From the Guidelines
For patients with mixed connective tissue disorder (MCTD) and interstitial lung disease (ILD), we conditionally recommend tocilizumab as a first-line ILD treatment option, as suggested by the 2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) guideline 1.
Treatment Approach
The treatment of MCTD-ILD typically involves immunosuppressive therapy, with the goal of halting inflammatory damage to lung tissue and preventing irreversible fibrosis.
- The use of glucocorticoids, such as prednisone, may be considered as a first-line treatment for MCTD-ILD, except in cases of systemic sclerosis (SSc)-ILD, where glucocorticoids are strongly recommended against as a first-line treatment 1.
- For maintenance therapy or as steroid-sparing agents, immunosuppressants like mycophenolate mofetil, azathioprine, or cyclophosphamide may be used.
- In severe or rapidly progressive cases, rituximab may be considered as a treatment option.
Monitoring and Supportive Care
Regular monitoring with pulmonary function tests and high-resolution CT scans every 3-6 months is essential to assess treatment response.
- Supportive care includes oxygen therapy for hypoxemia, pulmonary rehabilitation, and vaccination against pneumococcal disease and influenza.
Treatment of Progressive Disease
For people with MCTD-ILD progression despite first ILD treatment, we conditionally recommend mycophenolate, rituximab, cyclophosphamide, and nintedanib as treatment options, as well as tocilizumab for those with SSc-ILD, MCTD-ILD, or RA-ILD progression despite first ILD treatment 1.
From the Research
Treatment of Mixed Connective Tissue Disorder in ILD
- The treatment of mixed connective tissue disorder (MCTD) in interstitial lung disease (ILD) often involves immunosuppressive therapy, as indicated by studies 2, 3, 4, 5, 6.
- Corticosteroids (CS) and cyclophosphamide (CPH) are commonly used in the treatment of MCTD with ILD, with CS being used alone or in combination with CPH 2.
- Hydroxychloroquine (HCQ) and glucocorticoids (GC) are also used in the treatment of MCTD, with HCQ being shown to reduce the frequency of ILD and pulmonary arterial hypertension (PAH) 3.
- Disease-modifying antirheumatic drugs (DMARDs) and immunosuppressants (IS) are used in patients with musculoskeletal involvement, ILD, and PAH, as well as in patients who do not respond to HCQ and GC 3.
- Rituximab (RTX) has been shown to stabilize pulmonary functional tests (PFTs) in patients with ILD secondary to MCTD, although the changes were not significant 6.
- Immunosuppressive therapy, including corticosteroid, intravenous immunoglobulin, and cyclosporine A, has been shown to ameliorate myositis, interstitial lung disease, and inflammation in patients with MCTD complicated by microscopic polyangiitis (MPA) 5.
Treatment Outcomes
- The treatment outcomes for MCTD with ILD vary depending on the severity of the disease and the response to treatment, with some patients showing significant improvement and others experiencing stabilization of their condition 2, 3, 4, 5, 6.
- The use of immunosuppressive therapy has been shown to improve pulmonary function and reduce the frequency of ILD and PAH in patients with MCTD 2, 3.
- However, the treatment of MCTD with ILD is often challenging, and the prognosis can be poor, especially in patients with severe lung complications 4, 5.