CHEST Guidelines-A-66-Year-Old-Man-With-Mediastinal-Mass-and-Dyspne

A-66-Year-Old-Man-With-Mediastinal-Mass-and-Dyspne

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The document discusses the case of a 66-year-old man experiencing dyspnea and a mediastinal mass, confirmed as type A thymoma. He had a history of recurrent respiratory and ear infections and showed clinical signs of lichen planus. Chest imaging revealed a mass in the right anterior mediastinum and ground-glass lung nodules. Pulmonary function tests indicated restrictive lung disease. A lung biopsy showed granulomatous and lymphocytic interstitial lung disease (GL-ILD), ruling out other conditions like sarcoidosis and infections. The diagnosis was Good syndrome (GS), characterized by thymoma, immunodeficiency, hypogammaglobulinemia, and reduced B-cell count.<br /><br />Good syndrome is a rare immunodeficiency disorder associated with thymoma, mainly affecting adults. It's characterized by recurrent infections due to B-cell deficiencies, significant opportunistic infections, and bronchiectasis. GS differs from similar disorders like Common Variable Immunodeficiency (CVID) by specific immune deficiencies and older age onset. GL-ILD frequently appears in such patients, necessitating thorough radiological and histopathological evaluation to rule out infections and confirm the disease, respectively.<br /><br />The patient received thymectomy and intravenous immunoglobulin therapy (IVIG) for immune support. Although thymectomy does not reverse immunodeficiencies, it potentially reduces metastasis risk. The patient's pulmonary condition is under close observation without active treatment. Current management options for GL-ILD include corticosteroids and immunosuppressive therapies, although there are no standardized treatment protocols. The case underscores the importance of early diagnosis and treatment of noninfectious complications in immunodeficiency disorders to enhance outcomes and survival rates. Further research is necessary to explore the underlying mechanisms and improve therapies for such rare disorders.

Keywords

Good syndrome

thymoma

immunodeficiency

hypogammaglobulinemia

GL-ILD

thymectomy

intravenous immunoglobulin therapy

recurrent infections

restrictive lung disease

lichen planus