CHEST Guidelines-Evolution-of-Pulmonary-Involvement-in-Idiopathic-M

Evolution-of-Pulmonary-Involvement-in-Idiopathic-M

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This research provides insights into the pulmonary manifestations of idiopathic multicentric Castleman disease-not otherwise specified (iMCD-NOS). Prior studies on Castleman disease have not adhered to the Castleman Disease Collaborative Network’s classification criteria, and thus, little is known about the pulmonary involvement in iMCD-NOS, a clinical subset of MCD.<br /><br />The study enrolled 162 patients with iMCD-NOS, identifying 58 (35.8%) with pulmonary involvement. The pulmonary abnormalities included nodules (96.6%), cysts (65.5%), and consolidation (22.4%). The study established three stages of pulmonary manifestation: nodules, cysts, and consolidation, related to the duration of disease symptoms, with nodules appearing in earlier stages.<br /><br />Chest CT scans proved crucial in detecting abnormalities early, even in patients lacking respiratory symptoms. Crucially, the lesions evolved uniquely over time — from nodules to cysts or consolidation. After treatment, lesions showed improvement, except for cysts, demonstrating that effective systemic treatment can reverse other pulmonary manifestations.<br /><br />Distinct clinical phenotypes within iMCD include hyaline vascular and plasmacytic subtypes, with hyaline vascular patients only presenting nodules; this subtype did not progress, unlike others, which often did, particularly forming cysts or evolving in consolidation stages.<br /><br />This study's significance lies in mapping the progression of iMCD-NOS pulmonary involvement and underscoring the value of early diagnosis and systemic treatment intervention. However, limitations include its retrospective nature, small sample size, and single-center setting, suggesting the need for further research across diverse populations. The pulmonary manifestations' defined evolution stages could aid clinicians in assessing disease severity and tailoring timely interventions.

Keywords

idiopathic multicentric Castleman disease

iMCD-NOS

pulmonary manifestations

Castleman Disease Collaborative Network

chest CT scans

nodules

cysts

consolidation

hyaline vascular subtype

systemic treatment