
• Increasing numbers in African Americans
• Not directly inherited, some scientist feel a
  slight predisposition in families with history of
  rheumatoid disease

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General Outlook

• No cure
• There are current treatments to minimize
  irreversible damage
• Does not always shorten life span with proper
  diagnosis and treatments!
• Localized- good prognosis with normal life span
• Diffuse- can be acute or rapid, often affects
  organs
• Cost to US alone was over 1.5 billion a year in
  1998 (sorry no current number was found)

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Head and Neck Manifestations

• 80 have, 30 present with
• tight skin, thin lips, vertical perioral furrows
• dermal and subcutaneous inflammatory process
• edema precedes epidermal atrophy, loss of
  appendages

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• Head and Neck Manifestations
• decreased mouth opening
• initial complaint 19

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General Diagnosing

• History-including symptoms
• PE including skin, nails, lungs, tendons/joints
• Labs
• ANA (Those with scleroderma typically have a
  speckled, nucleolar, or centromere pattern test
  result.)
• Anti-scleroderma 70 antibodies(is positive in up
  to 60)
• FANA-an auto antibody(positive in most all
  scleroderma)
• anti-centromere antibody (Present in 60-80 of
  those with limited cutaneous scleroderma and
  strongly associated with CREST)

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General Diagnosing

• Non-Laboratory Tests
• Lung function tests may be performed when lung
  involvement is suspected.
• CT (Computed Tomography) scans may be performed
  to evaluate lung damage.
• X-rays may be done to detect calcium deposits and
  evaluate lungs.
• Cardiac testing and monitoring may be performed
  when heart involvement is suspected
• Nail fold capillary microscope exam
• Skin biopsy or Kidney biopsy
• Many will first be diagnosed with mixed
  connective tissue disease
• Refer to Rheumatologist and Dermatologist

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More Labs

• Special Stains and Immunohistochemistry
• Direct immunofluorescence
• usually negative29,30
• Immunoperoxidase techniques
• reduced number of CD34-positive cells31
• Other investigations
• Serum levels of various enzymes and substrates
  involved in sclerotic process3237
• have been used as indicator of disease activity
• Antibodies
• Anti-cytoplasmic antigen Ro/SSA 35 of cases45
• Antinuclear almost all cases
• usually speckled or nucleolar pattern40,4648
• Autoantibodies to Fc receptor
• 50 of cases49
• Antineutrophil cytoplasmic of perinuclear type
  (p-ANCA)
• Small number of cases

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GI Complications

• Most common is GERD-sched frequent scopes!
• Difficulty swallowing
• Scarring in lower intestine
• Perforation of bowel
• Constipation
• Diarrhea
• Mal-absorption
• Fecal incontinence

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GI Complications

• Diverticulitis
• Watermelon stomach-dilated blood vessels lining
  the walls of the stomach which may bleed
• Small intestine bacterial overgrowth syndrome
• Gastroparesis (the retention of food in the
  stomach)

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GI Treatments

• General GERD Treatments
• Decrease carbonated beverages
• Decrease caffeine
• Avoid regular and decaffeinated coffee
• Walk regularly
• Remain upright after meals
• Avoid bedtime snacks
• Increase head of bed
• Chew gum
• No set dietary restrictions except above

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GI Treatments

• GERD
• Antacids
• H-2 Blockers
• Proton Pump Inhibitors
• Carafate
• Dilated strictures in esophagus
• Dilatation

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GI Treatments

• Swallowing
• GI stimulants e.g. Reglan
• EES- may improve heartburn
• Sandostatin- IM, may improve motion of bowel
• Constipation
• Bulking agents
• Softening agents
• MiraLAX
• Zelnorm

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GI Treatments

• Small Intestine Dysfunction
• E.g.-Bacterial overgrowth-Diarrhea
• 2-3 weeks on then 1-2 weeks off, one of the
  following
• Tetracycline
• Ampicillin
• Flagyl
• Vanco
• Cipro
• Augmentin
• Zithromax
• Bactrim DS
• Some have such severe infections they need
  constant treatment, which leads to other possible
  problems

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Lung Complications(will occur in the first 5
years)

• Pulmonary fibrosis
• Pulmonary HTN
• Cor pulmonale
• Interstitial Lung disease- Scleroderma is the
  number one cause

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Lung Complications

• Frequently occurs in systemic, may be seen in
  limited, but will not be seen in localized
• Is worse in Men and African Americans
• Patients with positive Scl-70 antibody are at
  highest risk

