Basic immunosuppressive therapy in 280 patients with mixed connective tissue disease (MCTD).
| Clinical Symptoms (no. patients) | Treatment |
|---|---|
| Skin (rash, photosensitivity; n = 102) | CS and antimalarials |
| Arthritis (n = 207) | Antimalarials, low-dose CS (prednisone ≤ 20 mg/day), MTX for flares |
| Erosive arthritis (n = 44) | MTX, disease-modifying agents such as TNF-α blockers |
| Serositis (n = 83) | 0.25–1 mg/kg CS |
| Pulmonary involvement | |
| Interstitial lung disease (n = 132) | High-dose CS* (≥ 1 mg/kg), often pulse CS (1 g/day for 3 days), and CYC or MMF |
| Pulmonary arterial hypertension (n = 50) | High-dose CS* (≥ 1 mg/kg), often pulse CS (1 g/day for 3 days), CYC In addition: prostanoids, endothelin receptor antagonists, phosphodiesterase-5 inhibitor, and anticoagulants (LMWH) |
| Renal disease (n = 8) | CS (0.25–1.0 mg/day), CYC |
| Myositis (n = 91) | CS (0.5–1 mg/kg) Consider CYC or IVIG, azathioprine |
↵* Dosage ≥ 1 mg/kg/day methylprednisolone. MTX: methotrexate; CYC: cyclophosphamide; MMF: mycophenolate mofetil; IVIG: intravenous immune globulin; CS: corticosteroid; LMWH: low molecular weight heparin; TNF: tumor necrosis factor.