Intrathoracic manifestations in mixed connective tissue disease

Area of Science:

• Pulmonology
• Rheumatology
• Internal Medicine

Background:

• Mixed connective tissue disease (MCTD) is a systemic autoimmune disorder.
• Pleuropulmonary manifestations are frequently reported in MCTD literature.

Purpose of the Study:

• To determine the frequency and characteristics of pleuropulmonary involvement in adult patients with MCTD.
• To evaluate the efficacy of treatments for pleuropulmonary complications in MCTD.

Main Methods:

• Retrospective analysis of 81 adult patients diagnosed with MCTD between 1973 and 1977.
• Review of clinical data, chest roentgenograms, pulmonary function tests, and esophagograms.
• Assessment of treatment responses to systemic corticosteroids and nonsteroidal anti-inflammatory drugs.

Main Results:

• 25% of patients (20/81) exhibited pleuropulmonary involvement.
• Common symptoms included dyspnea (16%), chest pain (7%), and cough (5%).
• Radiographic findings revealed interstitial processes (19%), pleural effusion (6%), and pneumonic infiltrates (4%).
• Pulmonary function abnormalities were observed in 69% of tested patients.
• Esophagographic abnormalities were present in 53% of tested patients.
• Corticosteroids showed benefit in 2/8 patients; NSAIDs resolved symptoms in 4/7 patients.
• Six patients died within the 5-year follow-up period.

Conclusions:

• Pleuropulmonary involvement is a significant feature of MCTD.
• Pulmonary function and radiographic abnormalities are common.
• Treatment efficacy varied, with NSAIDs showing some promise.
• Further research is needed to optimize management strategies for MCTD-related lung disease.