Asociación entre infección por virus de la Hepatitis B y Poliarteritis nodosa / Association between Hepatitis B virus infection and Polyarteritis Nodosa

Introducción: la Poliarteritis nodosa (PAN) se define como una vasculitis necrotizante que compromete a las arterias de mediano calibre, y causa un compromiso sistémico. Existe una asociación entre la PAN y la infección por el virus de la Hepatitis B, en relación principalmente con el depósito de inmunocomplejos virales circulantes. Las manifestaciones clínicas son inespecíficas y requiere un alto índice de sospecha. Se han usado varios regímenes de inmunosupresores (corticoides-ciclofosfamida) plasmaféresis y drogas antivirales, con respuestas variables. Objetivo: presentación de un caso clínico de PAN asociado con infección por virus de Hepatitis B, y revisión de la literatura. Caso clínico: paciente masculino de 40 años, con manifestaciones severas de Poliartritis nodosa, asociado con infección por Hepatitis B. Conclusiones: la PAN es una enfermedad poco frecuente, la asociación con infección por Hepatitis B está bien documentada y las estrategias terapéuticas difieren de las de la PAN clásica. Es una patología potencialmente fatal cuando no se reconoce y trata adecuadamente

Backgraound: Polyarteritis nodosa (PAN) is defined by a necrotizing vasculitis of medium sized arteries that causes a systemic inflammatory disease. There is an association between PAN and hepatitis B virus (HBV) infections, related mostly with viral circulting immune complexes. Clinical manifestations are not specific and require a high index of suspicion. Several immunosuppressive regimens (corticoids-ciclophosphamide), plasmapheresis and antiviral drugs have been used with varying response. Objective: Report of a clinical case of PAN associated with Hepatitis B virus infection and review of the literature. Case-reporte: A 40 year old man with severe manifestations of Polyarteritis nodosa, associated with Hepatitis B infection. Conclusions: PAN is uncommon disease, the association with hepatitis B virus infection is well documented, and the treatment strategies differs from the classic PAN. It is a potentially fatal disease when unrecognized and untreated

Manifestaciones clínicas de la granulomatosis de Wegener y la poliangeítis microscópica en Santiago-Chile, 1990-2001 / Clinical features of Wegener granulomatosis and microscopic polyangiitis in Chilean patients

Background: Systemic vasculitis are a group of heterogeneous diseases characterized by inflammation and necrosis of blood vessel walls. The etiology is not known, but geographic and environmental factors are implicated. Aim: To describe the clinical features of microscopic polyangiitis (MPA) and Wegener's granulomatosis (WG) in a Chilean cohort of patients. Patients and methods: Retrospective review of the medical records of 123 patients with the diagnosis of systemic vasculitis (65 MPA and 58 WG), seen from 1990 to 2001. The diagnosis were made based on the American College of Rheumatology and Chapel Hill criteria. Results: The mean follow-up for MPA was 15 months (1-120) and for WG, 20 months (1-120). The median age (years) at diagnosis for MPA was 61 (19-82) and WG 50 (20-82). Gender distribution was similar in both groups (male: 68percent and 57percent respectively).The main clinical features in the MPA group were renal involvement (68percent), peripheral nervous system involvement (57percent), pulmonary hemorrhage (28percent), and skin disease (32percent). In the WG group were alveolar hemorrhage (62percent), renal involvement (78percent), paranasal sinus involvement (57percent), and ocular disease (26percent). In both, creatinine levels above 2.0 mg/dl were associated with a higher mortality (p< 0.01). ANCA by immunofluorescence was performed in 56 MPA patients (75percent had pANCA, 4percent had cANCA and 21percent were ANCA negative) and in 55 WG patients (17percent had pANCA, 79percent had cANCA and 4percent were ANCA negative). Global mortality was 18percent and 17percent respectively, and the most common causes of death were infections. Conclusions: The clinical features of our patients are similar to other published data. In our WG and MPA patients the main predictor for death was a serum creatinine above 2 mg/dl.

Anti-neutrophil cytoplasmic antibody (ANCA) in necrotising vasculitides with renal involvement.

Anti-neutrophil cytoplasmic antibody (ANCA) was detected in sera of 20 out of 32 patients with necrotising vasculitides involving the kidneys and other organs. It was of high titre and cytoplasmic type in Wegener's granulomatosis (8/9); and of low titre and cytoplasmic or perinuclear type in systemic polyarteritis (6/12), renal limited polyarteritis (5/9) and pulmonary renal syndrome (1/2). It fell to low or undetectable levels on clinical improvement or remission with cyclophosphamide therapy. Its detection was helpful in diagnosis and management of vasculitides with renal and extrarenal manifestations.