Skip to Main Content



Heterogeneous disorders that share certain common features, including inflammation of skin, joints, and other structures rich in connective tissue; as well as altered patterns of immunoregulation, including production of autoantibodies and abnormalities of cell-mediated immunity. While distinct clinical entities can be defined, manifestations may vary considerably from one pt to the next, and overlap of clinical features between and among specific diseases can occur.



SSc is a multisystem disorder characterized by thickening of the skin (scleroderma) and distinctive involvement of multiple internal organs (chiefly GI tract, lungs, heart, and kidney). Pathogenesis unclear; involves immunologic mechanisms leading to vascular endothelial damage and activation of fibroblasts.


  • Cutaneous: edema followed by fibrosis of the skin (chiefly extremities, face, trunk); telangiectasia; calcinosis; Raynaud’s phenomenon

  • Arthralgias and/or arthritis

  • GI: esophageal hypomotility; intestinal hypofunction, gastric antral vascular ectasia (GAVE)

  • Pulmonary: interstitial lung disease (ILD), pulmonary arterial hypertension, alveolitis

  • Cardiac: pericarditis, cardiomyopathy, conduction abnormalities

  • Renal: hypertension; renal crisis/failure

Two distinct subsets can be identified:

  1. Diffuse cutaneous SSc: rapid development of symmetric skin thickening of proximal and distal extremity, face, and trunk. At high risk for development of visceral disease early in course.

  2. Limited cutaneous SSc: often have long-standing Raynaud’s phenomenon before other features appear; skin involvement limited to fingers (sclerodactyly), extremity distal to elbows, and face; generally associated with better prognosis but can be associated with pulmonary arterial hypertension; a subset of limited SSc has features of CREST syndrome (calcinosis, Raynaud’s, esophageal dysmotility, sclerodactyly, telangiectasias).


  • History and physical examination with particular attention to blood pressure (heralding feature of renal disease).

  • Laboratories: ESR, ANA (anticentromere pattern associated with limited SSc), specific antibodies may include anti-topoisomerase I (Scl-70), (UA). An increased range of autoantibodies correlating with specific clinical features have become recognized (Table 353-3, HPIM-20).

  • Radiographs: CXR, barium swallow if indicated, hand x-rays may show distal tuft resorption and calcinosis.

  • Additional studies: ECG, echo, PFT, consider skin biopsy.


TREATMENT Systemic Sclerosis

  • Education regarding warm clothing, smoking cessation, anti-reflux measures.

  • Calcium channel blockers (e.g., nifedipine) useful for Raynaud’s phenomenon. Other agents with potential benefit include sildenafil, losartan, nitroglycerin paste, fluoxetine, bosentan, digital sympathectomy.

  • ACE inhibitors: particularly important for controlling hypertension and limiting progression of renal disease.

  • Antacids, H2 antagonists, omeprazole, and metoclopramide may be useful for esophageal reflux.

  • D-Penicillamine: controversial benefit to reduce skin thickening and prevent organ involvement; no advantages to using doses >125 mg every other day.

  • Glucocorticoids: no efficacy in slowing progression of SSc; indicated for inflammatory myositis or pericarditis; high doses may be associated with development of renal crisis.

  • Cyclophosphamide: improves lung function and survival ...

Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.