Scleroderma
Background
- Autoimmune disease, aka Systemic Sclerosis
- Inappropriate and excessive accumulation of collagen and matrix in various tissues
- Widespread vascular lesions
- Endothelial dysfunction
- Vascular spasm
- Vascular wall thickening
- Narrowed lumen
Clinical Features
- Systemic complaints (fever, malaise, fatigue, weight loss, myalgias)
- Skin lesions (fingers, hands, face)
- Carpal tunnel
- Raynaud's phenomenon
- Interstitial lung disease
- Renal impairment
- GI dysmotility
- GERD/aspiration
- Chronic esophagitis and stricture formation
Emergencies
- Scleroderma renal crisis
- Causes hypertensive emergency
- Treatment is strict BP control - ACEI drug of choice
- Was the most common cause of death prior to ACEi usage[1]
- Pulmonary
- Respiratory failure
- ARDS
- Aspiration pneumonitis
- Pulmonary hypertension
- Alveolar hemorrhage
- Hemoptysis, infiltrates on CXR, anemia.
- Emergent bronchoscopy
- High dose corticosteroids, cyclophosphamide, local vessel embolization or plasma exchange
- Cardiac
- Acute heart failure
- Can be from diastolic dysfunction, malignant hypertension during renal crisis, and decompensated pulmonary hypertension
- Cardiopulmonary complications are the most common causes of scleroderma-related death[2], with cardiac causes[3] and pulmonary fibrosis[4] being the most common.
- Acute heart failure
Differential Diagnosis
Evaluation
Management
Disposition
See Also
References
- ↑ Komocsi A, Vorobcsuk A, Faludi R, et al. The impact of cardiopulmonary manifestations on the mortality of SSc: a systematic review and meta-analysis of observational studies. Rheumatology (Oxford) 2012;51:1027–1036
- ↑ Nikpour, M., Baron, M. Mortality in systemic sclerosis: lessons learned from population-based and observational cohort studies. Curr Opin Rheumatol. 2014;26:131–137
- ↑ Komocsi A, Vorobcsuk A, Faludi R, et al. The impact of cardiopulmonary manifestations on the mortality of SSc: a systematic review and meta-analysis of observational studies. Rheumatology (Oxford) 2012;51:1027–1036
- ↑ Tyndall AJ, Bannert B, Vonk M, Airo P, Cozzi F, Carreira PE, et al. Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis. 2010;69:1809–15. doi: 10.1136/ard.2009.114264