26th January 2024

Management

Although there have been no controlled trials to guide the management of MCTD, general principles are guided by effective therapies for SLE, systemic sclerosis and polymyositis. 

• Glucocorticoids: Oral prednisolone is the steroid of choice initially and is effective in treating pleurisy, pericarditis, myocarditis and oesophageal dysmotility. Corticosteroids need to be weaned due to the adverse effects of long-term steroid therapy. 
•  Immunosuppressants/Steroid-sparing agents: Methotrexate or Hydroxychloroquine may be started while weaning off steroids; they are effective in preventing flares and symptom control. 
• Anti-hypertensives: Pulmonary arterial hypertension is a major cause of death in patients with MCTD. As such, routine echocardiography is recommended. Treatment for PAH includes anti-hypertensives such as calcium channel blockers and ACE-i. Other treatments include intravenous prostacyclins and anti-coagulation, endothelin -1 antagonist (e.g. Bosentan) and phosphodiesterase inhibitors (e.g. Sildenafil). 
• Rituximab: This is used in patients who have steroid-resistant thrombocytopenia, autoimmune haemolytic anaemia, myositis and severe eruptive skin disease.