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Lung Tests

• O2 Sats
• PFT
• High resolution CT
• Bronchoscope with lavage
• Biopsy
• Echo
• Cardio-pulmonary exercise test

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Respiratory Treatments

• First must ID alveolitis
• Then suppress with
• Steroids (low doses only-20mg)
• Immuno-suppressants
• Pneumovax
• Flu vax
• O2 as needed
• Rarely transplants
• Remember to treat GERD as well

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Respiratory Treatments

• Pulmonary Arterial HTN
• Refer to pulmonary HTN center
• Endothium receptor antagonist
• Prostaglandin derivatives
• CA Channel Blockers

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Raynaud's

• Seen in 95 of scleroderma patients
• Only 5 of population with Raynaud's have
  scleroderma
• Treatments
• CA Channel blockers
• Angiotensin II receptor blockers
• Other BP meds
• Vasodilators
• Keep warm, Live in warm climate
• Exercise and Relaxation
• Topical antibiotics-PRN

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Cardiac Complications

• Fibrosis
• Rhythm disturbance
• Congestive heart failure
• Pericarditis
• Pulmonary HTN

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Cardiac Treatments

• Same as with other causes for each problem
• Refer for all cardiac complications

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Kidney Complications

• Increased protein (if seen-do full renal workup)
• HTN (monitor BP very often)
• Renal crisis- may be treated with Ace inhibitors
  , Is now used routinely in these patients
• Malignant HTN-Refer
• May be fatal
• May lead to long term dialysis
• Once treated, rarely reoccurs, if treated early!!

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Kidney Complications

• WARNING Corticosteroids may lead to renal
  crisis. Prednisone or Medrol are used to treat
  several complications such as
• Pulmonary Fibrosis
• Pruritus
• Joint Pain
• New findings indicate corticosteroids can be
  implicated in precipitating renal crisis
• If used-monitor closely

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Kidney Complications

• Seen only in diffuse scleroderma
• Was leading cause of death until 25 years ago for
  these patients
• First see increase in BUN and then serum
  creatinine (which suggest chronicity), and
  increase protein in urine
• Seen in patients with rapidly progression skin
  disease
• Those with positive anti-RNA polymerase
  antibodies appear to be a greater risk

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SKIN

• Skin fibrosis
• Refer to Derm
• Treat with immuno-suppressants, but all have
  major side effects
• Pruritus and Dryness
• OTC lotions
• Antihistamines

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SKIN

• Localized Scleroderma and Systemic
• Plaquenil
• Methotrexate
• Prednisone
• Phenytoin
• Potassium P-aminobenzoate
• D-Penicillamine
• PUVA

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Anemia

• Related to
• GI bleed
• Fe deficiency
• Chronic disease
• Hemolytic
• Often treated with Procrit-epoetin

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Oral, Facial Complications

• Dry mouth
• Increased dental caries
• Widening of periodontal membranes
• Facial changes are a real possibility for
  patients with both types of Scleroderma
• 100 with systemic
• 20 with localized

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Oral, Facial Complications

• These changes are progressive and not static
• Most functional problems are due to microstomia
  (small mouth)
• Eating
• Talking
• Kissing
• ECT

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Face Skin Changes

• Occur over several months to years
• First is edematous which is painless
• Second is indurative which is thickened dermis
  and thinned epidermal layer which leads to loss
  of wrinkles, hair follicles, sweat glands, and
  sebaceous glands.
• Third is atopic-thickened dermis becomes thinner
  and tethering occurs with skin more attached to
  sub Q fat, then clusters of dilated and tortuous
  capillaries increase in a butterfly shape

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Joint and Tendon Pain

• Occurs with all systemic cases and can progress
• Treat as arthritis
• Stay active
• Avoid fatigue
• Avoid cold
• PT RRN
• MEDS
• NSAIDS, Cox-2 Inhibitor, Analgesics, Narcotics

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Complication of Reactive Depression

• Is not a surprising complication
• Treatment is the same
• SSRI
• Tricyclic Antidepressants
• ECT

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Other General Treatments

• Relaxin to help inhibit production of collagen
  and lessen existing excess collagen. Helpful with
  skin, lungs, hand flexing, and opening the mouth
• Iloprost-vasodilator, is a analogue of
  prostaglandin. It also blocks certain cytokines,
  immune factors that appear to play an important
  role in the development of scleroderma. Best
  given IV
• An ACE may save the kidneys, has even been known
  to bring back kidney function
• Many investigational drugs
• Stem cell and bone marrow transplants-used in
  early disease only, with limited benefits.

