Pulmonary Literature Review and Complex Case Presentations Post Graduate Course-Complex Cases Interstitial Lung Disease CHAN

Complex Cases Interstitial Lung Disease CHAN

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The presentation details a complex case of interstitial lung disease (ILD) in a 62-year-old African American woman, originally referred for dyspnea (shortness of breath). She has a history of asthmatic bronchitis, chronic cough, and gastroesophageal reflux disease (GERD). Initial treatments for suspected asthma provided no relief, and further testing revealed elevated IgE levels and signs of inflammation (elevated ESR and CRP). A biopsy indicated Sjogren’s Syndrome with non-specific interstitial pneumonia (NSIP).<br /><br />The diagnosis shifted to anti-synthetase syndrome, characterized by symptoms including muscle weakness and distinctive antibodies (anti-PL12), which overlap with secondary Sjogren's Syndrome. This condition is an autoimmune disorder that often involves myositis, arthritis, and ILD, with significant morbidity and mortality rates. The presentation revises the treatment plan, considering medications like corticosteroids, mycophenolate mofetil, and potentially others like calcineurin inhibitors or rituximab depending on the specifics.<br /><br />Recently published guidelines by the ACR/ACCP for treating SARD-related ILD highlight the importance of monitoring and screening using PFTs and HRCT, with recommendations for treatment focused on controlling disease progression. Mycophenolate is favored as a first-line treatment for various systemic autoimmune diseases related to ILD, while specific treatments like calcineurin and JAK inhibitors are noted for idiopathic inflammatory myositis-related ILD.<br /><br />The presentation emphasizes the evolving nature of diagnosing and managing ILD, underscoring the necessity of personalized treatment plans guided by recent guidelines and individual patient profiles. It concludes with an invitation to further educational opportunities to keep practitioners informed about advances in managing complex respiratory conditions.

Keywords

interstitial lung disease

anti-synthetase syndrome

Sjogren's Syndrome

non-specific interstitial pneumonia

autoimmune disorder

mycophenolate mofetil

corticosteroids

PFTs

HRCT

treatment guidelines