Progressive appearance of overlap syndrome together with autoantibodies in a patient with fatal thrombotic microangiopathy.
We describe an extraordinary patient with overlap syndrome (systemic lupus erythematosus, systemic sclerosis, and rheumatoid arthritis) having positive autoantibodies against Sm, double stranded DNA, DNA topoisomerase I, and centromere, together with rheumatoid factor. The patient had multiple organ involvement resulting from thrombotic microangiopathy that mimicked so-called normotensive scleroderma renal crisis, and died mainly of massive pulmonary hemorrhage caused by thrombotic thrombocytopenic purpura. The clinical presentations of the case support the concept of strong associations between disease-specific autoantibodies and clinical features.
Nanke
Y
Y
Institute of Rheumatology, Tokyo Women's Medical University, Japan. ynn@murayama.hosp.go.jp
Akama
H
H
Yamanaka
H
H
Hara
M
M
Kamatani
N
N
D000328:Adult; D001172:Arthritis, Rheumatoid / Q000150:complications* / Q000000981:diagnostic imaging / Q000276:immunology; D001323:Autoantibodies / Q000276:immunology*; D001324:Autoantigens / Q000276:immunology; D002503:Centromere / Q000276:immunology; D004247:DNA / Q000276:immunology; D004264:DNA Topoisomerases, Type I / Q000276:immunology; D017809:Fatal Outcome; D005260:Female; D006651:Histocytochemistry; D006801:Humans; D007668:Kidney / Q000473:pathology; D008168:Lung / Q000473:pathology; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000276:immunology; D016491:Peripheral Vascular Diseases / Q000150:complications / Q000000981:diagnostic imaging / Q000276:immunology / Q000473:pathology; D011697:Purpura, Thrombotic Thrombocytopenic / Q000150:complications* / Q000000981:diagnostic imaging / Q000276:immunology* / Q000473:pathology; D011859:Radiography; D012217:Rheumatoid Factor / Q000276:immunology; D017411:Ribonucleoproteins, Small Nuclear; D012595:Scleroderma, Systemic / Q000150:complications* / Q000276:immunology; D013577:Syndrome; D055517:snRNP Core Proteins
D001323:Autoantibodies; D001324:Autoantigens; D017411:Ribonucleoproteins, Small Nuclear; D055517:snRNP Core Proteins; D004247:DNA; D012217:Rheumatoid Factor; D004264:DNA Topoisomerases, Type I