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Referrals

• Rheumatologist
• Dermatologist- often make the diagnosis
• Primary Care- Is you!
• Will also see everyone else
• GI
• Pulmonology
• Cardiology
• Nephrology
• ECT

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Significance to Practice

• Education of providers is necessary
• Prevention of complications
• Remember prompt and proper diagnosis and
  treatment by a qualified provider may minimize
  the symptoms of scleroderma and decrease the
  chance of irreversible damage.

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Summaryhttp//www.niams.nih.gov/Health_Info/Scler
oderma/default.asp

• Greatly misdiagnosed
• Greatly under diagnosed
• Early treatment slows progression
• Early treatment slows complication and improves
  quality of life
• Further research may change the prognoses of this
  disease

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• http//www.clinicaladvisor.com/an-infrequent-cause
  -of-chronic-cough/article/162701/

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Sjögren's Syndrome

• May or not be associated scleroderma
• General
• immune mediated
• destruction of exocrine glands
• primary
• sicca syndrome- isolated d/o lacrimal and
  salivary glands
• secondary
• sicca complex- assoc. with other CTD

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Sjögren's Syndrome

• General
• prevalence
• 1 population
• 10-15 of RA patients
• 9 to 1 FgtM
• onset 40-60 yrs.
• increased risk of lymphoma
• perhaps 44 times risk

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Sjögren's Syndrome

• General
• clinical manifestations
• Xerophthalmia (dry eye)
• Keratoconjunctivitis sicca is characterized by
  inadequate tear film protection of the cornea
  because of either inadequate tear production or
  abnormal tear film constitution, which results in
  excessively fast evaporation or premature
  destruction of the tear film
• Xerostomia (dry mouth)
• other areas
• skin, vagina, genitalia, chronic bronchitis, GI
  tract, renal tubules
• diagnosis
• minor salivary gland biopsy
• labs
• RF and ANA
• SS-A/Ro 60
• SS-B/La 30

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Sjögren's Syndrome

• Head and Neck Manifestations
• 80 c/o xerostomia (dry mouth), most prominent
  symptom
• difficulty chewing, dysphagia, taste changes,
  fissures of tongue and lips, increased dental
  caries, oral candidiasis

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Sjögren's Syndrome

• Head and Neck Manifestations
• salivary gland enlargement
• xerostomia may be quantified by salivarv flow
  rate testing

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Sjögren's Syndrome

• Dry Mouth
• Treatment
• Pilocarpine
• is a drug which can stimulate salivary glands to
  make more saliva.
• clotrimazole/nystatin
• close dental supervision
• surveillance for malignancy

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Sjögren's Syndrome

• eye complaints
• dryness, burning, itching, foreign body sensation
• keratoconjunctivitis sicca
• corneal abrasions - rose bengal staining

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Sjögren's Syndrome

• Treatment
• symptomatic
• oral fluid intake
• saliva substitutes
• artificial tears
• avoid
• decongestants
• antihistamines
• diuretics
• anticholinergic

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Rheumatoid Arthritis

• A chronic, progressive, systemic inflammatory
  process that primarily affects the synovial
  joints.
• Joints are destroyed over a long course of
  disease
• Deformities are common
• Leads to emotional and physical trauma

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Rheumatoid Arthritis

• Patho
• Destroys the joints in two ways
• Proteolytic enzymes called proteases digest the
  tissue components of affected joints
• Due to locally released antigens that cause the
  inflammatory cascade
• They may be autoimmune substances
• Invasive rheumatoid pannus destroy joints
• A creation of inflamed synovium.
• Consists of granulated vascular tissue extending
  from the vascular bed into the joint

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Rheumatoid Arthritis

• Patho continued
• Also with in the synovial fluid, an inflammatory
  response occurs. Neutrophils infiltrate, and
  secrete a host of proteolytic enzymes in the
  fluid, as well as cytokines and chemotactic
  factors. This accumulation of joint fluid
  distends the joint capsule which leads to pain
• Aspiration may lead to instant relief

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Rheumatoid Arthritis

• Differential
• Connective tissue diseases are the differential
• Osteoarthritis, gout, chronic lyme disease,
  Lupus, parvovirus, polymyalgia rheumatica,
  Sjögren's, sarcoidosis
• Osteo usually does not affect wrists, fingers
• Gout is revealed with synovial aspirate of urate
  crystals
• Chronic lyme often involves a singular joint and
  have positive serologic markers
• Lupus arthritic changes are usually not deforming
• Parvo rash is different than RA
• Polymyalgia rheumatica are RF negative

