Chronic vasculitis causing unilateral ureteral stenosis

CHRONIC VASCULITIS CAUSING UNILATERAL URETERAL STENOSIS GARY R . GOODMAN, M .D . JEFFREY R . WOODSIDE, M .D . WILLIAM J . SLICHENMYER, M .D . From the Division of Urology and Department of Pathology, University of New Mexico School of Medicine, Albuquerque, New Mexico

ABSTRACT-We report a case of chronic vasculitis causing unilateral ureteral stenosis as an isolated phenomenon in an otherwise healthy forty-three-year-old man. Other causes of chronic vasculitis and ureteral stenosis are reviewed .

We report the case of a patient who underwent a distal ureterectomy for localized ureteral stenosis in which subsequent pathologic study revealed chronic vasculitis . We believe this localized entity in the absence of systemic disease has not been reported in the English literature . Case Report A forty-three-year-old man had a threemonth history of continuous and gradually worsening pain in the left flank which radiated to the lower left abdominal quadrant . There was no history of gross hematuria, dysuria, urolithiasis, tuberculosis, nausea, or travel outside the continental United States . Past medical history was significant for rheumatic fever as a child treated with penicillin without sequelae . Between twenty and thirty-one years old he was an alcoholic and had twelve hospitalizations for pancreatitis . A recent intravenous pyelogram (IVP) revealed normal kidneys but mild left hydroureteronephrosis to the level of the pelvic brim where there was a persistent narrowing (Fig . 1) . A retrograde pyelogram delineated a narrowing at this area and a suggestion of a filling defect .

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FIGURE 1 . Intravenous pyelogram shows mild left ureteropyelocalicectasis to level of pelvic brim and persistent narrowing .

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FIGURE 2 . (A) Medium-power photomicrograph of serosal region of ureter ; note thrombosed and recanal-

ized arteries with mural inflammation . (B) High-power photomicrograph of inflamed artery : infiltrate comprised of lymphocytes, eosinophils, and few plasma cells ; small vessels and veins also involved . (Elastic stains, original magnifications x 312 and 600, respectively.)

At our institution cystoscopic examination and left retrograde pyelogram confirmed the ureteral narrowing . Ureteroscopy was attempted, but the ureteroscope could not be negotiated as high as the lesion . A cup biopsy forceps obtained tissue from the lesion under fluoroscopic control . Pathologic evaluation revealed chronically inflamed stromal tissue with focal urothelial atypia . Urine cytologic findings were negative for malignancy. The patient subsequently underwent surgical exploration of the lower ureter . The ureter was diffusely indurated from the level of the lesion distally to the bladder, and the surrounding tissues were normal . A distal ureterectomy including a bladder cuff was performed . Frozen section revealed chronic ureteritis and no evidence of neoplasm . Ureteral continuity was reestablished by ureteral reimplantation into a Boari flap . Gross pathologic examination revealed a smooth and intact serosal surface with multiple areas of underlying induration . The longitudinally opened ureter showed several areas of luminal stenosis caused by firm, gray-tan intramural tissue . The mucosal surface was smooth with no ulcerative or polypoid lesions . The ureteral wall measured 9 mm in maximal thickness . Microscopic sections of the ureter revealed striking mural thickening and fibrosis most marked in the serosal layer, and a prominent panvasculitis involving the small muscular arteries, arterioles, and capillaries (Fig . 2A) . The inflammatory infiltrate consisted predominantly of lymphocytes, numerous eosinophils,

and scattered plasma cells . No necrosis or granulomatous inflammation was present, but there was perivascular fibrosis and inflammation in an "onion skin" pattern (Fig . 2B) . Scattered lymphoid follicles were present in adjacent tissue unassociated with vessels . There were focal urothelial ulcerations, but the majority of the inflammation and fibrosis was in the serosal layer. The final pathologic diagnosis was stenosing ureteritis secondary to chronic vasculitis of uncertain etiology. Pertinent laboratory tests included normal eosinophil count, rheumatoid factor, serum complement levels, rapid plasma reagin, and antinuclear antibody . A postoperative erythrocyte sedimentation rate was 76 mm/hr. Comment Common causes of ureteral obstruction include calculi, congenital stricture, intrinsic and extrinsic tumors, blood clot, pregnancy, and retroperitoneal fibrosis . Furthermore, many inflammatory conditions may affect the lower urinary tract and cause obstruction . Bacterial infection may lead to a nonspecific chronic ureteritis with polypoid, ulcerative, gangrenous, or hemorrhagic lesions . Follicular and eosinophilic lesions have also been described in the lower urinary tract . Malakoplakia, rheumatoid nodules, tuberculous cystitis, and infestation by Schistosoma haematobium can also occur in bladder or ureters .' Of these inflammatory conditions, only schistosomiasis 2 and retroperitoneal fibrosis' 4 are commonly acknowledged to produce a vasculitis . Clinical

