Successful Treatment of Autoimmune Pancreatitis Complicated with Autoimmune Thrombocytopenia and Interstitial Pneumonia by Prednisolone

Abstract

We report the second patient diagnosed with autoimmune pancreatitis complicated with autoimmune thrombocytopenia and interstitial pneumonia. The patient was treated with prednisolone and responded favorably. We demonstrated that anti-platelet (PLT) antibody of the patient was IgG4 and that it may react with HLA, not specific antigen, on both pancreas and PLT.

Autoimmune Pancreatitis Associated with Various Extrapancreatic Lesions during a Long-term Clinical Course Successfully Treated with Azathioprine and Corticosteroid Maintenance Therapy

“…We diagnosed the salivary gland lesions as IgG4-related sclerosing sialadenitis. (17)(18)(19)(20). In the present case, thrombocytopenia was not observed, and a skin biopsy revealed perivascular leukocytic infiltration.…”

Overlap of IgG4-related Disease and Primary Biliary Cirrhosis Complicated with Autoimmune Thrombocytopenia

“…Moreover, serum IgG4 levels fluctuate along with the state of IgG4-RD. A few cases have been reported regarding the association between IgG4-RD, especially with autoimmune pancreatitis, and autoimmune thrombocytopenia (21)(22)(23)(24). In the present case, no significant increase in the number of IgG4-positive cells or decrease in megakaryocytes in the bone marrow was observed.…”

“…Chan et al (25) reported that the most common subclass of anti-IIb/IIIa antibodies in ITP patients was IgG1. Murase et al (22) reported an IgG subclass of a PLT antibody to be potentially IgG4. While this suggested a role of IgG4 as an anti-PLT antibody in IgG4-RD patients, this finding is controversial because IgG4-RD is rarely complicated with thrombocytopenia.…”

A Case of IgG4-related Sclerosing Cholangitis Overlapped with Primary Biliary Cirrhosis

“…ITP is associated with PBC; however, an association between IgG4-related diseases and PBC has not been reported. Some cases of thrombocytopenia associated with AIP have been reported (13)(14)(15)(16), which indicates an association between thrombocytopenia with IgG4-related diseases. In the present case, the patient had a 3-year history of ITP, and ITP was the clinical sign for the diagnosis of PBC.…”