Life-threatening complications of systemic sclerosis
Section snippets
Prevalence
Pulmonary arterial hypertension is common among patients with SSc, even in the absence of clinical signs and symptoms. In a sample of 34 consecutive patients with SSc who did not have a clinical diagnosis of pulmonary hypertension, pulmonary artery systolic pressure estimated from the velocity of the tricuspid regurgitation jet during Doppler echocardiography ranged from 15 to 95 mm Hg. Twelve of these patients (35%) had a pulmonary artery systolic pressure of 30 mm Hg or greater [5]. The
Renal crisis
Scleroderma renal crisis is associated with acute malignant hypertension that requires intensive care. In a large, retrospective, case-control study, a significant association between antecedent high-dose corticosteroid therapy and the development of scleroderma renal crisis was found [61]. As in hypertensive emergencies from other causes, patients may notice increased fatigue, malaise, dyspnea, headache, and blurred vision. Cardiac decompensation is often seen. The pathogenesis involves
Summary
Pulmonary arterial hypertension is common in patients with SSc. Fig. 1 shows the diagnostic and therapeutic approach to PAH in SSc. Doppler echocardiography may suggest the diagnosis, but RHC is necessary to confirm PAH and to measure vasoreactivity. Therapy is directed at the underlying connective tissue disease. Vasoreactive patients often benefit from therapy with high-dose calcium-channel blockers, but most patients are not vasoreactive. Intravenous epoprostenol and oral endothelin-1
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Lung Transplantation in Autoimmune Diseases
2010, Clinics in Chest MedicineCitation Excerpt :Thus, it is clear that gastroesophageal disease is common after transplantation. An important early posttransplant concern is that this population is at high risk for developing severe aspiration pneumonia that compromises graft function; for this reason, severe gastroesophageal disease is an absolute or strong relative contraindication to lung transplantation at many LTxp centers.43,90 In the past decade, there has also been increasing evidence implicating GERD as a risk factor for chronic allograft dysfunction.
Vascular complications of scleroderma
2007, Autoimmunity ReviewsCitation Excerpt :Treatment is supportive, and prognosis is dismal. In contrast, scleroderma renal crisis is associated with systemic hypertension, onion skinning of afferent arterioles, and response to ACE inhibition and renal replacement therapy [23]. Novel therapies are currently being tested in the treatment of SSc and have the potential of modifying the disease process and overall clinical outcome [24].
Low-dose UVA1 phototherapy in systemic sclerosis: Effects on acrosclerosis
2004, Journal of the American Academy of DermatologyEpidemiology of systemic sclerosis: a multi-database population-based study in Tuscany (Italy)
2021, Orphanet Journal of Rare DiseasesInduced sputum analysis in subjects with systemic sclerosis
2016, Respiratory Care