Scleroderma: Difference between revisions
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***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>  
**Severe renal crisis is '''life threatening''' with incidence of 8-10% in Limited and 10-20% in diffuse
**Severe renal crisis is '''life threatening''' with incidence of 8-10% in Limited and 10-20% in diffuse
***Similar to TTP/HUS with microangiography
***Similar to [[TTP]]/[[HUS]] with microangiography
***Poor prognosis with sudden onset of [[hypertension]], [[encephalopathy]], [[CVA]], retinopathy
***Poor prognosis with sudden onset of [[hypertension]], [[encephalopathy]], [[CVA]], retinopathy
***Risk factors are rapidly progressing diffuse scleroderma, high dose glucocorticoid use, cyclosporine therapy, presence of anti-RNA-polymerase antibodies.<ref>Vymetal J, Skacelova M, Smrzova A, Klicova A, Schubertova M, Horak P, Zadrazil
***Risk factors are rapidly progressing diffuse scleroderma, high dose [[glucocorticoid]] use, [[cyclosporine]] therapy, presence of anti-RNA-polymerase antibodies.<ref>Vymetal J, Skacelova M, Smrzova A, Klicova A, Schubertova M, Horak P, Zadrazil
J. Emergency situations in rheumatology with a focus on systemic autoimmune
J. Emergency situations in rheumatology with a focus on systemic autoimmune
diseases. Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub. 2016
diseases. Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub. 2016
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*Pulmonary
*Pulmonary
**[[Respiratory failure]]
**[[Respiratory failure]]
**Interstitial lung disease, leading to pulmonary fibrosis
**[[Interstitial lung disease]], leading to pulmonary fibrosis
***Found in dorsal portion of both lower lobes, but can extend to upper lobes in severe diease
***Found in dorsal portion of both lower lobes, but can extend to upper lobes in severe diease
***Poor prognosis with severe pulmonary impairment.  42% die within 10 years of disease onset<ref>Luo Y, Xiao R. Interstitial Lung Disease in Scleroderma: Clinical Features and Pathogenesis. Rheumatology 2011 doi:10.4172/2161- 1149. S1-002. Available from: http://omicsonline.org/ interstitial-lung-disease-in-scleroderma-clinical-features-and- pathogenesis-2161-1149.S1-002.pdf</ref>
***Poor prognosis with severe pulmonary impairment.  42% die within 10 years of disease onset<ref>Luo Y, Xiao R. Interstitial Lung Disease in Scleroderma: Clinical Features and Pathogenesis. Rheumatology 2011 doi:10.4172/2161- 1149. S1-002. Available from: http://omicsonline.org/ interstitial-lung-disease-in-scleroderma-clinical-features-and- pathogenesis-2161-1149.S1-002.pdf</ref>
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***Gradually progressive exercise breathlessness
***Gradually progressive exercise breathlessness
***Fatigue
***Fatigue
***Hoarseness from nerve palsy caused by dilated pulmonary artery stem
***[[dysphonia|Hoarseness]] from nerve palsy caused by dilated pulmonary artery stem
**[[Diffuse alveolar hemorrhage|Alveolar hemorrhage]]
**[[Diffuse alveolar hemorrhage|Alveolar hemorrhage]]
***[[Hemoptysis]], infiltrates on CXR, anemia.
***[[Hemoptysis]], infiltrates on CXR, anemia.
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***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.
**Anginal pain from right ventricle ischemia and low cardiac output
**Anginal [[chest pain|pain]] from right ventricle ischemia and low cardiac output


==Differential Diagnosis==
==Differential Diagnosis==
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*Frequent blood pressure checks
*Frequent blood pressure checks
*Serologic markers for each subset
*Serologic markers for each subset
*[[UA]] for proteinuria or hematuria
*[[UA]] for [[proteinuria]] or [[hematuria]]
*BMP
*BMP
*[[CXR]], CT chest
*[[CXR]], CT chest
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** [[ACEI]] drug of choice, e.g. [[captopril]] 6.25 to 12.5mg PO, TID.  
** [[ACEI]] drug of choice, e.g. [[captopril]] 6.25 to 12.5mg PO, TID.  
*Avoid [[diuretics]]
*Avoid [[diuretics]]
**Consult renal, as 50% of patients will require dialysis.
**Consult renal, as 50% of patients will require [[dialysis]].


