1Regional Renal Unit Royal Liverpool University Hospital NHS Trust 2Department of Medicine and 3Department of Pathology University of Liverpool and 4Liverpool Womens Hospital Liverpool, UK Email: jlalexa{at}liv.ac.uk
Case
A 57-year-old housewife presented to the emergency department with seizures. She was drowsy and disorientated, febrile, tachypnoeic, tachycardic and tender in the left loin. There were a few brown macules on her ankles. Recent history revealed low back pain, ankle swelling, a purpuric rash and haematuria. Two years earlier, a nodular lesion on her left leg was diagnosed and treated as pyoderma gangrenosum.
Laboratory studies on admission showed: white cell count 23.8 x 109/l, haemoglobin 7.2 g/dl, potassium 7.4 mmol/l, urea 70.1 mmol/l and creatinine 1360 µmol/l.
She was treated with phenytoin, antibiotics and haemodialysis. An ultrasound scan demonstrated hydronephrosis of the right kidney and an enlarged left kidney. A CT scan of the pelvis revealed a mass adjacent to the right side of the uterus (Figure 1). Despite insertion of a nephrostomy on the right, she remained anuric and dialysis dependent.
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What is the diagnosis? What additional procedure and laboratory test would support this? What is the cause of the obstruction?
Answers to the quiz on the preceding page
Our patient had Wegeners granulomatosis (WG). The diagnosis was resolved by a left renal biopsy. All glomeruli (16) had extracapillary sclerosis. Some glomeruli contained residual cellularity and one glomerulus was surrounded by a lymphoplasmacytic inflammatory infiltrate (Figure 2). Moderate interstitial lymphocytic infiltration with tubular damage and patchy fibrosis was noted, and the vessels appeared normal. Immunofluorescence showed non-specific deposition of IgM and C3. The combination of extensive glomerular involvement with residual extracapillary cellularity and normal vessels was consistent with a burnt out crescentic glomerulonephritis. A positive cANCA (24 U; normal 010 U) was obtained and, with the renal histology, WG was diagnosed. The hydronephrosis on the right was caused by the pelvic mass. Examination under anaesthetic demonstrated rigid nodular tissue, and hysteroscopy showed an absent uterine fundus with communication between cervical canal and the pelviperitoneum. Biopsy of the uterus and parametrium revealed granulomatous inflammation (Figure 3), consistent with WG.
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The major differential diagnosis in this patient was pelvic malignancy, most probably gynaecological in origin. Malignant disease is a recognized association of rapidly progressive and crescentic glomerulonephritis [1], so this diagnosis would have explained the clinical presentation. Our case clearly illustrates that WG can mimic carcinomatous lesions, hence the importance of obtaining a histological diagnosis.
The unique feature of our case was the perforated uterus revealed by hysteroscopy. Genitourinary manifestations of WG are rare, but have been well described [24]. While cervical involvement has been noted [2,4], in contrast to other vasculitidies [5,6], there have been no reports of WG affecting the body of the uterus, let alone uterine perforation. Ureteric obstruction has also been described as a manifestation of WG, with unilateral hydronephrosis presenting as haematuria or a urinary tract infection, and bilateral hydronephrosis presenting with a retroperitoneal pseudotumour [2]. In our patient, the hydronephrosis was less clinically significant due to extensive glomerular and interstitial damage in the kidneys, and hence the explanation for the failure of percutaneous nephrostomy to improve renal function. Skin manifestations of WG are common. Necrotizing ulceration consistent with pyoderma gangrenosum, including unusual sites such as the face, neck, trunk and perineum, has been identified in patients with WG [7,8]. As in our patient, pyoderma gangrenosum may herald later renal involvement [7].
Treatment of WG is based upon immunosuppressive agents and is generally tailored to the severity of the disease. High dose steroids, sometimes pulsed intravenous prednisolone, combined with oral or intravenous cyclophosphamide are used to induce remission of disease activity. We used pulsed methylprednisolone followed by a reducing regime of oral prednisolone and oral cyclophosphamide in our patient. Her fever resolved, and over a period of months the pelvic mass disappeared. Our patient sadly succumbed from pneumococcal septicaemia 2 years later.
In summary, the case shows WG should be considered as a cause of a pelvic mass with ureteric obstruction, particularly when there are other features suggestive of vasculitis.
Conflict of interest statement. None declared.
References