Association of primary biliary cirrhosis with idiopathic thrombocytopenic purpura

Abstract

Although both primary biliary cirrhosis (PBC) and i d i o p a t h i c t h r o m b o c y t o p e n i c p u r p u ra ( I T P ) a r e a u t o i m m u n e d i s e a s e s , t h e a s s o c i a t i o n o f t h e 2 diseases is rare. Here, we report a case of ITP that developed during the follow-up of PBC in a 74-yearold man. The patient had been diagnosed with PBC 12 years previously, and had received treatment with ursodeoxycholic acid. The platelet count decreased from approximately 60 × 10 9 /L to 8 × 10 9 /L, and the association of decompensated liver cirrhosis (PBC) with ITP was diagnosed. Steroid and immune gamma globulin therapy were successful in increasing the platelet count. Interestingly, human leukocyte antigen genotyping detected the alleles DQB1*0601 and DRB1*0803, which are related to both PBC and ITP in Japanese patients. This case suggests common immunogenetic factors might be involved in the development of PBC and ITP.

Shared Autoimmunity: A Case Series of 56 Patients with Immune Thrombocytopenia (ITP) Associated with Other Autoimmune Disorders

“…The remaining AD associated with ITP appeared less frequently in our patients, between 1 and 4 times ( Table 1). Some of these associations have been previously described, such as those with psoriasis [25], autoimmune neutropenia [26] [27], atrophic gastritis with pernicious anemia [28], autoimmune hemolytic anemia [5] [9] [14] [26], ulcerative colitis [29], multiple sclerosis [14], lichen planus [2], pyoderma gangrenosum [27], primary biliary cirrhosis [30], celiac disease [31] [32], myastenia gravis [33], rheumatoid arthritis [34] [35], vitiligo [36], antiphospholipid syndrome [9], autoimmune hepatitis [37], and sarcoidosis [38]. However, we did not find any studies reporting the association of ITP with thromboangiitis obliterans.…”