Anticyclic citrullinated peptide antibodies in patients with mixed connective tissue disease.

The clinical significance of anticyclic citrullinated peptide (CCP) antibodies in patients with mixed connective tissue disease (MCTD) was assessed. Altogether, 86 sera from MCTD patients, 96 from rheumatoid arthritis (RA) patients, 42 from systemic lupus erythematosus (SLE) patients, 23 from systemic sclerosis (SSc) patients, 21 from polymyositis/dermatomyositis (PM/DM) patients, and 17 from those with Sjögren's syndrome (SjS) were tested for anti-CCP antibodies using an enzyme-lined immunosorbent assay. Among the 96 RA patients, anti-CCP antibodies were detected in 85%, with the frequency being significantly higher than in MCTD, SLE, SSc, PM/DM, and SjS patients (9%, 14%, 13%, 14%, and 18%, respectively; P < 0.001). Among eight MCTD patients who fulfilled the diagnostic criteria for RA, only 50% had anti-CCP antibodies, and the prevalence was significantly lower than for all RA patients (p < 0.01). All eight patients who fulfilled the criteria for RA had overlap of SLE and SSc, except one patient, whereas the four anti-CCP-positive patients who did not fulfill the criteria for RA had SjS without overlapping features of SLE and SSc; moreover, most of their antibody titers were low. These results suggested that anti-CCP antibodies are associated with RA in MCTD patients, but careful diagnosis of RA is required if patients with low titers of anti-CCP antibodies lack overlapping SLE and SSc.

Acute acalculous cholecystitis induced by mesenteric inflammatory veno-occlusive disease (MIVOD) in systemic lupus erythematosus.

A 43-year-old woman with systemic lupus erythematosus (SLE) was treated for lupus pleurisy. During the course of her illness, she abruptly suffered severe right hypochondriac pain and high-grade fever. Abdominal ultrasonography revealed a thickening of the gallbladder wall without cholelithiasis, and she was diagnosed with acute acalculous cholecystitis (AAC). Laparoscopic cholecystostomy was performed. Pathological examination revealed lymphocytic venulitis without arteritis. Antiphospholipid antibodies were not demonstrated during the course of illness. From these findings, the cause of AAC was revealed as a mesenteric inflammatory veno-occlusive disease (MIVOD), which is a novel venopathy mainly affecting the mesenteric vein and/or its branches, causing serious ischemic complications. MIVOD should be considered as a possible cause of AAC.