Urological manifestations and treatment of the primary systemic vasculitides

Table 1 Summary of the urogenital manifestations of the primary systemic vasculitides, defined according to the Chapel Hill Consensus Criteria

Primary systemic vasculitides		Urogenital manifestations
Large vessel	Takayasu arteritis	Obstructive uropathy secondary to retroperitoneal fibrosis
	Giant cell arteritis	Epididymo-orchitis
Medium vessel	Polyarteritis Nodosa	Orchitis, ureteric stricture, haematuria, glomerulonephritis, spontaneous peri-renal haemorrhage
	Kawasaki’s disease	Orchitis, ureteric stricture
Small vessel	Antineutrophil cytoplasmic antibody associated vasculitis
	Microscopic polyangiitis	Glomerulonephritis, prostatitis
	Granulomatosis with polyangiitis (Wegener’s)	Glomerulonephritis, prostatitis, orchitis, ureteric stenosis, penile ulceration, necrotizing urethritis, large renal and bladder granulomas ("pseudotumours")
	Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)	Prostatitis, ureteric stricture, urethral ulceration
	Immune complex small vessel vasculitis
	Anti-glomerular basement membrane disease	Glomerulonephritis, haematuria
	IgA vasculitis (Henoch-Schonlein)	Glomerulonephritis, haematuria
	Cryoglobulinaemic vasculitis	Glomerulonephritis, haematuria, epididymo-orchitis, ureteric strictures, bladder wall hematoma, haemorrhagic cystitis, penile and scrotal pain and swelling, priapism, spermatic vein thrombosis
	Hypocomplementaemic urticarial Vasculitis (anti-C1q vasculitis)	Glomerulonephritis, haematuria
Variable vessel	Behçets disease	Genital ulceration, epididymitis, sterile urethritis, bladder wall ulceration, cystitis, vesico-vaginal fistula, neuropathic bladder
	Cogan’s syndrome	Nil