The clinical presentation of visceral leishmaniasis shares similarities with other geographically specific infectious diseases associated with AIDS in terms of relapsing course and atypical presentation. However, visceral leishmaniasis has not, until now, been included in the AIDS case definition. The aim of this study was to describe the clinical features and determinants for relapse and case-fatality of visceral leishmaniasis in HIV-infected patients from a Spanish Mediterranean area. A chart review was conducted in 16 hospitals in the autonomous communities of Valencia and Murcia (Spain). From 1988 to 2001, a total of 228 episodes of visceral leishmaniasis were diagnosed in 155 HIV-infected patients by the detection of amastigotes in bone marrow aspirates or in other tissue samples. Most patients had advanced HIV disease, with a median CD4(+) lymphocyte cell count of 55 cells x 10(9) l, and 56% of them had a previous AIDS-indicator disease. The median duration of follow-up was 8.4 months. HIV-infected patients with visceral leishmaniasis presented with fever (76%), hepatomegaly (77%), splenomegaly (78%), and varying degrees of cytopenias. Leishmania was detected in atypical sites in 22 (14%) patients. A total of 37 (24%) patients had a relapse of visceral leishmaniasis. Female gender was a risk factor for relapse, whereas administration of secondary prophylaxis for visceral leishmaniasis and a completed therapy for visceral leishmaniasis were protective factors against relapse. A total of 86 (54%) patients died. Independent determinants for survival were CD4(+) lymphocyte cell count, completed therapy for leishmania, and secondary prophylaxis for visceral leishmaniasis. The findings show that, in HIV-infected patients, visceral leishmaniasis occurs in late stages of HIV disease and often has a relapsing course. Secondary prophylaxis reduces the risk of relapse. Visceral leishmaniasis in the HIV-infected population should be included in the CDC clinical category C for the definition of AIDS in the same way that other geographically specific opportunistic infections are included.

The aim of this study was to describe the clinical characteristics of ANCA-associated vasculitides (AAV) at presentation, in a wide cohort of Spanish patients, and to analyze the impact of the vasculitis type, ANCA specificity, prognostic factors, and treatments administered at diagnosis, in the outcome.A total of 450 patients diagnosed between January 1990 and January 2014 in 20 Hospitals from Spain were included. Altogether, 40.9% had granulomatosis with polyangiitis (GPA), 37.1% microscopic polyangiitis (MPA), and 22% eosinophilic granulomatosis with polyangiitis (EGPA). The mean age at diagnosis was 55.6 ± 17.3 years, patients with MPA being significantly older (P < 0.001). Fever, arthralgia, weight loss, respiratory, and ear–nose–throat (ENT) symptoms, were the most common at disease onset. ANCAs tested positive in 86.4% of cases: 36.2% C-ANCA-PR3 and 50.2% P-ANCA-MPO. P-ANCA-MPO was significantly associated with an increased risk for renal disease (OR 2.6, P < 0.001) and alveolar hemorrhage (OR 2, P = 0.010), while C-ANCA-PR3 was significantly associated with an increased risk for ENT (OR 3.4, P < 0.001) and ocular involvement (OR 2.3, P = 0.002). All patients received corticosteroids (CS) and 74.9% cyclophosphamide (CYC). The median follow-up was 82 months (IQR 100.4). Over this period 39.9% of patients suffered bacterial infections and 14.6% opportunistic infections, both being most prevalent in patients with high-cumulated doses of CYC and CS (P < 0.001). Relapses were recorded in 36.4% of cases with a mean rate of 2.5 ± 2.3, and were more frequent in patients with C-ANCA-PR3 (P = 0.012). The initial disease severity was significantly associated with mortality but not with the occurrence of relapses. One hundred twenty-nine (28.7%) patients (74 MPA, 41 GPA, 14 EGPA) died. The mean survival was 58 months (IQR 105) and was significantly lower for patients with MPA (P < 0.001). Factors independently related to death were renal involvement (P = 0.010), cardiac failure (P = 0.029) and age over 65 years old (P < 0.001) at disease onset, and bacterial infections (P < 0.001). An improved outcome with significant decrease in mortality and treatment-related morbidity was observed in patients diagnosed after 2000, and was related to the implementation of less toxic regimens adapted to the disease activity and stage, and a drastic reduction in the cumulated CYC and CS dose.