Rheumatological Emergencies and the General Physician

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Rheumatological Emergencies and the General
Physician
David G I Scott Norfolk and Norwich University
Hospital School of Medicine, Health Policy and
Practice University of East Anglia RCPSG
Triennial Conference Glasgow 7th November 2008
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• Some common medical emergencies
• Acute hot joint (monoarthritis)
• Acute polyarthritis
• Sick patient with known ct disease
• RA, SLE, AS etc
• complications of the disease
• complications of treatment
• Acute presentation of ct disease
• eg vasculitis

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Acute monoarthritis Septic arthritis mostly
staph aureus but. Crystal arthritis
gout/pseudogout Reactive arthritis Presentation
of polyarthritis
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Acute hot joint Presenting features - red,
hot immobile and exquisitely tender /-
pyrexia, ill leucocytosis Must
differentiate septic bursitis/cellulitis do NOT
put needle into potentially sterile joint through
infected soft tissue!!!! MUST ASPIRATE - gram
stain, microscopy and culture
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What about steroid injections? No problem even
with infection IF immobilise antibiotics
(wrong answer for MRCP however!) Danger is
misdiagnosis and discharge If in doubt await
microscopy and gram stain Good treatment for
crystal arthritis and reactive arthritis
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Gout and pseudogout Elderly,
drugs, dehydration Uric acid level helpful but
DROPS during acute attack Radiology
chondrocalcinosis NSAID, Colchicine, steroids for
acute attack Allopurinol - for gt2 attacks per
year severe
hyperuricaemia, tophi
increased risk of acute attack for up to 6
months dont stop during
acute attack why wait 2
weeks ? (textbook/MRCP v evidence)
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Acute polyarthritis Nb sepsis and especially
strep ?can be fatal in hours in
immunocompromised (post splenectomy etc) Viral
usually short lived (lt 2 weeks) -
transient - parvovirus and borellia often
more prolonged Acute RA Other ct diseases SLE
etc Polyarticular gout Seronegative arthritis
AS, Reiters/reactive, enteropathic, psoriatic
etc
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Temporal Patterns of Joint Involvement

• Migratory
• Rheumatic fever, gonococcal
• Additive
• RA
• Intermittent
• RA, psoriatic, gout, sarcoid

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Reactive Arthritis

• Infections
• Genitourinary - Chlamydia
• Gastrointestinal - Salmonella
• - Shigella
• - Campylobacter
• - Yersinia

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Reactive Arthritis Common
Extra-articular Features

• Ocular
• Genital
• Cutaneous
• Gastrointestinal
• Cardiac
• Rare
• Neuropathy
• IgA nephropathy
• Renal amyloidosis
• Thrombophlebitis
• Erythema nodosum (Yersinia)

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Rheumatoid Arthritis

• Serositis may be presenting feature
• Other EAM usually late
• vasculitis skin infarction/ulcers
• - acute neuropathy
• Felty neutropenia, infection
• fibrosing alveolitis /- infection
• amyloid and renal failure
• heart block, constrictive pericarditis,
• cervical myelopathy
• AND increased risk of IHD just like diabetes
  

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RA treatment effects

• Sepsis/neutropenia DMARDs MTX
• Breathless flare fibrosing alveolitis,
  biologics/MTX
  pneumonitis,
  pneumonia (in wrong order!)
• Cutaneous vasculitis - MTX, biologics
• Renal failure NSAIDs GI bleed
• TB esp atypical TB with biologics

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SLE

• Causes almost any medical problem!
• CNS fits, transverse myelitis
• Cardiac increase risk MI,
  pericarditis, tamponade
• Kidney - acute renal failure
• Thromboses especially with APL
• check ANA, ENA,
  DNA, ACA, ANCA,
  complement,
  
  RF and cryoglobulins

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Systemic vasculitis

• Another great mimic
• Many diseases,
  similar problems
• Major developments in
  classification and
  treatment
• Still early mortality
• Now also presenting as
  chronic relapsing diseases with increased
  cardiovascular risk