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and histologic findings in this patient preclude these diagnoses . Focal vasculitis may be caused by infectious arteritis .s This usually occurs adjacent to abscesses, caseous tuberculous reactions, or necrotizing pneumonias but also may arise from the hematogenous spread of bacteria . The histology of these vasculitic lesions is nonspecific, with fibrinoid and leukocytoclastic necrosis predominating. Arteritis restricted to the vermiform appendix, without local symptoms or systemic disease, has been described .' Vasculitis causing ureteral stenosis has been described only in association with systemic disease such as Wegener granulomatosis,''' polyarteritis nodosa,° allergic granulomatosis and angiitis, 10 or drug-related vasculitis ." A localized periarteritis of the right common iliac artery has been reported in which extrinsic compression caused ureteral obstruction . 12 The vasculitis in our patient seems to be unique because no associated systemic disease is apparent . Polyarteritis nodosa is unlikely because no necrosis exists. Furthermore, the absence of granulomas makes Wegener granulomatosis unlikely. Finally, the absence of asthma, skin lesions, and peripheral eosinophilia make allergic granulomatosis or angiitis or hypersensitivity vasculitis unlikely. 13,14 Thorough rheumatologic evaluation failed to reveal any evidence of systemic vasculitis or collagen vascular disorder . Close follow-up is planned to detect any systemic disorder of which ureteral vasculitis was the initial manifestation .

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Division of Urology University of Texas Health Science Center 6431 Fannin, Suite 6018 Houston, Texas 77030 (DR . WOODSIDE)

References 1 . Friedell CH, Soto EA, Parija GC, and Nagy GK : The renal pelvis, ureter, urinary bladder, and urethra, in Silverberg SG (Ed) : Principles and Practice of Surgical Pathology, New York, John Wiley and Sons, 1983, p 1140 . 2 . McCulley RM, Barron CN, and Cheever AW: Schistomiasis, in Binford CH, and Connor DH (Eds) : Pathology of Tropical and Extraordinary Diseases, Washington, D .C ., Armed Forces Institute of Pathology, vol 2, 1976, p 492 . 3 . Mitchinson MJ : The pathology of idiopathic retroperitoneal fibrosis, J Clin Pathol 23 : 681 (1070) . 4 . Lepor H, and Walsh PC : Idiopathic retroperitoneal fibrosis, J Urol 122 : 1 (1979) . 5 . Robbins SL, Cotran RS, and Kumar V ; Pathologic Basis of Disease, ed 3, Philadelphia, W. B . Saunders Co., 1984, p 528 . 6 . Plaut A : Asymptomatic focal arteritis of the appendix, 89 cases, Am J Pathol 27 : 247 (1951) . 7 . Ronco P, et at ; Ureteral stenosis in Wegener's granulomatosis, Nephron 30 : 201 (1982) . 8 . Hensle TW, Mitchell ME, Crooks KK, and Robinson D : Urologic manifestations or Wegener's granulomatosis, Urology 12 : 553 (1978) . 9 . Melin JP, et at : Polyarteritis nodosa with bilateral ureteric involvement, Nephron 32 : 87 (1982) . 10 . Chumbley L, Harrison EG, and DeRemee RA : Allergic granulomatosis and angiitis (Churg-Strauss syndrome), Mayo Clin Proc 52 : 477 (1977) . 11 . Mullick FG, McAllister IIA, Wagner BM, and Fenoglio JJ : Drug-related vasculitis, Human Pathol 10 : 313 (1979) . 12 . Ekeland A, and Sander S : Stenosis of the ureter caused by periarteritis, J Oslo City Hosp 24 : 78 (1974) . 13 . McCloskey RT, and Fienberg 0 : Vasculitis in primary vasculitides, granulomatoses, and connective tissue diseases, Human Pathol 14 : 305 (1983) . 14 . Cupps TR, and Fauci AS : The vasculitic syndromes, Adv Int Mad 27 : 315 (1982) .

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