A Case of CREST Syndrome with Rapidly Progressive Liver Damage.

The Development of Autoimmune Hepatitis and Primary Biliary Cirrhosis Overlap Syndrome During the Course of Connective Tissue Diseases: Report of Three Cases and Review of the Literature

“…Our patient was followed closely during steroid and ACE inhibitor therapy, and a biochemical remission was achieved in 6 weeks, but her clinical condition deteriorated. This is contrary to previous reports describing improvements during the therapy [26,[32][33][34]36].…”

“…Our data along with a literature indicate that the combination of two diseases has been reported in only nine cases. The first three cases of scleroderma with AIH were reported in Japanese patients [32][33][34], and the same association was also described in those of European ancestry [26]. Thus, AIH can be a liver manifestation in scleroderma patients, but the pathogenesis of these two conditions is still unclear.…”

CRST Syndrome (Calcinosis Cutis, Raynaud's Phenomenon, Sclerodactyly, and Telangiectasia) Associated with Autoimmune Hepatitis.

“…Further studies are necessary to confirm the relationship between the anticentromere antibody and AIH. The combination of the two diseases has been reported only by Yabe et al (6) and Numata et al (8) (Table 3). However, unlike the present case, the case ofYabe et al had simultaneous onset of CRSTsyndrome and AIH.…”

“…Wetreated an interesting case of Raynaud's phenomenon, followed by AIH occurring during the clinical course, and resulting in gangrene of the fingertips caused by CRSTsyndrome. The presence of anti-centromere antibodies in AIHis rare, and the combination ofAIH and CRSTsyndrome has been reported only by Yabe et al (6) in the English language literature. The following is a case report concerning such a combination weobservedin a patient.…”

Liver Cirrhosis Due to Autoimmune Hepatitis Combined with Systemic Sclerosis

“…However, AIH may be due, in part, to dysfunction of both cellular and humoral immunity related to SSc, as anti-centromere antibody has been detected in up to 13% of patients with AIH. [11][12][13] There have been only two reports in the literature regarding investigations related to anti-centromere antibodies in patients with AIH, which indicated the presence of these antibodies at a low frequency of 3% in cases of AIH. 14,15 In the Japanese population, the frequency of the discrete speckled pattern of FANA (closely associated with anti-centromere antibody) is increased in AIH patients with anti-centromere antibody, supporting the close association between the AIH and SSc.…”