CAN CAUSE ANGINA DUE TO MICROVASCULAR ABNORMALITIES. ENVIRONMENTAL FACTORS ASSOCIATED WITH SS & SCLERODERMA LIKE ILLNESS INCLUDE COAL & GOLD MINING ( SILICA DUST ), POLYVINYL CHLORIDE, BENZINE, TOLUENE & RAPESEED OIL
Medical Care:
" Skin thickening can be treated with D-penicillamine, methotrexate (efficacy not fully assessed), and other experimental drugs (interferon gamma, relaxin, cyclophosphamide). No US Food and Drug Administration (FDA)-approved therapies exist for scleroderma. No controlled studies demonstrate superiority; however, large uncontrolled series suggest beneficial effects from D-penicillamine. Interferon gamma is effective, but use is limited because it causes activation of inflammatory and endothelial cells.
" Pruritus can be treated with moisturizers, histamine 1 (H1) and histamine 2 (H2) blockers, tricyclic antidepressants, and trazodone.
" Raynaud phenomenon can be treated with calcium blockers (to tolerance), prazosin, dipyridamole, aspirin, and topical nitrates. In the event of thrombosis and vascular flow compromise, a tissue plasminogen activator, heparin, and urokinase may be necessary. In very severe cases, patients may benefit from a pharmacologic cervical sympathectomy. Bosentan, a dual endothelin receptor antagonist, is under investigation and may decrease new digital ulcer formation.
* IF A RAYNAUD PHENOMENON OCCURS IN HEART, VASODILATOR & SYMPATHOLYTIC AGENTS MAY BE HELPFUL. ACE INHIBITORS MAY ALSO BLOCK ANGIOTEN INDUCED MYOCARDIAL FIBROSIS.
" Gastrointestinal symptoms may be treated with antacids, H2 blockers, reflux precautions, proton pump inhibitors, prokinetic agents, octreotide, smaller meals, and laxatives.
" Pulmonary fibrosis may be treated with cyclophosphamide, either orally or in intravenous pulses. Pulmonary hypertension may require supplemental oxygen. Recent studies indicate that severe pulmonary hypertension may benefit from treatment with bosentan, a dual endothelin receptor antagonist used in the treatment of primary (idiopathic) pulmonary hypertension. Bosentan has demonstrated substantial improvement in patients with scleroderma-associated pulmonary hypertension in hemodynamic parameters and New York Heart Association classification.
" Renal crisis episodes are best prevented and treated with the aggressive use of ACE inhibitors at the earliest sign of hypertension.
" Myositis may be treated cautiously with steroids (first choice), methotrexate, and azathioprine. Doses of steroids greater than 40 mg/d are associated with a higher incidence of sclerodermal renal crisis.
" Arthralgias can be treated with acetaminophen and nonsteroidal anti-inflammatory drugs (NSAIDs).
" Proteinuria is not uncommon in patients with scleroderma who are receiving penicillamine.
Surgical Care: Digital sympathectomy may be used for patients with severe Raynaud phenomenon who have an acute attack that is not relenting and who are threatened by digital loss. Debridement or amputation may be required in severe ischemic or infected digital lesions. Hand surgery may be performed to correct severe flexion contractures.
Diet: Instruct the patient to avoid large does of vitamin C (>1000 mg/d) because it stimulates collagen formation and may enhance its deposition.
Activity:
" Ensure that the patient maintains a core body temperature to try to minimize the Raynaud phenomenon.
" Assist the patient in avoiding contamination of any skin wound that occurs as a result of ischemic lesions or calcinosis.
" Digital ulcers must be kept clean and dry.
" Instruct the patient to perform continuous physical and occupational therapy to maintain range of motion and to minimize or delay contractures.