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Pulmonary fibrosis as the sole manifestation of anti-Ku antibody positivity in the absence of myositis: A case report.

Respir Med Case Rep

January 2025

Department of Rheumatology of Lucania - UOSD of Rheumatology, "Madonna delle Grazie" Hospital, Matera, Italy.

Background: Anti-Ku antibodies are autoantibodies directed against the Ku protein complex involved in DNA repair. They are typically associated with overlap syndromes featuring polymyositis and systemic sclerosis. Isolated pulmonary involvement without myositis is exceedingly rare.

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While durable antibody responses from long-lived plasma cell (LLPC) populations are important for protection against pathogens, LLPC may be harmful if they produce antibodies against self-proteins or self-nuclear antigens as occurs in autoimmune diseases such as systemic lupus erythematosus (SLE). Thus, the elimination of autoreactive LLPC may improve the treatment of antibody-driven autoimmune diseases. However, LLPC remain a challenging therapeutic target.

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: To explore the potential association between positive ANA serology and all-cause mortality in a large cohort of patients, including those with and without rheumatological conditions and other immune-related diseases. : A retrospective cohort study analyzed all-cause mortality among 205,862 patients from Clalit Health Services (CHS), Israel's largest health maintenance organization (HMO). We compared patients aged 18 and older with positive ANA serology (n = 102,931) to an equal number of ANA-negative controls (n = 102,931).

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Background: Autoimmune hepatitis is a chronic liver disease marked by immune-mediated inflammation, necrosis, and the potential to progress to cirrhosis if not treated. This case report presents a rare and atypical presentation of autoimmune hepatitis in a Nigerian adolescent girl, highlighting diagnostic challenges in resource-limited settings. The case is unique owing to the absence of jaundice, a common symptom of liver dysfunction, and features such as delayed menarche and bilateral leg swelling.

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Antinuclear antibodies (ANA) are often found in ILD; whether ANA is associated with radiographic progression of quantitive interstital lung changes is unknown. We performed longitudinal analyses of adults in the Multi-Ethnic Study of Atherosclerosis using linear mixed effects models with random intercept and slope to evaluate whether baseline ANA was associated with change in the amount of lung with high attenuation areas on CT (HAAs, percentage of imaged lung with -600 to -250 HU). In 6,638 subjects with 17,293 CT scans over 18 years, 741 (11%) were ANA positive.

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