Scleroderma: Difference between revisions

(Text replacement - " ==" to "==")
(Text replacement - "HTN" to "hypertension")
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==Emergencies==
==Emergencies==
*Scleroderma renal crisis
*Scleroderma renal crisis
**Causes HTN emergency
**Causes hypertension emergency
**Treatment is strict BP control - ACEi drug of choice
**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>  
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**ARDS
**ARDS
**Aspiration pneumonitis
**Aspiration pneumonitis
**Pulmonary HTN
**Pulmonary hypertension
**Alveolar hemorrhage
**Alveolar hemorrhage
***[[Hemoptysis]], infiltrates on CXR, anemia.
***[[Hemoptysis]], infiltrates on CXR, anemia.
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*Cardiac
*Cardiac
**Acute heart failure
**Acute heart failure
***Can be from diastolic dysfunction, malignant HTN during renal crisis, and decompensated pulmonary HTN
***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 22:47, 30 July 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 hypertension 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 glucocorticoids, 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.

References

  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
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