Progranulin autoantibodies in systemic sclerosis and autoimmune connective tissue disorders: A preliminary study.

Abstract

OBJECTIVE:

The present study aimed to investigate progranulin autoantibodies in systemic sclerosis and autoimmune connective tissue disorders. Progranulin is a physiologic tumor necrosis factor (TNF) antagonist. Progranulin antibodies decrease progranulin levels.

METHODS:

Serum samples of 123 patients with systemic sclerosis and various autoimmune connective tissue disorders (Sjoegren's syndrome [SjS], mixed connective tissue disorder, polymyositis [PM] and dermatomyositis [DM], antiphospholipid syndrome [APLS], and undifferentiated connective tissue disease [UCTD]) were tested for progranulin antibodies using enzyme-linked immunosorbent assay.

RESULTS:

Progranulin antibodies were found in 34 of 123 (27.6%) patients at least once during their disease. In detail, 2 of 8 (25%) patients with limited cutaneous systemic sclerosis, 10 of 31 (32.3%) patients with diffuse cutaneous systemic sclerosis, 9 of 22 (40.9%) patients with SjS, 1 of 3 (33.33%) patients with mixed connective tissue disease, 4 of 33 (12.1%) patients with DM or PM, 6 of 15 (40%) patients with APLS, and 2 of 11 (18.2%) patients with UCTD were positive for progranulin antibodies during the course of disease.

CONCLUSIONS:

Progranulin antibodies are frequently present in patients with systemic sclerosis and other autoimmune connective tissue disorders. Despite the lack of specificity for a given autoimmune disease, progranulin antibodies might not only indicate a potential subtype but also play a pathogenic role in patients with autoimmune connective disorders. Given the important role of TNF-α in inflammatory processes in autoimmune connective tissue disorders, progranulin antibodies might support the proinflammatory environment by neutralizing the TNF blocker progranulin.

• Authors: Philipp Klemm, Gunter Assmann, Klaus-Dieter Preuss, Natalie Fadle, Evi Regitz, Thierry Martin, Michael Pfreundschuh, Lorenz Thurner
• Journal: Immunity, inflammation and disease (Immun Inflamm Dis) Vol. 7 Issue 4 Pg. 271-275 (12 2019) ISSN: 2050-4527 [Electronic] England
• PMID: 31502765 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: © 2019 The Authors. Immunity, Inflammation and Disease published by John Wiley & Sons Ltd.

Chemical References

• Autoantibodies
• GRN protein, human
• Progranulins

Topics

• Aged
• Autoantibodies (blood, immunology)
• Autoimmune Diseases (blood, immunology)
• Enzyme-Linked Immunosorbent Assay
• Female
• Humans
• Male
• Middle Aged
• Progranulins (blood, immunology)
• Scleroderma, Systemic (blood, immunology)