Disease Gene defect Attack duration Clinical features Risk of amyloidosis Treatment Familial Mediterranean fever (FMF) MEFV 1–3 days Episodic fever, peritonitis, pleuritis, erysipeloid erythema, monoarthritis M694V and M694I mutations Colchicine…
Type Subtype Features 1. Circumscribed morphea (a) Superficial Oval or round circumscribed areas of induration limited to the epidermis and dermis, often with altered pigmentation and violaceous, erythematous halo (lilac…
Class – name Light microscopy Immunofluorescence/electron microscopy (location of immune deposits) Proteinuria Hematuria Hypertension I – minimal mesangial Normal Mesangial Absent Absent Absent II – mesangial proliferative Mesangial expansion and/or…
Fig. 30.1 The scleroderma patterns in systemic sclerosis are characterised by progressive loss of capillaries. In an early pattern, there is a prevalence of giant capillaries without loss of capillaries….
Fig. 22.1 Cytolytic pathway proteins mutated in MAS. A diagram of the immunologic synapse between a cytolytic (to the left) lymphocyte and an APC (to the right) is portrayed. Proteins…
Fig. 37.1 Arrows showing multiple aneurysms in the medium-sized renal arteries. Upper arrow shows narrowing of a segment of the splenic artery Introduction Diagnosis of primary vasculitides in childhood is…
Fig. 27.1 Heliotrope rash, periorbital puffiness and malar rash in a patient with dermatomyositis Fig. 27.2 Gottron’s papules on (a): fingers and (b): knees Skeletal muscle weakness is typically proximal…
Malar rash It is a macular papular rash in the malar area and usually spares the nasolabial folds. This rash tends to be photosensitive and heals without scaring Alopecia It…
Fig. 15.1 MRI of the right hip shows an osteoid osteoma at the upper metaphyseal end of the right femur This case illustrates several learning points: (a) Many cases of…
Fig. 49.1 Cross section of eye showing areas affected in anterior, intermediate and posterior uveitis and potential complications (in brackets) (Permission obtained from Wiley © Hughes and Dick [4]) In…
