Systemic Sclerosis

  1. What is systemic sclerosis?
    Systemic sclerosis is a disease of unknown cause. The hallmarks of this condition are microangiopathy and fibrosis of the skin and visceral organs. Common pathophysiologic findings in affected patients include endothelial cell dysfunction, abnormal fibroblast function, and autoantibody production.
  2. What is Limited and Diffuse Cutaneous Systemic Sclerosis?
    Limited cutaneous systemic sclerosis (lcSSc) is characterized by skin disease that does not progress proximal to the elbows or knees. A subset of this condition is the CREST (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome. Diffuse cutaneous systemic sclerosis (dcSSc) is characterized by skin thickening that involves areas proximal to the elbows and/or knees.

    • Patients with lcSSc are more likely to develop pulmonary hypertension in the absence of other pulmonary manifestations.
    • Patients with dcSSc are more likely to develop interstitial lung disease (ILD) and/or renal disease.
  3. What is Systemic Sclerosis Sine Scleroderma?
    is characterized by visceral disease in the absence of cutaneous involvement.
  4. How is systemic sclerosis diagnosed?
    • Diagnosis of systemic sclerosis is established in patients with sclerodermatous skin changes (tightness, thickening, and nonpitting induration) that have sclerodactyly and skin disease that extends proximal to the metacarpophalangeal joints.
    • In the absence of these findings, diagnosis of systemic sclerosis may be established in patients with two of the following features: sclerodactyly (sclerodermatous skin changes limited to the fingers and toes), digital pitting (soft-tissue defects and scarring in the pulp space of the distal phalanges), or basilar fibrosis visible on chest radiography
    • Serologic markers of disease activity in systemic sclerosis have not yet been identified.
  5. What medication can potentially alleviate skin thickening in a pt with systemic sclerosis?
  6. What are appropriate interventions for a pt with systemic sclerosis who is now presenting with UGI discomfort?
    Potential complications of esophageal disease in patients with systemic sclerosis include esophagitis, esophageal stricture, Barrett esophagus, and aspiration pneumonitis. Management of these conditions includes the use of prokinetic agents such as metoclopramide, erythromycin, and octreotide. In addition, gastric acid suppression with proton pump inhibitors is indicated for nearly all patients with systemic sclerosis. Patients with systemic sclerosis also have an increased risk for the development of Barrett esophagus, and screening for this condition is appropriate.
  7. What are the principal pulmonary involvement of systemic sclerosis?
    ILD and PAH
  8. In a pt with systemic sclerosis and dyspnea, dry cough, and decreased exercise tolerance, what is an appropriate intervention
    High-resolution CT reveals ground-glass and reticular linear opacities, subpleural cysts, and honeycombing in patients with advanced disease.
  9. What medication may improve the pulmonary symptoms in a pt with systemic sclerosis, who now present with a diagnosis of ILD?
    Oral cyclophosphamide
  10. What is a the best intervention to evaluate possible pulmonary arterial hypertension (PAH) in a pt with systemic sclerosis?
    the gold standard for determining pulmonary artery pressure is direct measurement by right heart catheterization.
  11. What is Scleroderma renal crisis (SRC)?
    • Scleroderma renal crisis (SRC) occurs almost exclusively in patients with early dcSSc. This condition is characterized by the acute onset of severe hypertension, renal failure, and microangiopathic hemolytic anemia. The presence of a pericardial effusion is associated with an increased risk of SRC. Use of corticosteroid therapy in the management of inflammatory disease manifestations such as myopathy is classically associated with normotensive renal failure and is also a risk factor for SRC.
    • SRC is a medical emergency, and patients in whom this diagnosis is established should be admitted to the hospital immediately for aggressive blood pressure control.
    • ACE inhibitor therapy is currently considered the cornerstone of therapy for this condition.
  12. What is Morphea?
    Morphea involves only the skin in the absence of other systemic manifestations of systemic sclerosis.
  13. What is Eosinophilic fasciitis?
    Eosinophilic fasciitis is characterized by peripheral eosinophilia accompanied by woody induration of the skin that involves the extremities but usually spares the hands and face. Patients with eosinophilic fasciitis do not have Raynaud phenomenon or other features of systemic sclerosis (Table 12 ). Corticosteroid therapy usually is effective in patients with this condition.
Card Set
Systemic Sclerosis
Skin and visceral sclerosis