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Rheumatoid Arthritis

• Subjective
• Early
• Malaise, diffuse arthritis, weight loss,
  anorexia, fever, neuropathic pain in extremities,
  painful eyes and chest pain with deep breaths
• Classic symptoms
• Awakens in pain that gets better as the day goes
  on
• Swelling present in the am and decreases
  throughout the day
• Pain and swelling go down with moderate activity
• Late
• Pain and swelling increase that lead to
  diminished activity and this leads to more pain
  and immobility

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Rheumatoid Arthritis

• Objective
• Peripheral symmetric polyarthritis and morning
  stiffness- history esp. if lasts more than an
  hour
• Physical exam
• Cardiac rub, pulmonary friction rub, injected
  sclera with scleritis, loss of sensation,
  decreased range of motion, ecchymotic lesions on
  extremities, rheumatoid nodules may be on elbows

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Rheumatoid Arthritis

• Tests
• Rheumatoid factor RF
• Which is an IgM that reacts against IgG
• Gives not only positive and negative results ,
  also gives insight into the severity of the
  disease
• 25 of people with RA have a negative RF
• ESR and/or CRP
• Will be elevated
• CBC
• To rule out anemia
• Look for a leukocytosis
• Platelet count
• More elevated with more joint inflammation

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Rheumatoid Arthritis

• More TESTS
• Joint-fluid analysis
• With RA will show increased WBC/ml and increased
  neutrophiles
• X-rays
• May be normal in early stages
• Later will reveal bone erosions- 90 of patients
  show erosion with in the first 2 years
• ANA
• to help rule out lupus, if positive with low
  titers may be RA, if high titers is lupus

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Rheumatoid Arthritis

• Management
• Processes form conservative to aggressive
• Goal is to reduce pain and inflammation and to
  spare joint function
• Early on this is possible with out medication
• Physical and occupation therapies, heat and cold,
  exercise, rest, assertive devices, splints,
  chiropractic adjustment, weight loss, meditation
• Later on medication is necessary
• Analgesics, NSAIDS, steroid, GI upset
  treatments, Disease Modifying Anti rheumatic
  drugs
• The CRP should be monitored to determine the
  effectiveness of therapy

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Rheumatoid Arthritis

• Medications
• Analgesics
• Tylenol, capsaicin cream, aspirin
• NSAIDs
• Enteric-coated asa, Sulindac 150 mg BID, Aleve,
  Motrin at 2400mg day
• May need an H-blocker or a proton pump inhibitors
• Zantac
• Prilosec or Nexium
• Need to monitor liver and kidney function
• Steroids
• 7.5mg qd
• Remember the side effects!!
• Adrenal insufficiency, hyperglycemia,
  osteoporosis, and skin discoloration
• Must also give calcium, vitamin D and
  bisphosphonates with prolonged use

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Rheumatoid Arthritis

• Disease-modifying antirheumatic drugs DMARDs -
  are essentially immunosuppressants and include
• Hydroxychloroquine (Plaquenil
• Leflunomide (Arava)
• Cyclosporine (Neoral)
• Sulfasalzine (Azulfidine)
• Gold (Ridaura, Solganal, Myochrysine)
• Methotrexate (Rheumatrex, Trexall)
• Azathioprine (Imuran)
• Cyclophosphamide (Cytoxan)
• Biologics (Actemra, Cimzia, Enbrel, Humira,
  Kineret, Orencia, Remicade, Rituxan, Simponi)
• Use this class with active disease
• Treat early with active disease
• Combo therapy is more effective than mono
• Side effects include reactivating TB

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Rheumatoid Arthritis

• Older therapies
• Methotrexate, Imuran, penicillamine, Azulfidine,
  Minocin
• Methotrexate is most effect, but not used in
  patients over 65, but the risks must not outweigh
  the benefits
• refer a patient for this drug
• Cox 2 Inhibitors
• Only one left in the US is Celebrex
• Was originally thought to be an amazing class of
  drugs, with no side effects. Until the cardiac
  problems became evident. Never promise
  patients- miracle drugs.

