Sarcoidosis - PowerPoint PPT Presentation

1 / 30
About This Presentation
Title:

Sarcoidosis

Description:

Sarcoidosis T. Lianne Beck, MD Assistant Professor Emory Family & Preventive Medicine Objectives Epidemiology Pathogenesis Clinical presentation Organ systems ... – PowerPoint PPT presentation

Number of Views:172
Avg rating:3.0/5.0
Slides: 31
Provided by: Dept83
Learn more at: https://med.emory.edu
Category:

less

Transcript and Presenter's Notes

Title: Sarcoidosis


1
Sarcoidosis
  • T. Lianne Beck, MD
  • Assistant Professor
  • Emory Family Preventive Medicine

2
Objectives
  • Epidemiology
  • Pathogenesis
  • Clinical presentation
  • Organ systems involved
  • Diagnostic evaluation
  • Current evidence on treatment

3
Sarcoidosis
  • Multisystem disorder of unknown etiology that
    most commonly affects the lungs, but can also
    affect other organs.
  • Beethoven is thought to have been the first
    person described with this condition.

4
Epidemiology
  • 3rd or 4th decade of life.
  • More predominant in women with an incidence of
    6.3 vs 5.9 cases per 100,000 person-years.
  • Lifetime risk for US whites is 0.85 percent
    compared with 2.4 percent in US blacks.
  • More prevalent in Swedes, Danes, and US blacks.

5
Epidemiology
  • Annual incidence in the U.S. is 10/100,000 among
    whites and 36/100,000 among African Americans.
  • Most commonly seen in the mid-Atlantic and
    Southern Atlantic states but rare in the
    Southwest.
  • Affects siblings of first- or second- degree
    relatives in 15 of patients with sarcoidosis.
  • Familial cases described in 17 of African
    Americans, but only 6 of whites.

6
Etiology and Pathogenesis
  • Cause is unknown, although both genetic and
    environmental factors suspected.
  • Theory that disease develops in genetically
    predetermined hosts who are exposed to certain
    environmental agents that trigger an exaggerated
    inflammatory immune response leading to granuloma
    formation.

7
Etiology and Pathogenesis
  • Hallmark is noncaseating granulomas, composed of
    a central core of epithelioid histocytes and
    multinucleated giant cells.
  • Activated T cells and macrophages accumulate at
    site of inflammation.
  • Release chemoattractants and GFs lead to
    cellular proliferation and granuloma formation.
  • Progressive granulomatous inflammation leads to
    injury, dysfunction, and destruction of the
    affected organs.

8
Pathogenesis
T cells, Macrophages
Chemoattractants Growth Factors
Cellular proliferation Granuloma
Fibrosis
9
Clinical Presentation
  • 30-50 of patients are asymptomatic and are
    diagnosed on routine CXR.
  • One third have non-specific symptoms of fever,
    fatigue, weight loss and malaise.
  • A clinical variant of sarcoidosis, Lofgrens
    syndrome, includes constellation of erythema
    nodosum, polyarthritis, and BHL. Remission occurs
    in 80.

10
Clinical Presentation
  • Onset of sarcoidosis in white patients is usually
    asymptomatic.
  • African Americans tend to present with an earlier
    onset and a more aggressive and severe clinical
    course.
  • Chronic pulmonary sarcoidosis and the disfiguring
    cutaneous lesions of lupus pernio are also more
    common in African Americans.

11
Clinical Presentation
  • Spontaneous remission in two-thirds of patients
    within 2 years of presentation
  • 10-30 experience chronic disease causing
    progressive organ damage
  • Leads to death in 4 of patients, usually those
    with pulmonary, cardiac, or CNS involvement

12
Systems affected by Sarcoidosis
Signs and symptoms
Cardiac 30 Palpitations, syncope, dizziness, chest pain, arrhythmia, sudden death
Cutaneous 25 Erythema nodosum, lupus pernio, plaques, subcutaneous nodules, maculopapular eruption, alopecia, hyper/hypopigmentation
Endocrine Hypo/hyperthyroidism, adrenal insufficiency
Exocrine Painless swelling of parotid gland, keratocon-junctivis sicca
Hepatic 50-80 Asymptomatic or abdominal pain, abnormal LFTs, hepatomegaly
Lymphatic Extrapulmonary lymphadenopathy, splenomegaly
13
Erythema Nodosum
14
Lupus Pernio
15
Systems affected by Sarcoidosis
Signs and symptoms
Neurologic 5 Cranial nerve palsy, seizures, basal granulomatous meningitis, hypothalamic or pituitary lesions, hydrocephalus, peripheral neuropathy, psychiatric
Ocular 20 Uveitis, chorioretinitis, keratoconjunctivitis, glaucoma, cataracts, blindness, Heerfordt syndrome
Pulmonary 90 Asymptomatic or dyspnea, nonproductive cough, wheezing, radiographic findings from hilar adenopathy to fibrosis
Renal Hypercalcemia, hypercalciuria, renal insufficiency
16
Clinical Presentation
  • A progressive course is more likely in
  • Age of onset gt 40 yrs
  • Black race
  • Cardiac or renal involvement
  • Lupus pernio
  • Chronic uveitis
  • Hypercalcemia
  • Nasal mucosal involvement
  • Cystic bone lesions
  • Neurosarcoidosis
  • Pulmonary fibrosis

