Erdheim-Chester disease (ECD) is a rare form of non-Langerhans histiocytosis,with noncodified therapeutic management and high mortality. No treatment has yet been shown to improve survival in these patients. We conducted a multicenter prospective observational cohort study to assess whether extraskeletal manifestations and interferon-␣ treatment would influence survival in a large cohort of ECD patients. To achieve this goal, we thoroughly analyzed the clinical presentation of 53 patients with biopsyproven ECD, and we performed a survival analysis using Cox proportional hazard model. Fifty-three patients (39 men and 14 women) with biopsy-proven ECD were followed up between November 1981 and November 2010. Forty-six patients (87%) received interferon-␣ and/or PEGylated interferon-␣. Multivariate survival analysis using Cox proportional hazard model revealed that central nervous system involvement was an independent predictor of death (hazard ratio ‫؍‬ 2.51; 95% confidence interval, 1.28-5.52; P ‫؍‬ .006) in our cohort. Conversely, treatment with interferon-␣ was identified as an independent predictor of survival (hazard ratio ‫؍‬ 0.32; 95% confidence interval, 0.14-0.70; P ‫؍‬ .006). Although definitive confirmation would require a randomized controlled trial, these results suggest that interferon-␣ improves survival in ECD patients. This may be seen as a significant advance, as it is the first time a treatment is shown to improve survival in this multisystemic disease with high mortality. (Blood. 2011;117(10):2778-2782)

The incidence of tuberculosis (TB) is currently increasing in HIV-infected patients living in Africa and Asia, where TB endemicity is high, reflecting the susceptibility of this group of patients to mycobacteria belonging to the TB group. In this population, extension of multiple resistance to anti-tuberculous drugs is also a matter of anxiety. HIV-induced immunosuppression modifies the clinical presentation of TB, resulting in atypical signs and symptoms, and more frequent extrapulmonary dissemination. The treatment of TB is also more difficult to manage in HIV-infected patients, particularly with regard to pharmacological interactions secondary to inhibition or induction of cytochrome P450 enzymes by protease inhibitors with rifampicin or rifabutin, respectively. Finally, immune restoration induced by highly active anti-retroviral therapy (HAART) in developed countries may be responsible for a paradoxical worsening of TB manifestations.

These results question the benefit of very long-term maintenance of HAART in terms of CD4 gain and HIV-1 DNA reduction.

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