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Rheumatoid Arthritis

• Follow up and referral
• Routine clinical lab eval and episodic changes in
  interventions
• Routine lab every 90 days
• CBC, platelet count, liver and renal function,
  and fasting glucose
• Focus recheck visits on the effect of the relief
  measures
• Refer to rheumatologist
• refer everyone for twice a year visits

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Rheumatoid Arthritis

• Patient education
• Goals of TX
• Review the initial management section
• Address therapeutic and adverse effects of drug
• Explain this a chronic disease the cover the
  emotional, social, and spiritual sequelae of
  living with this condition
• Promote self care
• Educate patient and family to avoid the sick
  role
• Educate how the caregiver is so important in all
  areas of this condition

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Systemic Lupus Erythematosus

• An inflammatory autoimmune disease
• Affects many organ systems
• Spontaneous remissions and exacerbations
  characterize the clinical picture
• It can be mild, aggressive, or even
  life-threatening

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Systemic Lupus Erythematosus

• Epidemiology and cause
• 40 to 50 cases per 100,000
• Associated with age, gender, race and genetics
• 85 are women diagnosed in their 20s or 30s
• May present as late as 55 years
• Juvenile cases are not uncommon
• Descendants of African races are four times more
  likely to develop
• No cause has been identified, but some triggers
  for exacerbations have been
• Ultraviolet rays, infections, stress, pregnancy,
  surgery, exposure to cigarette smoke or silica
  dust

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Systemic Lupus Erythematosus

• Patho
• A condition of disordered immunity in which
  mechanisms that normally prevent immune cell
  activation by autoantigens are eliminated
• Inhibition of suppressor T cells
• Increase in CD4T helper cells
• Cytokine production and polyclonal B cell
  activation
• Deregulated intracellular signaling
• These factors contribute to a significant
  production of autoantibodies that are considered
  a hallmark of the disease

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Systemic Lupus Erythematosus

• Diagnosing
• must have four of the following
• arthritis, photosensitivity, oral ulcers, malar
  rash, discoid rash,
• serositis (inflammation of the pleura or
  pericardia), renal disease, hematologic
  disorders, neurologic disease, Positive ANA,
  immunologic abnormalities

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Systemic Lupus Erythematosus

• Subjective
• Complains of malaise, fever, anorexia, weight
  loss, blurred vision, conjunctive swelling,
  sleeplessness, depression. Joints may be
  reported as swollen/painful in the history.
  Shortness of breath and painful inspiration may
  be reported. Vague abdominal problems may be
  reported as well.

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Systemic Lupus Erythematosus

• Objective
• Skin
• Malar or Butterfly rash, discoid rash (scarring
  and highly inflammatory lesions), alopecia or
  scalpel exanthems, splinter hemorrhages, redness
  around finger nails, finger tip lesions,
  lymphadenopathy, Raynaud's
• The malar rash of lupus is red or purplish and
  mildly scaly. Characteristically, it has the
  shape of a butterfly and involves the bridge of
  the nose. Notably, the rash spares the
  naso-labial folds of the face, which contributes
  to its characteristic appearance. It is usually
  macular with sharp edges and not itchy. The rash
  can be transient or progressive with involvement
  of other parts of the facial skin.
• Discoid lupus lesions are often red, scaly, and
  thick. Usually they do not hurt or itch. Over
  time these lesions can produce scarring and skin
  discoloration (darkly colored and/or lightly
  colored areas). Discoid lesions that occur on the
  scalp may cause hair to fall out. If the lesions
  form scars when they heal, hair loss may be
  permanent.

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Systemic Lupus Erythematosus

• Musculoskeletal
• Swollen joints with no pattern, joint pain with
  palpation which is migratory and is the most
  common presenting complaint

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Systemic Lupus Erythematosus

• Objective
• Neurologic
• Impaired thought process, peripheral
  paresthesias, decreased deep tendon reflexes
• Cardiac
• A systolic murmur, distended jugular veins (with
  CHF)
• GI
• Painless or not --oral and nasal lesions,
  subglottic stenosis, right upper quadrant
  tenderness (hepatitis is common), light lower
  quadrant tenderness (right colon enlargement due
  to intestinal vasculitis)

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Systemic Lupus Erythematosus

• Differential Diagnosis
• Vasculitis, rheumatoid arthritis, scleroderma,
  juvenile RA, chronic active hepatitis, drug
  reactions, drug-induced lupus, polyarteritis.
• Rule thyroid disease, which could also have been
  caused by the lupus

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Systemic Lupus Erythematosus

• Diagnostic Testing
• CBC include platelets
• CMP-checking electrolytes and renal function
• Serum albumin reduced with nephropathy
• ANA
• UA
• Antibody screens for double stranded DNA
• Antiphospholipid antibodies
• Anti-Smith antibodies
• Single-stranded DNA and nucleoprotein will be
  positive for both Lupus and RA
• CPK

