CURRENT Essentials of Critical Care.pdf

220 Current Essentials of Critical Care■■■Scleroderma/Progressive Systemic SclerosisEssentials of Diagnosis• Signs and symptoms depend on organ involvement and includedyspnea, fatigue, right-heart failure, cough, hemoptysis, headache,blurred vision• Autoimmune disease characterized by exuberant fibrosis andsmall-vessel vasculopathy involving skin, lungs, heart, gastrointestinaltract, musculoskeletal system• Two major subsets: limited cutaneous sclerosis (CREST syndromewith calcinosis cutis, Raynaud phenomenon, esophagealdysmotility, sclerodactyly, telangiectasias) with indolent course;diffuse systemic sclerosis with aggressive course• Complications requiring ICU care: pulmonary hypertension, aspirationpneumonia, alveolar hemorrhage, renal crisis with malignanthypertension• Skin involvement may make intravenous access difficultDifferential Diagnosis• Pulmonary hypertension: primary or drug-induced, valvularheart disease• Aspiration pneumonia: community-acquired pneumonia, acuteinterstitial pneumonitis• Alveolar hemorrhage: bleeding telangiectasias, ARDSTreatment• Treatment targets systemic inflammation with immunosuppressiveagents such as prednisone, cyclophosphamide• Hyperalimentation may be required if GI involvement causesmalabsorption, malnutrition, pseudoobstruction• Elevate head of bed, prokinetic agents, acid-suppressing drugsto reduce aspiration pneumonia risk• Pulmonary hypertension may benefit from oxygen, pulmonaryvasodilators, cardiac inotropic agents, diuretics• Renal crisis: avoid corticosteroids; aggressive blood pressurecontrol; ACE inhibitors for treatment and prophylaxis; hemodialysisfor hyperkalemia or uremia■ PearlScleroderma renal crisis, typically characterized by hypertension anda rapidly rising creatinine, has been associated with the antecedentuse of high-dose corticosteroids.ReferenceCossio M et al: Life-threatening complications of systemic sclerosis. Crit CareClin 2002;18:819. [PMID: 12418443]