Scleroderma: Difference between revisions

Revision as of 05:24, 30 January 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
Interstitial lung disease
Renal impairment
GI dysmotility
GERD/aspiration
Chronic esophagitis and stricture formation

Emergencies

Scleroderma renal crisis
- Causes HTN 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 HTN
- Alveolar hemorrhage
  - Hemoptysis, infiltrates on CXR, anemia.
  - Emergent bronchoscopy
  - High dose glucocorticoids, cyclophosphamide, local vessel embolization or plasma exchange
Cardiac
- Acute heart failure
  - Can be from diastolic dysfunction, malignant HTN during renal crisis, and decompensated pulmonary HTN
- Cardiopulmonary complications are the most common causes of scleroderma-related death^[2], with cardiac causes^[3] and pulmonary fibrosis^[4] being the most common.

Source

Tintinalli

↑ 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

Authors:

[1] 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

[2] Nikpour, M., Baron, M. Mortality in systemic sclerosis: lessons learned from population-based and observational cohort studies. Curr Opin Rheumatol. 2014;26:131–137

[3] 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

[4] 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

[1]

[2]

[3]

[4]

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 == Emergencies ==
 *Scleroderma renal crisis
-**Most common cause of death
 **Causes HTN emergency
 **Treatment is strict BP control - ACEi drug of choice
-*Respiratory failure
+***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>
-*ARDS
+*Pulmonary
-*Aspiration pneumonitis
+**Respiratory failure
-*Pulmonary HTN
+**ARDS
-*Alveolar hemorrhage
+**Aspiration pneumonitis
-**[[Hemoptysis]], infiltrates on CXR, anemia.
+**Pulmonary HTN
-**Emergent bronchoscopy
+**Alveolar hemorrhage
-**High dose glucocorticoids, cyclophosphamide, local vessel embolization or plasma exchange
+***[[Hemoptysis]], infiltrates on CXR, anemia.
-*Acute heart failure
+***Emergent bronchoscopy
-**Can be from diastolic dysfunction, malignant HTN during renal crisis, and decompensated pulmonary HTN
+***High dose glucocorticoids, cyclophosphamide, local vessel embolization or plasma exchange
+*Cardiac
+**Acute heart failure
+***Can be from diastolic dysfunction, malignant HTN during renal crisis, and decompensated pulmonary HTN
+**'''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.
 == Source ==