Scleroderma

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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

  • Renal crisis
    • Causes are vessel narrowing and subsequent ischemic kidney disease
    • Leads to hypertensive emergency, acute renal failure, and microangiopathic hemolytic anemia
    • Occurs during first 5 years of the disease
      • Was the most common cause of death prior to ACEi usage[1]
    • Severe renal crisis is life threatening with incidence of 8-10% in Limited and 10-20% in diffuse
      • Similar to TTP/HUS with microangiography
      • 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.[2]
  • Pulmonary
    • Respiratory failure
    • Interstitial lung disease, leading to pulmonary fibrosis
      • 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[3]
    • ARDS
    • Aspiration pneumonitis
    • Pulmonary hypertension
      • Gradually progressive exercise breathlessness
      • Fatigue
      • Hoarseness from nerve palsy caused by dilated pulmonary artery stem
    • Alveolar hemorrhage
  • Cardiac
    • Acute heart failure
    • Cardiopulmonary complications are the most common causes of scleroderma-related death[4], with cardiac causes[5] and pulmonary fibrosis[6] being the most common.
    • Anginal pain from right ventricle ischemia and low cardiac output

Differential Diagnosis

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 hypercapnea (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 hypercapnea, 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
    • Ursodial 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