An approach to the diagnosis and management of systemic vasculitis

Abstract

The systemic vasculitides are a complex and often serious group of disorders which, while uncommon, require careful management in order to ensure optimal outcome. In most cases there is no known cause. Multi-system disease is likely to be fatal without judicious use of immunosuppression. A prompt diagnosis is necessary to preserve organ function. Comprehensive and repeated disease assessment is a necessary basis for planning therapy and modification of treatment protocols according to response. Therapies typically include glucocorticoids and, especially for small and medium vessel vasculitis, an effective immunosuppressive agent. Cyclophosphamide is currently the standard therapy for small vessel multi-system vasculitis, but other agents are now being evaluated in large randomized trials. Comorbidity is common in patients with vasculitis, including the cumulative effects of potentially toxic therapy. Long-term evaluation of patients is important in order to detect and manage relapses.

Fig. 2

Indirect immunofluorescence pattern of perinuclear anti-neutrophil cytoplasm antibody (p-ANCA) from a patient with microscopic polyangiitis.

Fig. 1

Indirect immunofluorescence pattern of cytoplasmic anti-neutrophil cytoplasm antibody (c-ANCA) from a patient with Wegener's granulomatosis.

Fig. 3

Cross-sectional low-power view of temporal artery biopsy from a patient with new-onset headache due to giant cell arteritis, showing an intense inflammatory infiltrate in the adventitia and media, and proliferation of the intima with narrowing of the lumen.

Fig. 5

Skin biopsy of a vasculitic rash showing dense infiltration of the subcutaneous tissue.

Fig. 6

Renal biopsy to show diffuse proliferative pauci-immune glomerulonephritis with basement membrane ruptures and cells in Bowman's space and an associated interstitial reaction (acknowledgements to Dr Lorraine Harper, Educational Director, The Wellcome Trust Clinical Research Facility at the Queen Elizabeth Hospital, Birmingham).

Fig. 7

Nasal biopsy showing dense infiltration of lymphocytes and a large granulomatous mass.

Fig. 9

Nodulopapular lesions on the elbow in a patient with Wegener's granulomatosis. These lesions are mobile in the dermis and may ulcerate.

Fig. 8

Henoch–Schonlein purpura showing recent-onset purpuric rash on calves with coalescence of lesions on the thighs secondary to pressure. There are haemosiderin deposits from previous episodes of purpura and a scar on the right calf from a healed ulcer.

Fig. 4

Low-power view of a sural nerve biopsy from a patient with mononeuritis multiplex showing increased cellularity in relation to perineural and epineural vessels (acknowledgements to Dr Colin Smith, Senior Lecturer/Consultant Pathologist, Pathology Department, University of Edinburgh).

Fig. 10

Plain chest radiograph of a patient with Wegener's granulomatosis showing extensive consolidation in the left upper lobe and a large granulomatous lesion in the right lower zone. Both resolved completely with immunosuppression.

Fig. 11

(a,b) Magnetic resonance angiogram of a patient with new onset Takayasu's arteritis. (a) Stenosis of multiple sites; (b) magnification view to show narrowing if the right internal carotid and occlusion of the left subclavian artery (arrows).

Fig. 12

2-fluoro-2-deoxy-D-glucose (FDG) positron emission tomography (PET) scan through the thorax, of a patient with Takayasu's arteritis to show increased glucose uptake throughout the aorta. The ascending and descending aorta show increased uptake in this cross-sectional image (arrows).

Fig. 14

High-resolution chest computed tomography (CT) scan of a patient with Wegener's granulomatosis showing multiple lung nodules bilaterally of varying size including subpleural nodules (arrow).

Fig. 13

Nasal computed tomography (CT) scan of a patient with Wegener's granulomatosis to show extensive soft tissue infiltration in the sinuses with bony destruction of the medial wall of the maxillary sinuses (arrow).