What are the tracheal and laryngeal manifestations of Mixed Connective Tissue Disease (MCTD)?

From the Guidelines

Tracheal and laryngeal manifestations of mixed connective tissue disease (MCTD) are a significant concern, as they can lead to severe morbidity and mortality, and thus, early detection and aggressive treatment are crucial to improve quality of life and prevent complications. MCTD can affect the trachea and larynx, causing symptoms like hoarseness, stridor, and breathing difficulties due to inflammation, fibrosis, and cricoarytenoid joint involvement 1. Patients may develop laryngeal edema, vocal cord dysfunction, subglottic stenosis, or tracheal narrowing, which can significantly impact quality of life and may become life-threatening if not properly addressed.

Key Considerations

• Treatment typically involves systemic immunosuppression with corticosteroids (prednisone 0.5-1 mg/kg/day initially, then tapered) combined with steroid-sparing agents like methotrexate (15-25 mg weekly), mycophenolate mofetil (1-3 g daily), or azathioprine (1-2.5 mg/kg/day) to reduce inflammation and prevent progression.
• For acute airway compromise, immediate intervention with high-dose IV methylprednisolone (1 g/day for 3 days) may be necessary to rapidly reduce inflammation and prevent airway obstruction.
• Local treatments include inhaled corticosteroids, vocal therapy, and in severe cases, surgical interventions such as tracheostomy or tracheal dilation to manage symptoms and prevent complications.
• Regular monitoring with laryngoscopy and pulmonary function tests is essential for early detection of airway involvement and to assess the effectiveness of treatment.

Multidisciplinary Management

• Multidisciplinary management involving rheumatologists, otolaryngologists, and pulmonologists provides the best outcomes, as these manifestations can significantly impact quality of life and may become life-threatening if not properly addressed.
• A team-based approach allows for comprehensive care, including medical and surgical interventions, as well as rehabilitation and supportive care to improve quality of life and reduce morbidity and mortality.
• According to the most recent study, early detection of interstitial lung disease in rheumatic diseases, including MCTD, is crucial for improving outcomes, and treatment during the acute inflammatory phase is associated with a good prognosis 1.

From the Research

Tracheal and Laryngeal Manifestations of Mixed Connective Tissue Disease

There is limited information available on the tracheal and laryngeal manifestations of mixed connective tissue disease (MCTD). However, the following points can be noted:

• Respiratory involvement is observed in 20% to 80% of patients with MCTD, with major respiratory manifestations including interstitial pneumonitis and fibrosis, pleural effusion, pleurisy, and pulmonary hypertension 2.
• Pulmonary vascular manifestations of MCTD include interstitial lung disease, pleural disease, and alveolar hemorrhage 3.
• While there is no specific information on tracheal and laryngeal manifestations, the disease can cause a range of respiratory symptoms due to its impact on the lungs and pulmonary vasculature.
• The clinical features of MCTD can be complex and varied, and may include symptoms such as esophageal dysfunction, muscle weakness, and fingers with a sausage-like appearance, in addition to respiratory symptoms 4, 5.
• Treatment for MCTD typically involves individualized therapy to address the specific organ involved and the severity of underlying disease activity, and may include corticosteroids, antimalarials, methotrexate, cytotoxics, and vasodilators 6.