ABSTRACT
OBJECTIVES: To describe the clinical presentation, management and prognosis of patients diagnosed with both primary Sjögren's syndrome (pSS) and anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). METHODS: French nation-wide survey completed by a systematic literature review. RESULTS: This work identified 7 new cases of coexisting pSS and AAV: 2 microscopic polyangiitis (MPA), 2 granulomatosis with polyangiitis (GPA), 2 anti-myeloperoxidase (MPO)-ANCA renal-limited AAV, and 1 eosinophilic granulomatosis with polyangiitis (EGPA). The systematic literature search identified 15 previously published cases. Among the 22 patients, 19 were females. Mean age at diagnosis of AAV was 63.9±9.8years. All individuals with available information experienced at least one extra-glandular manifestation attributable to pSS. p-ANCA with anti-MPO specificity were found in 76.2% (16/21), c-ANCA with anti-PR3 specificity in 14.3% (3/21) and isolated c-ANCA in 13.6% (3/22). Vasculitis involved kidneys (n=13), lungs (n=8), skin (n=6), peripheral nerves (n=5), central nervous system (n=2), small bowel (n=1), muscle (n=1), ear chondritis (n=1) and sinuses (n=1). The mean AAV follow-up was 73.5 (±120.0) months. While on treatment, disease remission occurred in 77.3% of cases, and one death was reported in the first 6months after diagnosis. CONCLUSION: This work shows that AAV may occur in patients with pSS. These are most commonly p-ANCA associated vasculitis with anti-MPO specificity. AAV may reveal an underlying pSS or arise during its evolution, but did not precede pSS in any of these cases. AAV occurrence appears to be correlated with extra-glandular manifestations of pSS.
Subject(s)
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/immunology , Sjogren's Syndrome/complications , Humans , Kidney Diseases/complications , PrognosisABSTRACT
French Guiana is the region of France where the HIV epidemic is most prevalent. To determine the risk factors for being lost for follow-up, we followed a cohort of 1,213 patients between 1992 and 2002 and determined which variables were related to two definitions of being lost to follow-up: permanently disappearing from HIV clinics and coming back after more than 1 year of missed appointments. The incidence rate for permanent follow-up interruption was 17.2 per 100 person-years. The median time to lost to follow-up was 4.3 years (interquartile range = 1.4-8.4 years). Cox modeling showed that the younger age groups, foreigners, patients with initial CD4 counts at the time of HIV diagnosis less than 500/mm3, and patients followed before the availability of highly active antiretroviral therapy (HAART) were significantly more likely to be permanently lost to follow-up, suggesting that some of the patients may have died. When looking at temporary loss to follow-up, younger age groups, untreated patients, patients consulting before the availability of HAART, and patients with CD4 counts more than 500/mm3 were more likely to not come back for a period of more than 1 year.
Subject(s)
Continuity of Patient Care/statistics & numerical data , HIV Infections/epidemiology , HIV Infections/prevention & control , Treatment Refusal/statistics & numerical data , Adult , Antiretroviral Therapy, Highly Active , CD4 Lymphocyte Count , Continuity of Patient Care/classification , Female , French Guiana/epidemiology , HIV Infections/etiology , Humans , Male , Middle Aged , Proportional Hazards Models , Risk FactorsABSTRACT
To determine whether the initiation of highly active antiretroviral therapy (HAART) had any influence on the incidence of disseminated histoplasmosis, a retrospective cohort study was performed on 1551 patients followed for up to 12 years. After controlling for CD4 counts, age, and sex, patients taking HAART for 2 months or less were more likely to develop disseminated histoplasmosis than untreated patients (respectively, hazard ratio, 3.7 [95% confidence interval, 1.57-8.7]; P = 0.003). In contrast, after 6 months of HAART, treated patients were less likely to develop disseminated histoplasmosis than untreated patients (hazard ratio, 0.6 [95% confidence interval, 0.37-0.98], P = 0.04). This increased incidence suggests that the initiation of HAART and the subsequent immune reconstitution may reveal undiagnosed latent disseminated histoplasmosis.
Subject(s)
Antiretroviral Therapy, Highly Active , HIV Infections/complications , HIV Infections/drug therapy , Histoplasmosis/epidemiology , Cohort Studies , Humans , Incidence , Retrospective Studies , Statistics as Topic , Time FactorsABSTRACT
Risk factors for delayed HIV diagnosis in French Guiana were studied in 1952 patients between 1992 and 2003. At the time of diagnosis, 30% of patients had less than 200 CD4 lymphocytes/mm3; age, male sex, and foreign nationality were independently associated with a low CD4 cell count. The availability of highly active antiretroviral therapy was not associated with an earlier HIV diagnosis. Promoting HIV information and testing should be done in several languages to reach minorities.
Subject(s)
Developing Countries , HIV Infections/diagnosis , Adult , Age Factors , Antiretroviral Therapy, Highly Active , CD4-Positive T-Lymphocytes , Female , French Guiana , HIV Infections/drug therapy , HIV Infections/immunology , Humans , Male , Middle Aged , Patient Education as Topic , Pregnancy , Risk Factors , Sex Factors , Time Factors , Transients and MigrantsABSTRACT
We aimed to identify prognostic factors for AIDS-associated disseminated histoplasmosis. In a multivariate analysis, we found that dyspnea, a platelet count of <100,000 platelets/mm3, and lactate dehydrogenase levels of >2 times the upper limit of the normal range were significantly independently associated with the death of the patient during the first 30 days of antifungal treatment.
