The development of clotting factor inhibitor autoantibodies is rarely observed, but can result in a potentially life-threatening haemorrhagic disorder. These acquired inhibitors are most frequently against factor VIII (FVIII), whilst the detection of inhibitors against other clotting factors is rarer. Inhibitors against FVIII and FIX are mostly observed in patients with classical hereditary haemophilia after receiving factor replacement therapy. We report a rare case of acquired FVIII and factor IX (FIX) inhibitors in a single, non-haemophilic patient with chronic hepatitis C virus (HCV) infection who was receiving antiviral treatment with pegylated interferon plus ribavirin. The FVIII and FIX activities were <1% and high titres of inhibitors autoantibodies were found in his serum samples. After achieving a sustained virological response, combined immunosuppression with oral corticosteroids (prednisone) and azathioprine was introduced, eradicating the inhibitory autoantibodies. The development of these inhibitors in association with antiviral therapy for chronic hepatitis C is poorly understood, and particular attention must be given to HCV-infected patients with worsening coagulopathy, particularly if coexistent with treatment related thrombocytopenia.
SUMMARYImmune reconstitution inflammatory syndrome (IRIS) in HIV-infected subjects initiating antiretroviral therapy most commonly involves new or worsening manifestations of previously subclinical or overt infectious diseases. Reports of non-infectious IRIS are much less common but represent important diagnostic and treatment challenges. We report on a 34-year-old HIV-infected male patient with no history of gout who developed acute gouty arthritis in a single joint one month after initiating highly active antiretroviral therapy.
r e v b r a s r e u m a t o l . 2 0 1 5;5 5(2):177-180 w w w . r e u m a t o l o g i a . c o m . b r Interstitial lung disease a b s t r a c t Antisynthetase Syndrome (ASS) is characterized by myositis, Raynaud's phenomenon, fever, interstitial lung disease, mechanic's hands and arthropathy associated with the presence of antibodies against tRNA synthetase, especially anti-Jo-1. This article aims to review the literature on ASS and report two cases where the first is a patient with polymyositis who developed subluxation on the proximal interphalangeal joint of bilateral first right finger after a few years of the disease, associated with pulmonary manifestations and positive anti-JO-1. In the second case, we present a patient with dermatomyositis, who developed a subluxation of the two first fingers, anti-Jo1 positive and chest CT changes, but without clinical evidence of pulmonary involvement. These cases reveal the importance of performing early diagnosis. The authors describe two cases of this rare syndrome, emphasizing the severity of interstitial lung disease and arthritis. Síndrome Antissintetase: relato de dois casos e revisão da literatura Palavras-chave: Síndrome antissintetase Polidermatomiosite Anti-Jo 1 Doença intersticial pulmonar r e s u m o A Síndrome Antissintetase (SAS) é caracterizada por miosite, fenômeno de Raynaud, febre, doença pulmonar intersticial, artropatia e mãos de mecânico associados à presença de anticorpos contra a sintetase do RNAt especialmente anti-Jo-1. Este artigo tem como objetivo revisar a literatura sobre SAS e relatar dois casos, sendo o caso 1 de uma paciente com Polimiosite que desenvolveu, após alguns anos de doença, subluxação da articulação interfalangeana proximal do primeiro quirododáctilo direito, associada a manifestaç ões pulmonares e antiJo-1 positivo. O caso 2 é de uma paciente com Dermatomiosite que evoluiu com subluxação dos dois primeiros quirodáctilos, anti-Jo-1 positivo e alteraç ões pulmonares intersticiais na
Antissintetase Syndrome (ASS) is characterized by myositis, Raunaud's phenomenon, fever, intertitial lung disease, mechanic's hands and arthropathy associated with the presence of antibodies against tRNA synthetase, especially anti-Jo-1. This article aims to review the literature on ASS and report two cases where the first is a patient with polymyositis who developed subluxation on the proximal interphalangeal joint of bilateral first right finger after a few years of the disease, associated with pulmonary manifestations and positive anti-JO-1. In the second case, we present a patient with dermatomyositis, who developed a subluxation of the two first fingers, anti-Jo1 positive and chest CT changes, but without clinical evidence of pulmonary involvement. These cases reveal the importance of performing early diagnosis. The authors describe two cases of this rare syndrome, emphasizing the severity of interstitial lung disease and arthritis.
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