Esophagitis Retrosternal pain, pyrosis, melena CBC Barium swallow Esophageal ph probe and manometry Antacids/cimetidine Surgical manipulation for chronic unremitting complaints EKG, electrocardiogram; CO, carbon monoxide; CT, computed tomography; ACE, angiotensin-converting enzyme; PA, posteroanterior; BUN, blood urea nitrogen; CBC, complete blood count Pulmonary hypertension is the most common cause of dyspnea in patients with JSSc On auscultation, there is a wide or fixed splitting of the second heart sound and the pulmonic component is accentuated The EKG shows right ventricular hypertrophy Echocardiography and right heart catheterization may be necessary to differentiate cardiac from pulmonary etiologies of respiratory deterioration Corticosteroids and cyclophosphamide (50 mg/day orally or 500 to 750 mg/m2 by monthly IV infusion) are the treatment of choice in patients without established interstitial fibrosis Renal Complications Sclerodermatous involvement of the vessels of the kidney is the most common cause of renal failure in adults with JSSc Risk factors include proteinuria, hypertension, rapid progression of skin thickening early in the illness, anemia, pericardial effusion, and CHF The development of a microangiopathic hemolytic anemia suggests imminent renal failure These complications appear to be less common in children than in adults Renal failure may develop gradually or acutely in a patient with known renal disease, and use of corticosteroids may precipitate its appearance Scleroderma renal crisis is characterized by precipitous hypertension Immediate investigation should include urinalysis, measurement of urine output and urinary electrolytes, serum electrolytes, BUN, creatinine, and plasma renin level A major advance in the pharmacologic management of scleroderma renal crisis has been the use of ACE inhibitors such as captopril Patients who fail to respond to this drug may still respond to potent vasodilators such as minoxidil, along with β-blockers and diuretics; regimens involving multiple drugs may also be necessary (see Chapters 37 Hypertension and 100 Renal and Electrolyte Emergencies ) Because most patients with severe scleroderma renal disease have a component of myocarditis and ventricular stiffness, maintenance of blood volume is essential to ensure adequate preload to support the circulation Peripheral Vascular Complications RP can often be incapacitating, particularly in cold weather Symptoms include severe pain in the extremities and loss of sensation in the tips of the digits Treatment with calcium-channel antagonists, such as slow-release nifedipine, may decrease the frequency or severity of attacks In urgent cases with impending gangrene, systemic (e.g., iloprost) or topical vasodilators (e.g.,