A Comprehensive Review of Sarcoidosis Treatment for Pulmonologists.

Due to frequent lung involvement, the pulmonologist is often the reference physician for management of sarcoidosis, a systemic granulomatous disease with a heterogeneous course. Treatment of sarcoidosis raises some issues. The first challenge is to select patients who are likely to benefit from treatment, as sarcoidosis may be self-limiting and remit spontaneously, in which case treatment can be postponed and possibly avoided without any significant impact on quality of life, organ damage or prognosis. Systemic glucocorticosteroids (GCs) are the drug of first choice for sarcoidosis. When GCs are started, there is a > 50% chance of long-term treatment. Prolonged use of prednisone at > 10 mg/day or equivalent is often associated with severe side effects. In these and refractory cases, steroid-sparing options are advised. Antimetabolites, such as methotrexate, are the second-choice therapy. Biologics, such as anti-TNF and especially infliximab, are third-choice drugs. The three treatments can be used concomitantly. Regardless of whether treatment is started, the clinician needs to organize regular follow-up to monitor remissions, flares, progression, complications, toxicity and relapses in order to promptly adjust the drugs used.

Regulatory T cell monitoring in severe eosinophilic asthma patients treated with mepolizumab.

Severe eosinophilic asthma (SEA) has been associated with T-helper type 2 (Th2) inflammatory response. A good understanding of T cell functions in asthma is important for therapy, especially in the choice of biological treatments for severe cases. Mepolizumab, an IL-5 antagonist, is indicated for the treatment of severe asthma. Regulatory T cells (Tregs) suppress inflammation by secreting cytokines that inhibit Th2 cell proliferation. We investigated peripheral Treg, CD4, CD8, CD19 and NK cell percentages and their relationship to clinical and functional parameters, including peripheral eosinophils, before and after anti-IL5 treatment. Subjects were 14 adult SEA patients (9 male, 54.1 ± 11.6 years), treated with mepolizumab, and 10 controls. T cells (CD4 and CD8), CD19, NK and Tregs were evaluated by flow cytometry. Comparison of lung function parameters before and after treatment with mepolizumab (T0 and T1) showed an increase in FEV1, FEV1/FVC ratio and a reduction in blood eosinophil percentages. CD8 and CD16/56+ CD3+ were significantly higher in SEA patients than controls (P = .04 and P = .03, respectively). A decrease in CD45+, CD8 + and CD16/56+ CD3+ cell percentages was observed between T0 and T1 (P = .02, P = .04, P = .03, respectively). A significant increase in Treg percentages (P = .0001) was recorded between T0 and T1. Mepolizumab therapy was found to modulate immune response, restoring immune balance in patients with SEA.

Tryptase concentrations in bronchoalveolar lavage from patients with chronic eosinophilic pneumonia.

In order to characterize BAL (bronchoalveolar lavage) in CEP (chronic eosinophilic pneumonia) and to investigate the possible role of mast cells and tryptase in the pathogenesis of this interstitial disease, cells and tryptase levels were determined in BAL of patients with CEP and in a group of healthy controls. The results show that a statistically significant increase in tryptase concentration was found in patients with CEP compared with the healthy controls. This is the first report that shows an increase in tryptase levels in CEP and could reflect higher mast cell activation as well as larger mast cell populations in the lungs of these patients. These results strongly support the involvement of mast cells and eosinophils in the immunopathogenesis of CEP.

Rare infiltrative lung diseases: a challenge for clinicians.

Rare diffuse infiltrative lung diseases are a challenge for clinicians, radiologists, and pathologists for at least three reasons: (a) their low incidence and prevalence hamper the acquisition of expertise and frequently the diagnosis is delayed; (b) therapeutic actions are mainly empirical and based on steroid use, and (c) pathogenetic events are difficult to explain and only recently new therapeutic measures taking advantage of innovative genetic and/or immunopathogenetic studies have been suggested. In this review rare diffuse lung disorders are briefly discussed (pulmonary alveolar proteinosis, inherited lipidoses, acute eosinophilic pneumonia, amyloidosis, pulmonary ossification, pulmonary alveolar microlithiasis). The list is obviously not exhaustive and arbitrarily chosen. The intent is, however, to emphasize that in this difficult field multidisciplinary expertise and the knowledge of the most recent pathogenetic mechanisms have the main role in diagnosis and treatment.

Disseminated basidiobolomycosis in an immunocompetent woman.

Basidiobolomycosis is a chronic subcutaneous infection of the trunk and limbs due to Basidiobolus ranarum. The disease is well known in tropical areas, although recent cases of gastrointestinal basidiobolomycosis have also been reported in Arizona. We describe a young immunocompetent women who had presented with eosinophilia and lung infiltrates. She subsequently died, and diagnosis of disseminated basidiobolomycosis was made on the basis of histological features at autopsy.