Abstract
BackgroundCalciphylaxis is a syndrome consisting of vascular calcification, thrombosis, and skin necrosis. The syndrome develops often in chronic hemodialysis patients. However, there have been several case reports on calciphylaxis in patients with POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome, a systemic disease associated with plasma cell dyscrasia and upregulation of vascular endothelial growth factor (VEGF).MethodsIn 76 POEMS patients and 86 age- and gender-matched disease controls, we studied abnormal small vessel calcification by computed tomography (CT) of the soft tissues. Clinical and laboratory profiles were compared between POEMS patients with and without calciphylaxis. Histological examination was performed in six autopsy cases.ResultsSmall vessel calcification on CT was found in 17 % of POEMS patients and in none of the controls (P < 0.001). Autopsy confirmed calciphylaxis in 2 (33 %) patients. Among POEMS patients, higher disease activity, including more severe neuropathy and ascites, higher serum levels of interleukin-6, and lower serum albumin levels, was associated with the development of calciphylaxis. Serum levels of creatinine, calcium, and phosphate were not related to the presence of calciphylaxis.ConclusionsCalciphylaxis is often present in patients with POEMS syndrome. Upregulation of multiple inflammatory cytokines such as VEGF and interleukin-6 may contribute to the development of calciphylaxis, by entirely different mechanism from that in chronic dialysis. POEMS syndrome should be recognized as a potential cause of calciphylaxis.
Highlights
Calciphylaxis is a syndrome consisting of vascular calcification, thrombosis, and skin necrosis
End-stage renal disease and resulting hyperparathyroidism have been considered as major causes for calciphylaxis, which occurs in approximately 4 % of patients on chronic hemodialysis [4]
Small vessel calcification on computed tomography (CT) was observed in 17 % of POEMS patients, and in none of the controls (P = 0.00079), indicating that calciphylaxis was not caused merely by aging, but was present in patients with POEMS syndrome (Fig. 2)
Summary
Subjects We reviewed the records of 76 patients with POEMS syndrome (52 men), seen at Chiba University Hospital between 1992 and 2013. Clinical and laboratory evaluation We investigated the presence of ulcers and vessel calcification on CT, and vascular risk factors such as diabetes, hypertension, and hyperlipidemia. We compared clinical and laboratory data and treatment history between POEMS syndrome patients with probable calciphylaxis and those without it. The presence of pleural effusion/ascites on CT was evaluated and history of events suggestive of thrombosis, such as cerebral infarction, myocardial infarction, and pulmonary thromboembolism, was compared between POEMS patients with and without probable calciphylaxis. The differences in gender, prevalence of diabetes, hypertension, and hyperlipidemia between the POEMS syndrome group and the neurological disease control group were compared using Pearson’s chisquared test. The differences in age and serum concentration of creatinine were compared using the Mann–Whitney U test and the differences in the prevalence of skin ulcers and calcification seen on CT were compared using Fisher’s exact test. The differences in the presence of pleural effusion and history of steroid therapy were compared using Pearson’s chi-squared test and the differences in age, disease duration, and laboratory values were compared using the Mann–Whitney U test
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