To the Editor.—
Progressive systemic sclerosis (PSS) is a multisystem disease characterized by fibrosis of the skin and of internal organs such as the esophagus. The fibrosis of PSS may be associated with excessive accumulation of collagen. Its cause is unknown, although the detection of autoantibodies has suggested an autoimmune disease.1 Thivolet et al2 and Delbarre et al3 reported systemic improvement of patients with PSS who received blood coagulation factor XIII (factor XIII). They attributed this benefit to the removal of the excess collagen by factor XIII. We gave factor XIII to two patients with PSS and obtained interesting results.
Report of Cases.—
Two patients with a generalized type of PSS, a 71-year-old woman in Japan (case 1) and a 26-year-old woman (case 2), were, respectively, given factor XIII, in daily doses of 480 U in an intravenous drip infusion for three weeks.To determine the effect
Maekawa Y, Nogita T, Yamada M. Favorable Effects of Plasma Factor XIII on Lower Esophageal Sphincter Pressure of Progressive Systemic Sclerosis. Arch Dermatol. 1987;123(11):1440–1441. doi:10.1001/archderm.1987.01660350034012
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