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Pulmonology / Respiratory

Pleuroparenchymal Fibroelastosis (PPFE)

ICD-10 Code
J84.117

Clinical Criteria for Pleuroparenchymal Fibroelastosis (PPFE).

Clinical Presentation & Protocol

Patient Usually Complains Of

Patient presents with progressive exertional dyspnea and non-productive cough. History significant for recurrent respiratory infections, weight loss, and thoracic discomfort. Note: Evaluate for characteristic "flat-chested" body habitus and history of autoimmune conditions or prior lung transplantation.

Clinical Examination Findings

Physical exam reveals a thin, asthenic body habitus with flattened thoracic cage. Auscultation demonstrates bilateral apical inspiratory crackles. Chest wall inspection may show decreased expansion. Evaluate for signs of chronic hypoxemia, including digital clubbing or cyanosis.

Treatment Protocol

Management plan includes supplemental oxygen for hypoxemia, pulmonary rehabilitation, and nutritional support to address cachexia. Consider immunosuppressive therapy if secondary to autoimmune disease. Monitor for complications including pneumothorax and secondary pulmonary hypertension. Evaluate for lung transplant candidacy.

Detailed clinical guide coming soon.