JRSM >
Volume 96, Number 7 >
Pp. 353-354
Centre for Nephrology, Royal Free Hospital, Pond Street, London NW3 2QG, UK
Correspondence to: Dr Gareth Jones E-mail: gl.jones{at}rfc.ucl.ac.uk
Small-vessel vasculitis associated with anti-neutrophil cytoplasmic antibodies (ANCA) has been reported in malignant disease1,2 but can also produce inflammatory masses that mimic neoplastic lesions.
CASE HISTORY
A man of 56 was referred with a short history of lumbar back pain, night sweats and weight loss. Examination was unremarkable except for dipstick haematuria. An intravenous urogram and lumbar spine X-rays were normal. Renal function (creatinine 85 mmol/L), calcium and full blood count were within normal limits but his inflammatory markers were raised—C reactive protein (CRP) 215 mg/L, erythrocyte sedimentation rate 53 mm/h. He was initially treated with antibiotics and non-steroidal anti-inflammatory drugs for a presumed urinary tract infection but did not improve. At review, a CT scan of his pelvis demonstrated a large presacral mass (Figure 1). CT guided biopsies yielded only necrotic material and he was referred for open surgical biopsy.
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On admission, he reported recent development of red lesions on his shins, pain in his knees and a sore left eye. Routine chemistry revealed a rise in his creatinine and he was transferred to our institute. On physical examination he had multiple nailfold splinter haemorrhages, sublingual vasculitic lesions, raised erythematous lesions on both shins and necrotizing scleritis of the left eye. The deterioration in renal function (creatinine 584 mmol/L) was associated with anaemia (haemoglobin 7.8 g/dL), thrombocytosis (platelets 6706109) and a persistently high CRP (250 mg/L). Indirect immunofluorescence identified perinuclear ANCA (pANCA) and plasma contained antibodies against myeloperoxidase (MPO). An extensive sepsis screen, echocardiography, and blood tests including protein electrophoresis, tumour markers and other autoantibodies yielded nothing of note. A renal tract ultrasound revealed unobstructed normal sized kidneys and a subsequent renal biopsy showed 14 normocellular glomeruli, although 3 had capsular adhesions. There was no evidence of focal necrosis or inflammation in blood vessels or tubules.
Vasculitis was diagnosed and treatment was started with methylprednisolone, followed by oral prednisolone (initially 60 mg daily) and oral cyclophosphamide (2 mg/kg). The lumbar pain and the lesions on shins, tongue and eye healed; creatinine (87 mmol/L) and CRP (4 mg/L) became normal within 2 months from the start of immunosuppression. On repeat CT scan 3 months after referral the presacral mass was unchanged and open biopsy was scheduled. At operation he had a large, homogeneous, postrectal mass adherent to the sacrum. Histological examination of wedge biopsies revealed necrotic fibrofatty tissue with evidence of acute and chronic inflammation but no granulomata, malignant change or vasculitis. A CT scan 6 months after the start of immunosuppression showed little change but a year later the mass had resolved completely (Figure 2).
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COMMENT
This patient was initially thought to have a vasculitis secondary to malignancy but the response to immunosuppression, the positive serology and the results of the wedge biopsy suggest that the mass was secondary to a systemic inflammatory disorder such as a small-vessel vasculitis. In ANCA-positive vasculitis inflammatory masses have been reported in the lung,3 mediastinum,4 orbit, parotid and pancreas5 and positive ANCA-IIF has also been reported in a patient with retroperitoneal fibrosis.6 ANCA-positive vasculitis would explain the presenting features, clinical evidence of vasculitis and positive serological tests. Against this was the lack of histological evidence on renal or pelvic mass biopsies; however, at the time of the mass biopsy the patient had been on immunosuppressive treatment for several weeks. The renal impairment may not have been related to his vasculitis. One possible explanation is the combination of non-steroidal anti-inflammatory drugs and radiographic contrast, which might have induced acute tubular necrosis not apparent in the biopsy specimen. Vasculitis might, however, have been missed in the sampled renal tissue, because of the focal nature of small-vessel vasculitis, and the only evidence in this sample was the 3 capsular adhesions.
Clearly, in a patient with ANCA/MPO vasculitis and a pelvic mass, the possibility of malignant disease must be excluded. This case, however, shows that a pelvic inflammatory mass can resolve with immunosuppression.
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