94
Labs from the Lupus website

• AlbuminANAANCA (antineutrophil cytoplasmic
  antibodies), P-ANCA (perinuclear) C-ANCA
  (cytoplasmic)Anti-Cardiolipin (Anti-Phospholipid)
  Anti-DNAAnti-Ro/SS-AAnti-Sm antibodiesBlood
  Urea Nitrogen (BUN)CBC Complement
  studiesCholesterolCPKCreatinineCreatinine
  ClearanceDifferential Neutrophils, Lymphocytes,
  Monocytes, Eosinophils, BasophilsENA
  (Extractable Nuclear Antigens)Erythrocyte
  Sedimentation Rate (Sed Rate, ESR)Immunoglobulins
  (IG)IronRheumatoid FactorWhite Blood Count
  (WBC, Leukocyte count)

95
LUPUS

• Results of labs
• Anemia or leukopenia or both
• 90 have an elevated ANA
• Rising anti-double stranded DNA
• Rising complement levels
• Decreasing CH50, C3. C4
• Elevated ESR and CRP
• Proteinuria
• RPR can be falsely positive
• CPK elevated with muscle involvement

96
LUPUS

• Other tests
• Chest x-ray
• Renal US
• Plain films of joints
• Renal biopsy
• EEG, echo
• Ventilation perfusion scans
• CT of lungs

97
LUPUS

• Management
• Goal of therapy is symptom control and organ
  protection
• Many require very little interventions
• Joint pain management
• Corticosteroids
• Cyclophosphamide
• Antimalarials
• Care for fatigue
• Kidney protection!
• GERD treatments
• Pulmonary treatments

98
Treatments from the Lupus website

• NSAIDsAntimalarialsCorticosteroidsCytotoxics/Im
  munosuppressantsCellCept (MMF)
• New treatments on the horizonStem Cell
  TransplantsRiquentPrasteroneMinocycline -
  friend or foe?

99
LUPUS

• Follow up and referral
• See at least 2 times a year if not on meds
• Check CBC, UA, platelet count, and ANA
• Depends on how much medical support is required
• Referral to a Rheumatologist is helpful

100
LUPUS

• Education
• Give national organization info
• Instruct on the course of the disease
• Advise a lot of REST
• Fluids
• NSAIDS
• Call with a fever over 101.5
• Avoid sulfonamide antibiotics
• Avoid pregnancy until remission for 6 months
• Prognoses varies with each patient

101
Giant cell arteritis

• http//emedicine.medscape.com/article/1201429-over
  view
• More common in Caucasians than African Americans
• More common in women
• Rare under the age of 50
• 5-27 cases per 100,000 people over 50 in the US
• is also called temporal arteritis, cranial
  arteritis, and granulomatous arteritis.
• GCA is a systemic inflammatory vasculitis of
  unknown etiology that affects medium- and
  large-sized arteries.

102
Giant cell arteritis

• Can result in a wide variety of systemic,
  neurologic, and ophthalmologic complications.
• Visual loss is one of the most significant causes
  of morbidity
• Newly recognized GCA should be considered a true
  neuro-ophthalmic emergency.
• Prompt treatment with steroids can prevent
  blindness and other vascular sequelae of GCA.15

103
Giant cell arteritis

• Criteria for diagnosis
• Age at onset of 50 years
• New headache
• Abnormalities of the temporal arteries
• Erythrocyte sedimentation rate (ESR) of 50 mm/h
• Positive results of a temporal artery biopsy
  (vasculitis characterized by a predominance of
  mononuclear infiltrates or granulomas, usually
  with multinucleated giant cells)

104
Giant cell arteritis

• Pathophysiology
• GCA typically involves inflammation of the aortic
  arch and its branches, but almost any artery of
  the body as well as some veins may be affected
  occasionally. The inflammation tends to involve
  the arteries in a segmental or patchy manner,
  although long portions of arteries may be
  involved

105
Polymyalgia rheumatica

• Polymyalgia rheumatica (PMR) is a clinical
  syndrome characterized by severe aching and
  stiffness in the neck, shoulder girdle, and
  pelvic girdle. It is classified as a rheumatic
  disease, although the etiology is undetermined.

