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Henoch-Schönlein purpura (HSP) is a systemic small vessel vasculitis predominantly affecting children and, less commonly, adults. Classical HSP includes a tetrad of palpable purpura, arthritis, abdominal pain, and glomerulonephritis. Adults may present with any two of the four criteria in the tetrad (87% sensitivity and specificity). Gastrointestinal disease has been recorded in up to 82% of adult patients in one series1 and is usually self limiting with colicky abdominal pain, but may progress to ischaemic bowel perforation.2
We present the case of a 63 year old man with IgA vasculitis, probably HSP, confounded by undiagnosed hepatitis C related cirrhosis.
He was admitted with a two week history of dyspnoea, malaise, cough, fevers, and chills, myalgias, one day of a non-blanching erythematous rash on his legs, and an ileus. His hepatitis C antibody was positive; table 1 shows the results of other laboratory studies. Cultures of cerebrospinal fluid, blood, and urine were negative. A colonoscopy was non-diagnostic.
Leucocytoclastic vasculitis was confirmed by skin biopsy, and direct immunofluorescence staining was positive for IgA deposits consistent with HSP (fig 1).
Treatment with high dose (1 mg/kg/day) intravenous corticosteroids was started. A computed tomographic (CT) scan of the abdomen showed portal hypertension, a small cirrhotic liver, small spleen, omental and perisplenic varices, an atrophic pancreas, and modest ascites. The purpuric lesions and ileus improved; however, on day 4 he became tachycardic and developed a tender abdomen. A second CT scan showed massive ascites, a partial superior mesenteric vein thrombosis, thickening, and focal and nodular irregularities throughout the small bowel (probable ischaemia), and pneumoperitoneum. Blood cultures disclosed septicaemia with Bacteroides fragilis. His clinical course rapidly deteriorated and he died on day 8.
There are two previous case reports of the association between HSP and hepatitis C.3,4 The diagnosis of HSP in our patient is most likely, given palpable purpura, haematuria, abdominal pain, and a skin biopsy demonstrating IgA complexes (fig 1). However, the possibility of hepatitis C associated IgA/IgM mixed cryoglobulinaemia cannot be ruled out despite a negative cryoglobulin screen5 on two occasions. In this patient an IgA mediated vasculitis may have been the nidus for thrombus formation and abdominal catastrophe.
The role of liver cirrhosis in the development of HSP is intriguing. Patients with cirrhosis may develop HSP as a consequence of defective liver metabolism of IgA circulating immune complexes (CICs), resulting in tissue deposition, although this is known to occur without overt vasculitis.6
Adult and paediatric HSP differ in the incidence and severity of renal involvement, with nephropathy and progression to renal insufficiency being greater in adult HSP,7 which is associated with a poor outcome.8 Gastrointestinal manifestations vary widely and include abdominal pain, nausea/vomiting, intestinal haemorrhage and, rarely, perforation.
There have been no large clinical trials in adults with complicated HSP. Corticosteroids used in a series of children have been shown to relieve symptoms,9 but fail to deal prospectively with the prevention of abdominal complications. Adults respond favourably to corticosteroids and may be managed with short courses of treatment,10 but corticosteroids may also mask severe abdominal catastrophe.
Several important points can be learnt from this case report:
Although nephritis is the most important long term prognostic factor in HSP, in the short term, gastrointestinal disease can lead to death despite early therapeutic intervention
Liver cirrhosis secondary to hepatitis C may precipitate development of HSP or mixed cryoglobulinaemic vasculitis through the defective metabolism of CICs
Given the increasing incidence of hepatitis C related liver disease world wide, the association of these diagnoses and their clinical implications should be considered more often.
We thank Drs Karen Stout, Brett Sheppard, Amy Howard, and Sandhya Venugopal for their participation in, and discussions about, this case.
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