===[[Pulmonary hypertension]]===
===[[Pulmonary hypertension]]===
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*Acute decompensation:  
*Acute decompensation:  
**Optimize (usually reduce) RV preload:
**Optimize (usually reduce) RV preload:
***Usually euvolemic or hypervolemic, rarely need IV fluids so diuretics can benefit and treat the RV failure<ref>Ternacle, J et al. Diruetics in Normotensive Patients with Acute Pulmonary Embolism and Right Ventricular Dilation. Circulation Journal. Vol 77(10) 2013. 2612-618.</ref>
***Usually euvolemic or hypervolemic, rarely need IV fluids so [[diuretics]] can benefit and treat the RV failure<ref>Ternacle, J et al. Diruetics in Normotensive Patients with Acute Pulmonary Embolism and Right Ventricular Dilation. Circulation Journal. Vol 77(10) 2013. 2612-618.</ref>
***[[Furosemide]] 20-40mg IV or drip at 5-20 mg/hr
***[[Furosemide]] 20-40mg IV or drip at 5-20 mg/hr
***If suspect [[sepsis]] or [[hypovolemia]], small (250-500cc) NS challenge to assess fluid responsiveness.  If not responsive to IVF challenge, start [[norepinephrine]] (MAP > 65 mmHg).
***If suspect [[sepsis]] or [[hypovolemia]], small (250-500cc) NS challenge to assess fluid responsiveness.  If not responsive to IVF challenge, start [[norepinephrine]] (MAP > 65 mmHg).
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**Reduce RV afterload
**Reduce RV afterload
***Avoid [[hypoxia]], maintain O2 sat >90% (increases pulmonary vasoconstriction)
***Avoid [[hypoxia]], maintain O2 sat >90% (increases pulmonary vasoconstriction)
***Avoid hypercapnea (increases pulmonary vascular resistance)
***Avoid hypercapnia (increases pulmonary vascular resistance)
***Avoid [[acidosis]]
***Avoid [[acidosis]]
**Treat arrhythmias:'''
**Treat arrhythmias:'''
***Avoid calcium channel blockers and β-blockers
***''Avoid'' calcium channel blockers and β-blockers
**Optimize oxygenation
**Optimize oxygenation
***Intubated patients should be optimized to increased O2 delivery and minimize hypercapnea, maintain low tidal volumes and low PEEP as tolerated
***Intubated patients should be optimized to increased O2 delivery and minimize hypercapnia, maintain low tidal volumes and low PEEP as tolerated


===Interstitial lung disease===
===Interstitial lung disease===
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*Vessel embolization if localizable
*Vessel embolization if localizable
*[[Hemoptysis|Management of Hemoptysis]]
*[[Hemoptysis|Management of Hemoptysis]]
**[[Hemoptysis]], infiltrates on CXR, anemia.
**Emergent bronchoscopy
**High dose [[corticosteroids]], [[cyclophosphamide]], local vessel embolization or [[plasma exchange]]


===CREST Syndrome===
===CREST Syndrome===
*Empiric treatment with a [[PPI]] to prevent reflux and stricture formation
*Empiric treatment with a [[PPI]] to prevent reflux and stricture formation
*Transfuse as needed for anemia due to mucosal telangiectasias
*Transfuse as needed for anemia due to mucosal telangiectasias
*Ursodial treatment  if concomitant primary biliary cirrhosis to prevent progression to secondary cirrhosis
*[[Ursodiol]] treatment  if concomitant primary biliary cirrhosis to prevent progression to secondary cirrhosis


**[[Hemoptysis]], infiltrates on CXR, anemia.
**Emergent bronchoscopy
**High dose [[corticosteroids]], [[cyclophosphamide]], local vessel embolization or [[plasma exchange]]


==Disposition==
==Disposition==

Latest revision as of 18:01, 16 October 2019

Background

  • Autoimmune collagen vascular 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
  • Two types:
    • Diffuse Systemic Sclerosis: skin changes, which can progress to internal organ involvement
    • Limited Cutaneous Systemic Sclerosis, aka "CREST Syndrome"