106
Polymyalgia rheumatica

• Pathophysiology
• Polymyalgia rheumatica causes severe pain in the
  proximal muscle groups however, no evidence of
  disease is present at muscle biopsy. Muscle
  strength and electromyographic findings are
  normal. Some evidence suggests the presence of
  cell-mediated injury to the elastic lamina in the
  blood vessels in the affected muscle groups.
• Polymyalgia rheumatica is closely linked to giant
  cell arteritis (temporal arteritis), but this is
  believed to be a separate disease process.1

107
Polymyalgia rheumatica

• Clinical History
• The patient's history may include the following
  features
• Pain and stiffness in the proximal muscle groups
  that usually is symmetrical and worse in the
  morning
• Gel phenomenon (stiffness after prolonged
  inactivity)
• Fever (low grade)
• Weight loss
• Fatigue
• Depression
• No weakness
• Abrupt onset of symptoms

108
Polymyalgia rheumatica

• Frequency in the US
• One study revealed a prevalence of 1 in 200
  people aged 50 years or older.
• Mortality/Morbidity
• Polymyalgia rheumatica is not a life-threatening
  disease, but it does require treatment for 2-4
  years.
• Race
• Whites are affected more than other ethnic
  groups.
• Sex
• Females are affected twice as often as males.
• Age
• Polymyalgia rheumatica usually affects people
  older than 50 years.

109
Polymyalgia rheumatica

• Physical
• The signs and symptoms of polymyalgia rheumatica
  are nonspecific, and objective findings on
  physical examination often are lacking. If
  present, findings may include the following
• Muscle tenderness
• Decreased active range of motion of joints
  secondary to pain
• No muscle atrophy

110
Polymyalgia rheumatica

• The following laboratory studies should be
  obtained for suspected polymyalgia rheumatica
• Erythrocyte sedimentation rate greater than 50
  mm/h
• Normochromic normocytic anemia in 50 of cases
• Normal creatinine kinase level
• Negative finding for rheumatoid factor
• Mild elevations with liver function test results
• Mild nonspecific synovitis
• Negative muscle biopsy findings

111
Polymyalgia rheumatica

• Imagining
• The cause of musculoskeletal symptoms in
  polymyalgia rheumatica (PMR) is not clearly
  defined because joint synovitis may only
  partially explain the diffuse discomfort. MRI
  imaging of the shoulders, hip and extremities of
  patients with PMR has been analyzed. MRI showed
  that subacromial and subdeltoid bursitis of the
  shoulders and iliopectineal bursitis and hip
  synovitis are the predominant and most frequently
  observed lesions in active PMR. The inflammation
  of the bursae associated with glenohumeral
  synovitis, bicipital tenosynovitis and hip
  synovitis may explain the diffuse discomfort and
  morning stiffness.

112
Polymyalgia rheumatica

• The goal of therapy is to suppress autoimmune
  activity.
• Corticosteroids
• These agents have anti-inflammatory properties
  and may cause profound and varied metabolic
  effects. They modify the body's immune response
  to diverse stimuli. Based on a systematic
  examination of the peer-reviewed literature,
  which included 30 studies, remission of
  polymyalgia rheumatica seemed to be achieved with
  a 15 mg/d dose of prednisone for most patients. A
  slow tapering of the prednisone, less than 1
  mg/month, was associated with fewer relapses
• Methotrexate, azathioprine, and other
  immunosuppressive drugs have been used in some
  centers in an effort to limit dosage and duration
  of corticosteroid therapy
• NSAIDS esp helpful during steroid taper
• Ensure adequate calcium and vitamin D intake with
  corticosteroid use
• PT (activity restriction is unnecessary)

113
GOUT

• Epidemiology
• Estimated prevalence 1.4-2 Male-to-female
  ratio approximately 41 Age of onset typically
  40-60 years in men, seven years later (on
  average) in women rare after menopause

114
GOUT

• Causes and risk factors
• Urate underexcretion (more common than
  overproduction) may be primary or associated with
  such conditions as renal impairment,
  hypertension, hypothyroidism, or various
  medications (e.g., aspirin). Urate
  overproduction may be primary or associated with
       Dietary factors excessive intake of
  purines, fructose, or alcohol     
  Lymphoma/myeloproliferative disorders, psoriasis,
  high cell-turnover disorders      Medications
  cytotoxic agents or vitamin B12     
  Hypoxanthine-guanine phosphoribo- syltransferase
  deficiency (Lesch-Nyhan syndrome)     
  Metabolic conditions obesity or
  hypertriglyceridemia Hyperuricemia most
  important risk factor      Age at onset of
  first attack inversely related to serum uric acid
  level      Majority of patients with elevated
  serum uric acid levels, however, will not develop
  clinical gout.

115
GOUT

• Documented risk factors in males      Obesity,
  weight gain, and hypertension (metabolic factors)
       High intake of alcohol (especially beer),
  sugars, meats, and seafood      Diuretic use
  (may not be independent of other risk factors,
  such as hypertension and congestive heart
  failure)      High intake of dairy products
  and coffee (more than four cups daily) may be
  protective. History of organ transplantation
  (especially heart or kidney)/use of cyclosporine
  Risk factors for chronic tophaceous gout
  include diuretic use in renal insufficiency/conges
  tive heart failure (especially in women) and long
  history of disease or alcohol abuse.

