Defintion and Classification of Scleroderma, MTCD & UCTD
MTCD & UCTD
Scc 는 원인 불명의 만성질환으로 과도한 결합조직(overproduction and accumulation of collagen and other extracellular matrix proteins, including fibronectin, tenascin, fibrillin-1, and glycosaminoglycans)의 침착으로 인해 skin, the gastrointestinal tract, the respiratory, renal, cardiovascular, and genitourinary systems, as well as numerous vascular structures, 등 여러 내부장기가 침범된다.
이런 침범은 inflammation and progressive tissue fibrosis and occlusion of the microvasculature by excessive production and deposition of types I and III collagens에 의해여 생기는 것으로 알려져 있다.
Scc는 진단을 위해 크게 두가지 criteria로 나눌수 있다.
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
Sclerodactyly includes the above major criterion characteristics but is 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 roentgenograms. These densities may assume the appearance of diffuse mottling or a honeycomb
Defining Unclassifiable Connective Tissue Diseases: Incomplete, Undifferentiated, or Both?
J. Rheumatol. Vol. 32 No. 2 February 2005. Editorial.
Mosca M, Baldini C, Bombardieri S: Undifferentiated connective tissue diseases in 2004. Clin Exp Rheumatol 2004 Jan-Feb
M. Smiti Khanfir. AB0290 EULAR 2006.