Urological manifestations and treatment of the primary systemic vasculitides

Abstract

The primary systemic vasculitides (PSV) are a group of rare inflammatory disorders affecting blood vessels of varying size and multiple organs. Urological manifestations of PSV are uncommon. Testicular vasculitis is the most commonly reported finding and is associated with Polyarteritis Nodosa (PAN), Henoch-Schönlein Purpura (HSP), anti-neutrophil cytoplasm antibody associated Vasculitides (AAV), Giant Cell Arteritis (GCA) and Kawasaki disease. Prostatic vasculitis has been reported in association with GCA and AAV. Ureteric involvement has been noted in PAN, HSP and AAV. Other urogenital manifestations of PSV include genital ulceration and bladder dysfunction in Behçets Disease and haematuria which is commonly seen in many of the PSV. Finally, therapies used to treat the PSV, especially cyclophosphamide, are associated with urological side-effects including haemorrhagic cystitis and urothelial malignancy. The aim of this review is to examine how the urological system is involved in the PSV. Each PSV is examined in turn, with a brief clinical description of the disease followed by a description of the urological manifestations and management. Identification of urological manifestations of PSV is important as in many cases symptoms may improve with immunosuppressive therapy, avoiding the need for invasive surgery. Additionally, patients who present with isolated urogenital PSV are at higher risk of developing subsequent systemic vasculitis and will need to be followed up closely.

Keywords: Urology; Vasculitis; Takayasu arteritis; Giant cell arteritis; Polyarteritis Nodosa; Kawasaki disease; Henoch-Schönlein Purpura; Anti-neutrophil cytoplasm antibody associated vasculitides; Granulomatosis with polyangiitis; Microscopic polyangiitis; Behcets disease; Eosinophilic granulomatosis with polyangiitis; Epididymo-orchitis; Prostatitis; Ureteric stenosis

Core tip: Urogenital manifestations of primary systemic vasculitides are rare. Patients will usually have other systemic signs and symptoms to suggest an underlying vasculitic process. Inflammatory markers including erythrocyte sedimentation rate and C-reactive protein are often raised and specific auto-antibody testing may be useful in identifying the underlying diagnosis. If an underlying vasculitic process is suspected, early involvement of a specialist is essential. Symptoms usually improve with immunosuppressive therapy and may avoid the need for invasive surgery. Patients who present with isolated single-organ vasculitis of the urogenital tract will need to be followed up closely in case they subsequently develop systemic disease.