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

Table 2 Main clinical manifestations of patients with polyarteritis nodosa[7^,14]

Manifestation	Specific problems	Frequency
Systemic symptoms	Fever, malaise, weight loss, myalgias arthralgias	80%
Neuropathy	Mononeuritis multiplex, polyneuropathy	75%
Arthralgias and/or myalgias	Articular and/or diffuse extremity pain	60%
Cutaneous	Livedo reticularis, purpura, ulcers	50%
Renal disease	Elevated creatinine, haematuria, proteinuria	50%
Gastrointestinal symptoms	Abdominal pain, nausea/vomiting, diarrhoea rectal bleeding, ulceration, peritonitis	40%
Hypertension	New onset	35%
Central nervous system disease	Stroke, confusion	20%
Orchitis	Testicular pain, swelling	20%
Cardiac involvement	Cardiomyopathy, pericarditis, palpitations, myocardial infarction	10%
Peripheral vascular disease	Claudication, ischaemia, necrosis	10%
Ophthalmological	Exudates, vasculitis	10%

Table 3 International Study Group Criteria for Behçets disease[153]

Required criteria
Recurrent oral ulceration (obligatory)	Minor aphthous, major aphthous, or herpetiform ulceration (observed by physician or patient); recurring at least 3 times in a 12 mo period
Plus 2 of
Recurrent genital ulceration	Aphthous ulceration or scarring (observed by physician or patient)
Eye lesions	Anterior uveitis, posterior uveitis, or cells in vitreous on slit lamp examination; or retinal vasculitis observed by ophthalmologist
Skin lesions	Erythema nodosum (observed by physician or patient), pseudofolliculitis, or papulopustular lesions; or acneiform nodules (observed by physician) in post-adolescent patients not on corticosteroid treatment
Positive Pathergy test	Read by physician at 24-48 h