17
Clinical Presentation
  • Most patients have the pulmonary manifestations,
    most commonly presenting with incidental findings
    on CXR.
  • Interstitial disease
  • Symptoms include dry cough, dyspnea, and chest
    discomfort
  • Unpredictable course

18
4 Stages of Pulmonary Sarcoidosis
I Bilateral hilar lymphadenopathy and paratracheal adenopathy 55-90 remission
II Mediastinal adenopathy with pulmonary parenchymal involvements 40-70
III Pulmonary parenchymal without adenopathy 10-20
IV Pulmonary fibrosis with honeycombing 0-5
19
Stages
20
Approach to Suspected Sarcoid
  • History (occupational and environmental)
  • PE (lungs, skin, eyes, liver, and heart)
  • CXR, PFTs and EKG
  • CBC, CMP, ACE level
  • PPD
  • Biopsy for histological confirmation of
    noncaseating granulomas and culture and/or
    special staining to R/O fungal or TB
  • Ophthalmologic evaluation

21
Approach to Suspected Sarcoid
  • Follow-up
  • Monitor for resolution or progression of disease
    and for additional organ involvement.
  • Refer if there is evidence of disease progression
    or additional organ involvement.
  • Coordinate care.

22
Approach to Suspected Sarcoid
  • An aggressive work up may be unnecessary in
    asymptomatic patients with symmetric BHL,
    unremarkable exam, no history of malignancy, and
    normal results on routine bloodwork.
  • The course of disease usually becomes evident
    within 2 years of presentation. Absence of
    remission within this period predicts a chronic,
    persistent, or stable course.

23
Differential Diagnosis of BHL
  • Granulomatous infections
  • TB
  • Histoplasmosis
  • Coccidiomycosis
  • Autoimmune disorders
  • Malignancy (Lymphoma)

24
Differential Diagnosis of Noncaseating Granulomas
  • TB
  • Fungal infections
  • Lymphoma
  • Epithelioid tumors of the breast
  • Lung cancer

25
Treatment
  • Observation
  • Initiating corticosteroid therapy when
    appropriate
  • Monitoring response to therapy
  • Discontinuing corticosteroids when clinically or
    physiologically indicated.

26
Treatment
  • Topical therapy for cutaneous or ophthalmic
    disease.
  • Systemic corticosteroids for patients with
    unresponsive ophthalmic manifestations, cardiac,
    neurologic and progressive pulmonary involvement.
  • Systemic therapy for patients with hypercalcemia.

27
Treatment
  • Prednisone, 20 to 40 mg/d in divided doses or
    alternate-day dosing is used for organ
    involvement that is not life threatening.
  • Higher dosage is used off-label for potentially
    life threatening disease.
  • High-dose inhaled corticosteroids may be useful
    in patients with symptomatic pulmonary disease.

28
Treatment
  • Clinical improvement should be assessed after 3
    months of corticosteroids.
  • If no improvement is found, further treatment is
    unlikely to be beneficial.
  • Long term adverse affects of therapy include
    weight gain, mood swings, cataracts, GERD,
    osteoporosis

29
Alternatives
Drug, dosage Use in sarcoidosis Comment
Methotrexate, 10-25 mg once weekly to max of 1 g or 2 yrs Chronic or worsening disease, common second line drug Effect may take 6 mo, Nausea, neutropenia, and liver toxicity
Azathioprine (Imuran), 50-200 mg QD Chronic or worsening disease, advanced fibrotic sarcoidosis Nausea, neutropenia
Cyclophosphamide (Cytoxan) 50-150 mg QD or 500-2,000 mg q 2wk IV Refractory cases only Toxicity limits use, Nausea, neutropenia Requires intense monitoring and F/U
Hydroxychloroquine (Plaquenil), 200-400 mg QD Cutaneous manifestations, hypercalcemia, chronic pulmonary fibrotic disease Corneal deposits, retinopathy. Ophtho exam prior to treat-ment and q 6 mo
30
Thank You!
Write a Comment
User Comments (0)
About PowerShow.com