Although<unk>  was originally described as a disease with a good treatment response to corticosteroids, the treatment of the disease is based on the specific manifestations and clinical complications, similar to how other signs and symptoms are treated in other connective<unk>  disease. Standard For arthritis, non-steroidal anti-inflammatories or low dose prednisone are usually used, which can be used in association with methotrexate or hydroxychloroquine. Temporomandibular joint arthritis has been shown to be successfully treated with condylar reconstruction using chondral grafts. Higher doses of corticosteroids (0.25 to 1 mg/kg/day) are used in complications such as myositis, meningitis, pleuritis, pericarditis, myocarditis, interstitial lung disease, or hematologic abnormalities. On the contrary, Raynaud’s phenomenon, acrosclerosis or peripheral neuropathies are usually resistant to corticosteroids. Cyclophosphamide are useful in interstitial lung disease and in the eventual serious renal involvement. In cases of myositis or thrombocytopenias resistant to corticosteroids, intravenous immunoglobulins may be useful. For Raynaud, general measures (such as tobacco cessation, protection against the cold), calcium antagonists, endovenous prostaglandins or endothelin-2 antagonists may be useful. In patients with gastroesophageal reflux, proton pump inhibitors and H2 receptor antagonists can be used, following protocol for the usual treatment of these scleroderma problems. Since pulmonary hypertension is the leading cause of death, its early diagnosis by routine echocardiography and the rapid initiation of treatment with endothelin-1 antagonists (bosentan), phosphodiesterase 5 inhibitors (sildenafil) or endovenous prostacyclins (epoprostenol) manage to considerably improve morbidity and mortality. Investigational Further investigation into appropriate treatment options for<unk>  are in progress. Treatment for various rheumatoid diseases are currently undergoing research and have the potential to be used for patients presenting with similar signs and symptoms. Better understanding the pathophysiology of the disease and its progression will enable better targeted treatment options.
Mixed connective tissue disease
Carnitine palmitoyltransferase II
Undifferentiated connective tissue disease
Scleroderma
Systemic scleroderma
Antisynthetase syndrome
CREST syndrome
Cryopyrin-associated periodic syndrome