Systemic Sclerosis

An Update

Uwe-Frithjof Haustein, MD, PhD

Disclosures

Lab Med. 2011;42(9):562-572. 

In This Article

Classification

During the last 20 years, the majority of researchers have used the classification into limited vs diffuse cutaneous SSc (dSSc) according to LeRoy and colleagues.[6] These categories are described as follows (Table 1)

  1. 1. More than 50% of SSc patients belong to the limited cutaneous SSc. They have a more insidious onset of illness, a long history of Raynaud's phenomenon and swelling of digits, a more benign course, and a lower incidence of renal involvement and restrictive pulmonary disease with a much better prognosis.[7] There are 45% to 60% of cases that are associated with ACAs. The CREST-syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, teleangiectasia) belongs to this subset.

  2. 2. Patients with dSSc have a short history. These patients often have acral sclerosis, arthritis, Raynaud's phenomenon, and rapid progression of skin involvement including arms and trunk. In addition, they have a higher incidence of renal,[7,8] cardiac,[9] pulmonary disease,[10] and tendon friction rub.[7] Anti-topoisomerase antibodies (ATA) or anti-fibrillarin antibodies (against U3 RNA-associated protein) may be present; ATA exist in about 55% of cases. When associated with anti-RNA polymerase (RNAP) antibodies, patients with diffuse SSc have the shortest survival time and worst prognosis.[11]

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