23: Vasculitis and Related Rashes

CHAPTER 23
Vasculitis and Related Rashes


Richard A. Watts1 and David G.I. Scott2


1 Ipswich Hospital NHS Trust, Ipswich; Norwich Medical School, Norwich, UK


2 Norfolk and Norwich University Hospital NHS Trust; Norwich Medical School, Norwich, UK


The vasculitides are a heterogeneous group of uncommon diseases characterized by inflammatory cell infiltration and necrosis of blood vessel walls. Systemic necrotizing vasculitis can be rapidly life‐threatening, so early accurate diagnosis and treatment are vital. Vasculitis may be primary (granulomatosis with polyangiitis (Wegener’s), eosinophilic granulomatosis with polyangiitis (EGPA; Churg–Strauss syndrome), microscopic polyangiitis, polyarteritis nodosa) or secondary to established connective tissue disease (such as rheumatoid arthritis), infection or malignancy. The severity of vasculitis is related to the size and site of the vessels affected. Classification is based on vessel size and determines the treatment approach (Table 23.1, Box 23.1).


Table 23.1 Classification of vasculitis

























































Dominant vessel Idiopathic (primary) Probable aetiology (secondary)
Large Takayasu’s Syphilis

Giant cell arteritis Tuberculosis


Aortitis – RA, AS
Medium Polyarteritis nodosa (classic) HBV‐associated polyarteritis nodosa

Kawasaki’s disease
Small
ANCA Microscopic polyangiitis Drugs (propylthiouracil, hydralazine)*

Granulomatosis with polyangiitis

Eosinophilic granulomatosis with polyangiitis
Immune complex Anti‐GBM disease Cryoglobulinaemic vasculitis (HCV)

Cryoglobuinaemic vasculitis (non‐HCV) RA, SLE, Sjögren’s syndrome

IgA vasculitis Serum sickness

Hypocomplementaemic vasculitis Drug induced ‡
Variable Behçet’s

Cogan’s

AS, ankylosing spondylitis; GBM, glomerular basement membrane; HBV, hepatitis B virus; HCV, hepatitis C virus; RA, rheumatoid arthritis; SLE, systemic lupus erythematosus.


Large‐vessel vasculitis


Large‐vessel vasculitis includes giant cell arteritis and Takayasu’s arteritis. Giant cell arteritis is described elsewhere (Chapter 17). Takayasu’s arteritis is uncommon and affects young adults, who initially present with a non‐specific illness and later with loss of pulses, claudication (especially of the upper limbs) and stroke.


Medium‐vessel vasculitis


Polyarteritis nodosa


A multisystem vasculitis characterized by formation of microaneurysms in medium‐sized arteries. Patients present with a constitutional illness, which is often associated with rash, mononeuritis multiplex, vascular hypertension and organ infarction. Polyarteritis nodosa may be confined to the skin. Angiography shows typical microaneurysms. Polyarteritis nodosa is associated with hepatitis B infection.


Kawasaki’s disease (mucocutaneous lymph node syndrome)


An acute vasculitis that primarily affects infants and young children. It presents with fever, rash, lymphadenopathy and palmoplantar erythema. Coronary arteries become affected in up to one‐quarter of untreated patients; this can lead to myocardial ischaemia and infarction.


Medium‐ and small‐vessel vasculitis


This group includes the major necrotizing vasculitides: microscopic polyangiitis, granulomatosis with polyangiitis (Wegener’s), eosinophilic granulomatosis with polyangiitis (Churg–Strauss), with involvement of both medium and small arteries. The symptoms depend on the size and site of vessel affected and on the individual diagnosis. They are associated with the presence of antineutrophil cytoplasmic antibodies (ANCA).


Granulomatosis with polyangiitis (Wegener’s)

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Nov 5, 2018 | Posted by in RHEUMATOLOGY | Comments Off on 23: Vasculitis and Related Rashes
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