A Mass, Abdominal Pain and Ulcerative Colitis - Could this be Lupus?
Philip P. Stapleton, Fiona Hayes, Fernando Moro-Azuela, Bhaskar Dasgupta
Department of Rheumatology, Southend University Hospital, Essex, UK
We describe an atypical presentation of Systemic Lupus Erythematous (SLE) with lupus nephritis in a man with quiescent ulcerative colitis (UC) who presented with abdominal symptoms.
A 61-year old man with inactive UC was reviewed by Gastroenterology for severe back pain, weight loss, lethargy and anorexia. Colonoscopy was unremarkable and CT of the abdomen demonstrated a mild left side hydronephrosis with a soft tissue mass thought to be retroperitoneal fibrosis partially encasing the aorta. Elevating creatinine levels assumed secondary to ureteral stricture by the periaortic mass did not reverse with ureteral stenting. Rheumatology input was requested.
Imaging and serologic tests were requested. Diagnostic tests included bone marrow aspirate and trephine biopsy with CT guided biopsy of the paraaortic mass.
18FDG PET CT uptake was suggestive for lymphoma and possible retroperitoneal fibrosis with mediastinal 18FDG avid nodes. No mediastinal nodes were seen by EBUS. Renal biopsy identified proliferative glomerulopathy. Bone marrow aspirate and trephine biopsy indicated normal cellularity without evidence of lymphoma. Infection screen was negative for HIV I/II, TB, Hepatitis B, C, CMV, EBV and ASO. Myeloma screen was negative. Antibody titres indicated a marked increase in ANA titre between the first and second readings (< 8 weeks, Table 1). Complement (C3, C4) levels were undetectable (Fig. 1).
The patient was diagnosed with SLE complicated by proliferative glomerulonephritis with retroperitoneal fibrosis. He was given pulse dose steroids then oral steroids and IV cyclophosphamide (initially 1g x 6 cycles). Complement levels became detectable after the first cycle with normal levels after the second cycle. After the fifth treatment the patient was admitted with sepsis. Cyclophosphamide was reduced to 500mg for two more treatments. He remains well with tapering steroids and Azathioprine.
Acute renal impairment due to co-existing obstructive uropathy and proliferative glomerulonephritis has not been reported. This case represents an atypical presentation of SLE with lupus nephritis in a man with quiescent UC. Initial thoughts were for UC reactivation with imaging then suggestive for malignancy although biopsy findings were reassuring. ANA titres converted from marginal to markedly positive after symptom onset, while dsDNA titres remained within normal ranges pre- and post-treatments. C3 and C4 levels normalized with therapy and renal function noticeably improved. An association between UC and SLE is rare. Our patient had profound hypocomplementemia with a high ANA titre but did not show an autoimmune profile typically encountered in active SLE (negative dsDNA Ab titre). While a definitive association between his indolent UC and SLE cannot be confirmed, we believe that an association is likely and that co-existence of SLE with inflammatory bowel disease should be considered.