CHEST Guidelines-A-61-Year-Old-Woman-With-Insidious-Dyspnea-and-Dif

A-61-Year-Old-Woman-With-Insidious-Dyspnea-and-Dif

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The document describes the case of a 61-year-old woman with insidious dyspnea and diffuse cystic lung disease, ultimately diagnosed with amyloid-associated cystic lung disease. The patient, with a history of 10 pack-year smoking but no significant comorbidities or occupational exposures, presented with dyspnea on exertion and dry cough persisting for 1.5 years. Initial clinical evaluations, including physical examination and high-resolution CT (HRCT), revealed diffuse, thin-walled, irregularly shaped lung cysts with multiple calcified nodules and no discernible ground-glass opacities or adenopathy.<br /><br />Despite normal routine blood tests, negative HIV testing, and no indications of connective tissue disease, pulmonary function tests indicated an abnormal residual volume and reduced diffusing capacity for carbon monoxide. However, bronchoscopy with bronchoalveolar lavage (BAL) showed no significant findings. A lung biopsy via video-assisted thoracoscopic surgery confirmed nodular amyloid deposition, revealing findings typical of pulmonary amyloidosis using Congo red staining demonstrating apple-green birefringence.<br /><br />Amyloidosis is a rare disorder involving abnormal fibrillary protein deposition. Pulmonary amyloidosis can manifest as nodular or cystic lung disease and is often asymptomatic, detected incidentally. In this case, cyst formation may relate to airway narrowing or vascular amyloid deposits causing alveolar wall destruction.<br /><br />Treatment decisions favored a conservative approach due to the patient's stable condition and preserved lung function. Over a two-year follow-up, the patient maintained stable dyspnea and normal function without systemic amyloid involvement. Suggested management for localized pulmonary amyloidosis includes monitoring, but more severe cases may warrant chemotherapy or lung transplantation.<br /><br />The document highlights the importance of considering nodular pulmonary amyloidosis in differential diagnoses for diffuse cystic lung diseases and underscores the necessity of lung biopsy for definitive diagnosis.

Keywords

amyloid-associated cystic lung disease

pulmonary amyloidosis

nodular amyloid deposition

dyspnea

cystic lung disease

lung biopsy

high-resolution CT

Congo red staining

pulmonary function tests

conservative treatment