Autoimmune Neutropenia Due to Na1 Specific Antibodies in Primary Biliary Cirrhosis

Autoimmune neutropenia due to antineutrophil antibodies in a patient with primary sclerosing cholangitis

“…In fact, the reported PBC patient who developed AIN experienced neutropenic episodes three times, and continuous treatment with prednisone and cyclosporine A was required [11]. By contrast, the neutropenia in the present patient recovered without the use of these agents and has not occurred subsequently.…”

“…4 The findings of bone marrow aspiration, indicating no abnormalities, with no dysplasia and no abnormal differentiation of blood cells autoimmune pathogenesis was suggested to explain the neutropenic episodes in both patients, the presence of antineutrophil antibody was not demonstrated, and thus it is not acceptable for these AIH cases to be categorized as AIN. By contrast, in the patient with PBC [11] who developed neutropenia several times during the clinical course, antineutrophil antibody in the patient's sera was clearly detected by GIFT, and the diagnosis of AIN was well established. Yet the occurrence of AIN in patients with PSC has not yet been demonstrated in the previous literature.…”

“…AIN has been reported to be linked with various autoimmune diseases, including autoimmune liver diseases, such as PBC [11]. Recently two patients with autoimmune hepatitis (AIH) who developed agranulocytosis, defined as ANC less than 100/ll, with a flare of liver injury were reported [13,14].…”

“…While primary AIN, not associated with other detectable pathological conditions, is mainly observed in infancy and in early childhood [3][4][5], secondary AIN is usually associated with autoimmune diseases, infections, malignancy, and drugs, and is more commonly seen in adults [1,2]. Several autoimmune diseases have been demonstrated to be associated with secondary AIN [6], including systemic lupus erythematosus (SLE) [7], rheumatoid arthritis (RA; Felty's syndrome) [8], Sjögren syndrome [9], systemic sclerosis [10], and primary biliary cirrhosis (PBC) [11]. Herein we present the first report of a patient with primary sclerosing cholangitis (PSC) who developed AIN.…”

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“…Although AIN is most likely to be secondary to systemic autoimmune diseases [23-27], it is also seen in other clinical situations. Examples are infectious diseases [28-30], solid or haematological neoplasms [31,32], neurological diseases [33], bone marrow or stem cell transplants [34,35], kidney transplants [36,37], and use of certain drugs [38-40] (Table 3).…”