Life-threatening spontaneous splenic rupture with systemic lupus erythematosus: case report and literature review

Abstract

Although involvement of the reticuloendothelial system in systemic lupus erythematosus (SLE) is a well-recognised concomitant of the disease, spontaneous splenic rupture is an unusual occurrence. We observed a 54-year-old woman with SLE who had spontaneous splenic rupture during the late course of the disease and showed some changes histopathologically. The courses and the clinical characteristics of such patients are reviewed, and the diagnosis and treatment of these cases are discussed. Early evaluation for SLE patients with spontaneous splenic rupture should be considered and included in the differential diagnosis of acute abdomen, as it may affect follow-up and treatment, although the condition is rare.

Systemic lupus erythaematosus presenting as spontaneous splenic rupture

“…In another two of the analysed cases, the splenic parenchyma was actually normal 2 8. That is in contrast with our case, where the discovery of foci of vasculitis was in fact very important for the diagnosis of an immune based disorder.…”

“…On the other hand, although a variety of clotting abnormalities may exist in patients with SLE, such as in the setting of antiphospholipid syndrome, neither our patient nor any other of the reported cases showed them 2 3…”

“…In four of the patients, the SSR occurred close to the onset of SLE,2 7–9 and in two of these it was one of the first manifestations of the disease,7 9 as in the case of our patient. The other three patients had a longstanding disease course, although two were in stable condition by the time SSR supervened 2 3 6…”

“…Quite interestingly, in four of the five cases in which a histopathological examination of the removed spleen was available, no definite explanation could be established for the rupture, as vasculitis was not found and other histopathological changes were almost unremarkable 2 3 8 9. In a detailed pathological analysis of a spontaneously rupture spleen in a patient with SLE and in comparison with a traumatically ruptured spleen, the SLE spleen only showed decreased splenic corpuscles, in number and volume, and no specific type of cell proliferation was seen,10 therefore shedding no light on our understanding of the pathophysiology of this condition.…”

“…The majority of patients with SLE have mild splenomegaly as a result of lymphoid hyperplasia and some show other splenic abnormalities such as massive splenomegaly, splenic infarction or splenic atrophy 3 4 6. Nonetheless, SSR in the setting of SLE is extremely unusual, and so far only seven cases have been reported in the English literature, the first in 1971 and the last in 2011 2 3 6–9…”