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Vasculitis - a histological triad
leucocyte infiltration
thrombotic vascular occlusion
fibrinoid necrosis
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Classification of Systemic Vasculitis Primary
Secondary LARGE GCA aortitis
- RA, AS ARTERIES Takayasu infection -
syphilis MEDIUM cPAN infection - Hep
B ARTERIES Kawasaki malignancy - hairy cell
leuk SMALL/MEDIUM Wegeners RA, SLE,
SS VESSELS MPA infection - HIV, Hep
C Churg-Strauss drugs SMALL HSP RA, SLE,
SS VESSELS ess.cryo.angiitis infection cut.leuco.
vasculitis drugs ANCA, cyclo responsive,
glomerulonephritis Scott
Watts 1994 BJR
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Norfolk Vasculitis Register 1988 - 2008

• Total 690 patients
• Primary Systemic Vasculitis
• WG 138 Aortitis
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• MPA 59 Takayasus 5
• CSS 37 Behcets
  5
• Secondary Vasculitis SRV 81 NFV 48
• Small Vessel Vasculitis cutaneous LV 74
• cryo
  vasculitis 5
• adult
  HSP 23

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SYSTEMIC RHEUMATOID VASCULITIS the present
incidence/ n
million/yr m f 1988-92 24
11.6 (7.4-17) 12 11.2 1993-97 19
8.9 (5.3-13.6) 7.7 9.9 1998-02
8 3.6 (1.6-7.1) 3.7 3.5

• ?12/million/year
• 2 yr mortality 23-46
• ? risk mononeuritis,
• gut involvement, arteritis
• Scott et al 1981

Watts, Mooney, Lane Scott Rheumatology
200443920-3
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Wegeners Granulomatosis
granulomatous inflammation
respiratory tract and
necrotising vasculitis of small to medium
sized vessels

• ACR 1990
• nasal or oral inflammation
• (ulcers, discharge)
• abnormal CXR (nodule/ cavity)
• urine sediment (red cells/casts)
• biopsy changes
• v
  assn with PR3ANCA
• (95 generalised 50 local)

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MICROSCOPIC POLYANGIITIS

• Term first used by Wainwright Davson 1950
• Not considered by ACR
• CHCC definition -
• necrotising arteritis - pauci- immune
• involving small vessels
• ? small/medium sized arteries
• necrotising glomerulonephritis
• very common
• pulmonary capillaritis often occurs
• Strong association with ANCA (mpo)

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CHURG-STRAUSS SYNDROME

• Asthma
• Eosinophilia gt 1 x 109/l
• Systemic vasculitis
• involving ? 2 extra
• pulmonary organs
• (Lanham et al 1984)
• 50 ANCA (mpo)

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Clinical Features Wegeners
Granulomatosis Microscopic Polyangiitis Churg
Strauss syndrome
Cases
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Classic POLYARTERITIS NODOSA (CHCC 1994)
Necrotising inflammation of medium or small
arteries Without - glomerulonephritis -
vasculitis in
arterioles, capillaries or venules Using
this definition - cPAN is usually ANCA
negative And much rarer than other PSVs
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Recognition

• Spectrum of vasculitis is broad
• Fulminating life threatening disease
• Minor rash with no organ involvement
• Rare but must be considered in severe
  multisystem disease
• Take good history asking for suggestive symptoms

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Important mimics

• S.B.E
• Atrial myxoma
• Cholesterol embolism
• Antiphospholipid
• antibody syndrome
• Calciphylaxis

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Specific and important scenarios 1

• Skin rash
• unwell
• ve urinary sediment
• arthralgia
• .

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2 chronic/persistant ENT disease

• eg crusting, epistaxis
• unwell
• ve urinary sediment
• arthralgia

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3 persistant/recurrant eye inflammation

• Usually iritis/scleritis
• not conjunctivitis
• unwell
• ve urinary sediment
• arthralgia

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4 neuropathy

• Usually acute and especially motor
• ve urinary sediment
• rash
• history of asthma (recent flare)

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5 atypical pneumonia

• ve urinary sediment
• unwell
• arthralgia

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OUTCOME

• Renal Creatinine alive 138 vs.
  Creatinine dead 485 plt0.001

• Bx sclerosis

• age
• EUVAS mortality
• ANCA - PR3, persistence
• GI involvement
• cardiac involvement

Booth et al. Am J Kid Dis 2003 41776 - 84
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Sepsis RR 14

active CVD other
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4
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Cause of death
87.5
469 patients included in the long term follow-up
study
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River Wensum - Norwich