Clinical Features

  • Systemic complaints (fever, malaise, fatigue, weight loss, myalgias)
  • Diffuse Systemic Sclerosis
    • hypo- or hyper- pigmentation of skin, extending from fingers and toes proximally, also involving chest and abdominal wall
    • Pigment is perserved around hair follicles leading to salt and pepper appearance
      Hypopigmentation in Diffuse Scleroderma, courtesy of Regional Derm website
    • Symmetric hand edema and Raynaud's phenomenon
    • Abrupt disease presentation; worse in first 18 months, then either improvement or worsening to involve internal organs
  • CREST Syndrome
    • Longstanding Raynaud's
    • Skin thickening and fibrosis distal to elbows and knees, and on face
    • Subcutaneous calcinosis
    • Esophageal dysmotility
    • Sclerodactyly
    • Telangiectasia
    • Indolent course

Emergencies

Differential Diagnosis

Blue Digit

Evaluation

  • Frequent blood pressure checks
  • Serologic markers for each subset
  • UA for proteinuria or hematuria
  • BMP
  • CXR, CT chest
  • +/- echo, right heart cath, lung biopsy
  • CREST syndrome
    • May need endoscopy and Hemoccult test to evaluate for blood loss from mucosal telangiectasias (gastric antral vascular ectasia, described as "watermelon stomach."

Management

Renal crisis

  • Rapid control of blood pressure
  • Avoid diuretics
    • Consult renal, as 50% of patients will require dialysis.

Pulmonary hypertension

  • Chronic management may include prostaglandin derivatives (e.g. epoprostenol), PDE-5 inhibitors (e.g. sildenafil), and/or endothelin receptor antagonists (e.g. bosentan)
  • Acute decompensation:
    • Optimize (usually reduce) RV preload:
      • Usually euvolemic or hypervolemic, rarely need IV fluids so diuretics can benefit and treat the RV failure[7]
      • Furosemide 20-40mg IV or drip at 5-20 mg/hr
      • If suspect sepsis or hypovolemia, small (250-500cc) NS challenge to assess fluid responsiveness. If not responsive to IVF challenge, start norepinephrine (MAP > 65 mmHg).
    • Increase cardiac output
      • Once MAP >65 mmHg, start low dose dobutamine (5-10mcg/kg/min)
      • Improves inotropic support and theoretically decreases pulmonary vascular resistance
    • Reduce RV afterload
      • Avoid hypoxia, maintain O2 sat >90% (increases pulmonary vasoconstriction)
      • Avoid hypercapnia (increases pulmonary vascular resistance)
      • Avoid acidosis
    • Treat arrhythmias:
      • Avoid calcium channel blockers and β-blockers
    • Optimize oxygenation
      • Intubated patients should be optimized to increased O2 delivery and minimize hypercapnia, maintain low tidal volumes and low PEEP as tolerated

Interstitial lung disease

Alveolar hemorrhage

CREST Syndrome

  • Empiric treatment with a PPI to prevent reflux and stricture formation
  • Transfuse as needed for anemia due to mucosal telangiectasias
  • Ursodiol treatment if concomitant primary biliary cirrhosis to prevent progression to secondary cirrhosis


Disposition

  • Hospitalization for any elevation in blood pressure, difficulty breathing, evidence of heart strain or pulmonary edema.

See Also

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. ↑ Vymetal J, Skacelova M, Smrzova A, Klicova A, Schubertova M, Horak P, Zadrazil J. Emergency situations in rheumatology with a focus on systemic autoimmune diseases. Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub. 2016 Mar;160(1):20-9. doi: 10.5507/bp.2016.002. Epub 2016 Feb 10. Review. PubMed PMID: 26868300.
  3. ↑ Luo Y, Xiao R. Interstitial Lung Disease in Scleroderma: Clinical Features and Pathogenesis. Rheumatology 2011 doi:10.4172/2161- 1149. S1-002. Available from: http://omicsonline.org/ interstitial-lung-disease-in-scleroderma-clinical-features-and- pathogenesis-2161-1149.S1-002.pdf
  4. ↑ Nikpour, M., Baron, M. Mortality in systemic sclerosis: lessons learned from population-based and observational cohort studies. Curr Opin Rheumatol. 2014;26:131–137
  5. ↑ 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
  6. ↑ 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
  7. ↑ Ternacle, J et al. Diruetics in Normotensive Patients with Acute Pulmonary Embolism and Right Ventricular Dilation. Circulation Journal. Vol 77(10) 2013. 2612-618.
  8. ↑ Park M. Diffuse Alveolar Hemorrhage. Tuberc Resp Dis (Seoul) 2013. 74(4):151-162
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