Scleroderma: Difference between revisions
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==Clinical Features== | ==Clinical Features== | ||
*Systemic complaints (fever, malaise, fatigue, weight loss, myalgias) | *Systemic complaints ([[fever]], malaise, fatigue, weight loss, myalgias) | ||
*Skin lesions (fingers, hands, face) | *Skin lesions (fingers, hands, face) | ||
*Carpal tunnel | *Carpal tunnel | ||
*Raynaud's | *Raynaud's phenomenon | ||
*Interstitial lung disease | *Interstitial lung disease | ||
*Renal impairment | *Renal impairment | ||
*GI dysmotility | *GI dysmotility | ||
*GERD/aspiration | *[[GERD]]/aspiration | ||
*Chronic esophagitis and stricture formation | *Chronic [[esophagitis]] and stricture formation | ||
==Emergencies== | ==Emergencies== | ||
*Scleroderma renal crisis | *Scleroderma renal crisis | ||
**Causes | **Causes [[hypertensive emergency]] | ||
**Treatment is strict BP control - | **Treatment is strict BP control - [[ACEI]] drug of choice | ||
***Was the most common cause of death prior to ACEi usage<ref>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</ref> | ***Was the most common cause of death prior to ACEi usage<ref>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</ref> | ||
*Pulmonary | *Pulmonary | ||
**Respiratory failure | **Respiratory failure | ||
**ARDS | **[[ARDS]] | ||
**Aspiration pneumonitis | **[[Aspiration pneumonitis]] | ||
**Pulmonary hypertension | **[[Pulmonary hypertension]] | ||
**Alveolar hemorrhage | **[[Diffuse alveolar hemorrhage|Alveolar hemorrhage]] | ||
***[[Hemoptysis]], infiltrates on CXR, anemia. | ***[[Hemoptysis]], infiltrates on CXR, anemia. | ||
***Emergent bronchoscopy | ***Emergent bronchoscopy | ||
***High dose | ***High dose [[corticosteroids]], [[cyclophosphamide]], local vessel embolization or [[plasma exchange]] | ||
*Cardiac | *Cardiac | ||
**Acute heart failure | **Acute [[heart failure]] | ||
***Can be from diastolic dysfunction, malignant hypertension during renal crisis, and decompensated pulmonary hypertension | ***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<ref>Nikpour, M., Baron, M. Mortality in systemic sclerosis: lessons learned from population-based and observational cohort studies. Curr Opin Rheumatol. 2014;26:131–137</ref>, with cardiac causes<ref>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</ref> and pulmonary fibrosis<ref>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</ref> being the most common. | **'''Cardiopulmonary''' complications are the most common causes of scleroderma-related death<ref>Nikpour, M., Baron, M. Mortality in systemic sclerosis: lessons learned from population-based and observational cohort studies. Curr Opin Rheumatol. 2014;26:131–137</ref>, with cardiac causes<ref>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</ref> and pulmonary fibrosis<ref>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</ref> being the most common. | ||
Revision as of 23:13, 13 November 2016
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
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