The American College of Rheumatology (ACR) criteria for the classification of systemic sclerosis require one major criterion or two minor criteria, as follows:
•Major criterion: Proximal scleroderma is characterized by symmetric thickening, tightening, and induration of the skin of the fingers and the skin that is proximal to the metacarpophalangeal or metatarsophalangeal joints. These changes may affect the entire extremity, face, neck, and trunk (thorax and abdomen; see images below). Tightening of the skin in the face, with a characteristic beaklike facies and paucity of wrinkles. Sclerodactyly with digital ulceration, loss of skin creases, joint contractures, and sparse hair.
-Sclerodactyly is characterized by thickening, induration, and tightening of the skin, limited to only the fingers.
-Digital pitting scars or a loss of substance from the finger pad: As a result of ischemia, depressed areas of the fingertips or a loss of digital pad tissue occurs.
-Bibasilar pulmonary fibrosis includes a bilateral reticular pattern of linear or lineonodular densities most pronounced in basilar portions of the lungs on standard chest roentgenography.
-These densities may assume the appearance of diffuse mottling or a honeycomb lung and are not attributable to primary lung disease.
The initial evaluation of patients with suspected systemic sclerosis includes a complete blood count; a comprehensive chemistry panel; and serologic studies, including antinuclear, anticentromere, and antitopoisomerase antibodies. Creatine kinase measurements, erythrocyte sedimentation rate, and C-reactive protein measurements may be useful; elevated results suggest myositis, vasculitis, malignancy, or overlap of systemic sclerosis with another autoimmune disease.
Hinchcliff, Monique, and John Varga. “Systemic sclerosis/scleroderma: a treatable multisystem disease.” American family physician 78.8 (2008):961-968.
Systemic sclerosis is a connective tissue disease associated with autoimmunity, vasculopathy, and fibrosis. The annual incidence is estimated to be 10 to20 cases per 1 million persons. Patients who have systemic sclerosis can be classified into distinct clinical subsets with different patterns ofskin and internal organ involvement, autoantibody production, and patient survival. The most common subsets are limited cutaneous (approximately 60 %) and diffuse cutaneous (approximately 35 %)