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MR Vol.10 No.4 indexに戻る
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MODERN RHEUMATOLOGY
Vol.10 No.4 |
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Thrombotic thrombocytopenic purpura in a patient
with rapidly progressive scleroderma
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| T. Bohgaki1, A. Notoya1, M. Mukai1, M.
Kohno1 |
| (1)Division of Clinical Immunology and Hematology,
Department of Medicine, Sapporo City General Hospital, N-11, W-13,
Chuo-Ku, Sapporo 060-8604, Japan |
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| Abstract |
| Abstract A 57-year-old man presented with palpitations,
shortage of breath on exertion, and rapidly progressive scleroderma.
On admission, a computed tomographic scan of his lung showed active
interstitial pneumonia. We treated him with d-penicillamine and intravenous
pulse methylprednisolone. After this treatment, severe abdominal
pain, microangiopathic hemolytic anemia, thrombocytopenia, and progressive
renal involvement appeared. We diagnosed him as having systemic sclerosis
(SSc) complicated by thrombotic thrombocytopenic purpura. At postmortem,
thromboses of capillaries, arterioles, and small arteries were found
in several organs. As well as the differential diagnosis of SSc with
thrombocytopenia, microangiopathic hemolytic anemia (MAHA), and renal
involvement, we diagnosed scleroderma renal crisis (SRC), normotensive
renal crisis (NRC), and SSc complicated by TTP. Typical SRC and NRC
were excluded because his blood pressure was in the normal range
without elevation of plasma renin activity or azotemia over his clinical
course. Although distinguishing TTP from renal crisis is difficult,
an evaluation of ultra-large multimers of von Willebrand factor (UL-vWF)
concentration may be helpful in these situations. |
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| Key words |
| Key words Microangiopathic hemolytic anemia ・ Normotensive renal
crisis ・ Scleroderma renal crisis ・ Systemic sclerosis ・ Thrombotic
thrombocytopenic purpura |
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