Medical Care:
" The overall goal of therapy is to control symptoms and maintain function. Target medical therapy to specific organ involvement and extent of disease activity. Monitoring for development of complications, such as pulmonary hypertension or infection, is important.
* Strongly consider annual echocardiogram, pulmonary function tests, and barium swallow
Diet:
" Patients with hypertension, esophageal reflux, malabsorption, or other sclerodermatous-type bowel involvement may need special considerations.
" Because atherosclerotic heart disease remains a major risk for all patients, advocate a heart-healthy diet. However, no specific dietary manipulations have been demonstrated to be effective in treating MCTD.
Activity: Convincing data support the value of an active lifestyle and an exercise program tailored to the needs of patients with arthritis of various types. This approach also appears to be appropriate in MCTD.
MCTD is a chronic and usually mild disease, which can be treated symptomatically or with corticosteroids or immunosuppressives if the severity of disease justifies it. Combined treatment allows dose reduction of systemic steroids (corticosteroid-sparing effect). Treatment depends on internal organ involvement. Usually middle doses of systemic corticosteroids are effective in most cases. Anti-inflammatory agents are helpful for arthralgia, myalgia, and swelling of the hands. Skin lesions can be treated with topical corticosteroids. In all cases, photoprotection is recommended.
DRUG THERAPY :
1. STEROIDS : Use corticosteroids to treat children with hypocomplementemia and elevated levels of anti-DNA antibodies, children with active myositis, and children with significant manifestations of scleroderma. Dose varies with intensity of disease activity. Consider daily prednisone (1 mg/kg/d) or higher-dose alternate-day prednisone (5 mg/kg/d, not to exceed 150-250 mg depending on size of patient). Alternatively, lower-dose daily prednisone (0.5 mg/kg) may be used in conjunction with intermittent high-dose IV methylprednisolone (30 mg/kg/dose, not to exceed 1 g) on a weekly basis. Of note, recent case reports suggest that 3 days of pulse IV methylprednisolone followed by moderate-to-high dose oral steroids improved pulmonary artery pressures in a patient with MCTD and pulmonary artery hypertension.
- PREDNISOLONE
2. IMMUNOSUPPRESSANTS : Evaluate children with signs of active nephritis to determine World Health Organization (WHO) classification category of their nephritis. For patients with class IV nephritis and some patients with class III nephritis, treat with corticosteroids and cyclophosphamide. Use azathioprine for individuals with milder nephritis. Use methotrexate for persons with arthritis not controlled by NSAIDs and for persons with fibrosis, especially sclerodermatous skin. Consider cyclophosphamide for individuals with severe systemic involvement of other vital organs, especially brain and lung. Consider other agents (eg, mycophenolate mofetil, cyclosporine) when standard therapies have failed. Other treatments under study include hormonal therapy, biologic agents that target cytokine production, and anti-DNA antibodies. For patients with severe persistent disease, autologous and stem cell transplantation is under study.
- CYCLOSPORINE
- CYCLOPHOSPHAMIDE
- AZATHIOPRINE
- METHOTREXATE
3. NSAIDS :
- NAPROXEN
- TOLMETIN
- DICLOFENAC
4. ANTIMALARIALS : Patients in whom major disease manifestation is lupus, rash, and other minor symptoms can be treated with hydroxychloroquine
- HCQS
5. TOPICAL STEROIDS : THESE CAN DECREASE EPIDERMAL PROLIFERATION
- FLUTICASONE
6. CALCIUM & VITAMIN D THERAPIES : All patients who are on corticosteroids or who have arthritis are at greater risk for osteopenia and its complications. Diet and appropriate supplementation with vitamin D and calcium are important tools for bone health in these patients.
- CALCIUM CARBONATE
- CALCIFEDIOL
7. RHEOSTATIC AGENTS : These agents are used to improve peripheral blood flow and to improve delivery of oxygen to tissue suffering from peripheral vascular disease. In individuals with MCTD, used to decrease symptoms and damage from Raynaud phenomenon.
- PENTOXIFYLLINE (TRENTAL)
8. PHOSPHODIESTERASE ( TYPE 5 ) ENZYME INHIBITORS :These agents increase peripheral vasodilation and may be helpful in treating symptoms associated with Raynaud disease. Inhibits phosphodiesterase type 5 in smooth muscle of pulmonary vasculature in which phosphodiesterase type 5 is responsible for the degradation of cGMP. Increased cGMP concentration results in pulmonary vasculature relaxation; vasodilation in the pulmonary bed and the systemic circulation (to a lesser degree) may occur. The systemic vasodilation may be helpful to reduce Raynaud disease symptoms.
- SILDENAFIL : 20 MG OD TO 20 MG TDS