What is the management approach for Mixed Connective Tissue Disease (MCTD)?

From the Guidelines

The management of Mixed Connective Tissue Disease (MCTD) should prioritize mycophenolate as the first-line therapy for interstitial lung disease (ILD), with azathioprine and tocilizumab as additional options, and short-term glucocorticoids may be used cautiously. This approach is based on the most recent guidelines from the American College of Rheumatology (ACR) and the American College of Chest Physicians (CHEST) 1. The treatment of MCTD requires a tailored approach, considering the specific organ involvement and symptom severity.

Key Considerations

• Mycophenolate is conditionally recommended over other listed therapies for MCTD-ILD, based on a hierarchy established by head-to-head votes 1.
• Azathioprine and tocilizumab are listed as additional options for MCTD-ILD, with the decision on which therapy to use dependent on specific situations and patient factors 1.
• Short-term glucocorticoids may be used in MCTD, but with caution, especially in patients with an SSc phenotype who may be at increased risk of renal crisis 1.
• The management of MCTD-ILD should also consider the potential benefits and risks of other therapies, such as nintedanib, which is not recommended as a first-line option for MCTD-ILD 1.

Additional Therapies

• For people with SARD-ILD, including MCTD-ILD, additional integrative and pharmacologic interventions may be considered, such as exercise, palliative care, physiotherapy, pulmonary rehabilitation, smoking cessation, and supplemental oxygen 1.
• Symptom-specific treatments, such as NSAIDs for pain and inflammation, calcium channel blockers for Raynaud's phenomenon, and proton pump inhibitors for esophageal reflux, are also important in the management of MCTD 1.

Monitoring and Follow-up

• Regular monitoring of disease activity, medication side effects, and screening for pulmonary hypertension with echocardiography is essential in the management of MCTD 1.
• A multidisciplinary approach, involving rheumatologists and pulmonologists, is necessary for the early recognition and management of MCTD-ILD, and for providing holistic care to patients with this complex disease 1.

From the Research

Management Approach for Mixed Connective Tissue Disease (MCTD)

The management of MCTD involves a multi-faceted approach, considering the disease's complexity and its overlap with other autoimmune conditions. Key aspects of management include:

• Treatment: The treatment of MCTD is largely based on the management of similar problems in other rheumatic conditions, such as systemic lupus erythematosus, scleroderma, and polymyositis, as no controlled clinical trials have been performed to guide therapy in MCTD 2.
• Medications: Corticosteroids, antimalarials, methotrexate, cytotoxics (most often cyclophosphamide), and vasodilators have been used in the treatment of MCTD with varying degrees of success 2. A combination of low-dose corticosteroids and hydroxychloroquine sulfate may be an effective therapeutic approach for MCTD with multiple neurological complications 3.
• Immunosuppressive Therapy: Strong immunosuppressive therapy, including corticosteroid, intravenous immunoglobulin, and cyclosporine A, can ameliorate myositis, interstitial lung disease, and inflammation in patients with MCTD complicated by microscopic polyangiitis 4.
• Individualized Therapy: Given the heterogeneous clinical course of MCTD, therapy should be individualized to address the specific organ involved and the severity of underlying disease activity 2.
• Follow-up Exams: Regular follow-up exams are essential to monitor the disease's progression and potential complications, although formal recommendations for follow-up exams are currently lacking 5.

Specific Considerations

• Neurological Complications: MCTD with multiple neurological complications, such as transverse myelitis, mononeuritis multiplex, and multiple lacunar infarctions, poses diagnostic and therapeutic challenges, and requires prompt treatment 3.
• Microscopic Polyangiitis: Complication of MCTD with microscopic polyangiitis is rare, but can be effectively managed with strong immunosuppressive therapy 4.
• Methotrexate Therapy: Methotrexate has been used in the treatment of MCTD, particularly in patients with scleroderma and mixed connective tissue disease, although evidence from clinical trials is limited 6.