Fatal scleroderma renal crisis caused by gastrointestinal bleeding in a patient with scleroderma, Sjögren's syndrome and primary biliary cirrhosis overlap

Abstract

A 57-year-old female patient presented with a long history of overlapping autoimmune disease, including limited cutaneous systemic sclerosis, Sjögren's syndrome and primary biliary cirrhosis. Another unusual finding was that the mild skin involvement (limited cutaneous systemic sclerosis, subcutaneous calcinosis) was combined with serious internal organ involvement, including honeycombing and finally scleroderma renal crisis. The most important finding was, that two decades later she developed severe telangiectasia of the gastrointestinal and urinary tract. Furthermore, a specific type of vascular malformation, i.e. gastric watermelon stomach was also found. The chronic gastrointestinal bleeding primarily derived from a watermelon stomach caused protein overload, which provoked the onset of the scleroderma renal crisis that finally led to the patient's death.

Watermelon stomach in systemic sclerosis: its incidence and management

“…Interestingly, we observed that watermelon stomach was an early manifestation in SSc, as it more often occurred in earlier phase of the disease (<5 years after SSc diagnosis). Moreover, progression of watermelon stomach and activity of SSc have rarely been reported previously; the manifestations of watermelon stomach and the activity of SSc did not seem to parallel each other (62.5%) 18–40 . Our findings underline that both watermelon stomach and SSc progressed simultaneously in only 33.3% of patients; in other patients, watermelon stomach occurred when other manifestations of SSc were quiescent.…”

“…Various clinical presentations may reveal watermelon stomach. In the literature analysis of 77 SSc patients, the initial presentation of watermelon stomach was more commonly recurrent anaemia related to occult gastrointestinal bleeding (90.9% of cases) 18–40 . In our experience, we found that 86.7% of patients presented with iron‐deficiency anaemia.…”

“… 12 have, in fact, noted that 62.2% of patients had autoimmune diseases. Such an association has subsequently been reported rarely in SSc patients 18–41 . Our literature search using the Medline database (1966–2008) indeed identified 77 cases of watermelon stomach in SSc patients; these 77 cases have been reported in 22 articles and one abstract 18–40 .…”

“…The Medline review of 77 cases shows that watermelon stomach was more often diagnosed in patients who were already established as having SSc (80.8%); the median time interval between SSc diagnoses to watermelon stomach onset was 18 months. Both watermelon stomach and SSc were diagnosed concomitantly in other patients (8.2%); in the remaining patients, watermelon stomach preceded the SSc onset (10.9%) 18–40 . This latter finding indicates that because systemic manifestations may precede cutaneous impairment in SSc, when watermelon stomach is diagnosed, an evaluation for SSc (including clinical examination, antinuclear antibodies, nailfold capillaroscopy) should be therefore carried out systematically.…”

Scleroderma renal crisis in a patient with anticentromere antibody-positive limited cutaneous systemic sclerosis

“…3 Several studies of limited cutaneous scleroderma have described that renal crisis occurred in the patients who had such autoantibodies as anti-RNA polymerase III antibody, anti-Th/To antibodies, or anti-U1/ U3 RNP antibodies. [8][9][10][11][12][13] Katrib et al have reported three patients with anticentromere antibody-positive limited cutaneous systemic sclerosis who developed renal failure, but these patients showed normal blood pressure, active urinary sediments, positive antineutrophil cytoplasmic antibodies, and crescentic glomerulonephritis, 12 indicating that these cases were not classical scleroderma renal crisis. A case has been reported of a patient with scleroderma and primary biliary cirrhosis overlap who had fatal scleroderma renal crisis, but she had massive gastrointestinal bleeding and had no anticentromere antibody, 13 suggesting that this case was also not typical scleroderma renal crisis.…”