116
GOUT

• Complications and associated conditions
• Progressive kidney failure Joint
  erosion/destruction (chronic tophaceous gout)
  Risk for development of type 2 diabetes,
  hypertriglyceridemia, and cardiovascular disease
  History
• Common triggers for acute event include     
  Infection, acidosis (metabolic)     
  Initiation of chemotherapy, starting/stopping
  allopurinol, diuretic therapy      Trauma,
  surgery, or use of IV contrast media     
  Alcohol use Acute attack typically
  self-limited, with spontaneous resolution in 3-14
  days      Sudden onset of extreme pain,
  tenderness, and joint inflammation      Often
  begins at night or early morning, with pain and
  other symptoms increasing for 24-48 hours     
  90 of initial attacks are monoarticular, most
  commonly the first metatarsophalangeal.
  Subsequent attacks are typically longer, involve
  more joints, and may not resolve without medical
  therapy. Recurrent attacks lead to
  accumulating crystal deposits in joints (chronic
  tophaceous gout), which become stiff and swollen

117
GOUT

• Physical exam
• Affected joint typically swollen, red, and
  extremely tender Skin may resemble cellulitis,
  with desquamation over inflamed area.
  Characteristics of tophi include     
  Overlying skin is pulled taut.     
  Visible/palpable soft-tissue masses or
  asymptomatic intradermal/subcutaneous nodules
       Typically contain whitish or yellowish
  deposits

118
GOUT

• Making the diagnosis
• Demonstration of urate crystals in synovial
  fluid analysis or in tophus by polarized light
  microscopy Presumptive diagnosis can be made
  based on presence of hyperuricemia, documentation
  of risk factors, and suggestive findings on
  history and physical exam. Rule out     
  Septic arthritis      Calcium pyrophosphate
  dihydrate deposition disease (pseudogout)     
  Rheumatoid/psoriatic arthritis      Erosive
  osteoarthritis or sarcoid arthropathy     
  Bacterial cellulitis

119
GOUT

• Testing
• Synovial fluid analysis/culture needed to
  exclude septic arthritis if patient is febrile
  and has leukocytosis Serum uric acid ( gt6.8
  mg/dL 404 µmol/L is sufficient for crystal
  precipitation level often normal during acute
  attack). X-ray may help confirm. 24-hour
  urine uric acid level as adjunctive test for
       Identifying/excluding overproducers of
  urate      Patients being considered for
  uricosuric therapy Blood culture to rule out
  infection

120
GOUT

• Prognosis
• Risk of recurrence after initial attack 60
  within one year and gt90 within 10 years
  Treatment of acute attack
• Oral nonsteroidal anti-inflammatory drugs
  (NSAIDs) at maximum dose for one to two weeks
  (drugs of choice for symptomatic relief)
  Colchicine effective but slower to work than
  NSAIDs Corticosteroids, especially in patients
  with contraindications to NSAIDs Rest, ice
  packs, joint elevation (Bed cage may keep
  bedclothes off inflamed joint.) Avoid alcohol
  ensure adequate fluid intake (gt2 L water daily).
  Allopurinol should not be started or
  discontinued during acute attack (avoid sudden
  changes in serum urate levels). Consider
  discontinuation of diuretics.

121
Foods with estrogenic effects

• An anti-estrogenic diet is simple and delicious.
  It has three main points Eat down on the food
  chain. Eat foods that are more in tune with our
  genetic makeup such as fruits, vegetables, beans,
  roots, nuts, seeds, eggs, dairy and wild-caught
  fish. Minimize foods treated with chemicals.
  Eat as much organic food as possible, and avoid
  synthetic vitamins. Supplement your diet with
  nutrients that support your hormones. The
  flavones chrysene, apigenin, quercetin, and
  narigenin all inhibit estrogen, as well as the
  indoles indole 3 carbinol, diindolymethane
  (DIM), and indole 3 acetate.
• Soy,Millet,Barley,Flax seed,Lentils,Kidney
  beans,Lima beans,Rye,Clover,Fennel,Chick peas are
  rich in phytoestrogens, a natural estrogen.

122
Foods with estrogenic effects

• Reduce and eliminate ALL beef, pork, lamb, and
  dairy products (they contain an unusable form of
  estrogen for the female body and create estrogen
  imbalance)
• Avoid all caffeine, alcohol, sugars, acid-forming
  foods (red meat, dairy, excesscarbohydrates,
  especially refined foods)