Rheum

Question 1

19yr, Back and joint paint, brown/bluish pigmentation in eyes and sclera, dark urine

Answer 1

Alkaptonuria..

Hereditary Deficiency of enzyme homogentisic oxidase

Question 2

classic presentation of Polyarteritis nodosa (PAN)

Answer 2

medium vasculitis w necrotizing inflammation leading to aneurysm formation

must have 3 of:

>5kg weight loss
Livedo reticularis
Testicular pain/tenderness
Myalgia
Mono/polyneuropathy
Diastolic BP > 90
Raised Ur Cr
Hep B serology positive (in 30%)

Question 3

net-like rash and neuropathy in a patient with hepatitis B is suggestive of…

Answer 3

Polyarteritis nodosa (PAN)

Question 4

vasculitic rash and neuropathy in a patient with hepatitis C is suggestive of…

Answer 4

cryoglobulinaemia

Question 5

causes of mononeuritis multiplex…

Answer 5

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) (sinusitis, asthma, pANCA 60%, blood eosinophilia)
Polyarteritis nodosa  (sinusitis, testicular pain, livedo retic, 20% pANCA, Hep B)
Microscopic polyangiitis (palpable purpura, fever weight loss, pANCA 60%, cANCA 40%)

Question 6

presentation of Still’s disease in adults

Answer 6

arthralgia
macpap salmon pink rash (most prominent in afternoon/evening)
elevated ferritin and other acute phase reactants
lymphadenopathy
pyrexia

Rheum factor and ANA neg

Mx w NSAIDs
Steroids (may help symptoms only)

Question 7

malaise over months

Fever, right auricular swelling, bilateral anterior uveitis, and a symmetrical small joint polyarthritis

Answer 7

Relapsing polychondritis

multi-systemic condition which most commonly causes relapsing episodes of auricular chondritis