Systemic sclerosis sine scleroderma associated with antiphospholipid syndrome.
The antiphospholipid syndrome (APS) can be primary, when it occurs alone, or secondary, when it is associated with another autoimmune disease, mainly systemic lupus erythematosus and rarely other autoimmune diseases. Cases described in literature (Medline 1966 to December 2009) associate the presence of antiphospholipid antibodies with the presence of APS and systemic sclerosis (SS). Currently, however, no cases of the SS variant sine scleroderma with APS have been described. In this study, the authors describe the case of a patient with APS characterised by thrombosis of the retinal veins, in May 2006, the presence of lupus anticoagulant and an anticardiolipin IgG antibody. In May 2007, this patient developed Raynaud's phenomenon, a lack of oesophageal motility and nailfold capillaroscopy with a scleroderma pattern. The patient was positive for the anti-centromere antibody but lacked any evidence of cutaneous thickening or involvement. In summary, the authors describe the first case of a patient with APS associated with SS sine scleroderma.
Leite
Pollyanna d'Ávila
Pd
Rheumatology Division, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, Av. Dr. Arnaldo, 455, 3º anda, sala 3190, São Paulo 01246-903, Brazil.
de Carvalho
Jozélio Freire
JF
D017153:Antibodies, Anticardiolipin / Q000097:blood; D000974:Antibodies, Antinuclear / Q000097:blood; D016736:Antiphospholipid Syndrome / Q000097:blood / Q000150:complications* / Q000175:diagnosis / Q000276:immunology / Q000628:therapy; D015415:Biomarkers / Q000097:blood; D002196:Capillaries / Q000473:pathology*; D005260:Female; D006801:Humans; D016682:Lupus Coagulation Inhibitor / Q000097:blood; D020678:Microscopic Angioscopy; D008875:Middle Aged; D009262:Nails / Q000098:blood supply*; D011928:Raynaud Disease / Q000209:etiology; D012170:Retinal Vein Occlusion / Q000209:etiology; D012595:Scleroderma, Systemic / Q000097:blood / Q000175:diagnosis / Q000209:etiology* / Q000276:immunology / Q000628:therapy
D017153:Antibodies, Anticardiolipin; D000974:Antibodies, Antinuclear; D015415:Biomarkers; D016682:Lupus Coagulation Inhibitor; C538950:anticentromere antibody