Epidemiology and geographic clustering of Erdheim-Chester disease in Italy and France.

This geoepidemiological study, performed in Italy and France, shows that Erdheim-Chester disease is increasingly diagnosed and cases cluster in specific geographic areas, namely southern Italy and central France. Disease frequency inversely correlates with the Human Development Index.

Does transbronchial lung cryobiopsy give useful information in asthmatic patients?

INTRODUCTION: Lung biopsy in asthmatic patients is justified in case of atypical presentations of asthma, when other differential diagnoses, such as hypersensitivity pneumonitis or eosinophilic granulomatosis with polyangiitis, could be possible or for research purposes. AIM: We aim to describe the utility and the safety of TBLC (transbronchial lung cryobiopsy) in asthmatic patients, providing data on the pathological changes occurring in the airways and in the lung parenchyma. METHODS: We reviewed asthmatic patients that underwent TBLC, that eventually had only a final diagnosis of asthma. RESULTS: Three patients were detected. TBLC described pathological abnormalities in peribronchiolar and alveolar spaces already well identified with SLB (surgical lung biopsy); the pathological information provided could be useful to better understand the pathobiology of the disease. Finally, we had no complications, confirming a satisfactory safety profile of TBLC. CONCLUSION: We suggest the potential role of TBLC in asthmatic patients: its safety and its acceptable diagnostic accuracy lead to consider this procedure instead of SLB when histological changes in lung parenchyma are needed for the differential diagnosis. Furthermore, TLBC could be useful for research in the pathobiology of asthma and severe asthma.

[Intravenous immunoglobulin treatment in autoimmune diseases]. / La terapia con immunoglobuline endovena nelle malattie autoimmuni.

Our experience with intravenous immunoglobulin (IVIg) in autoimmune diseases is reported. In 16 subjects with polymyositis and dermatomyositis, IVIg has been given in case of steroid resistance or dependency. Subjects treated with IVIg achieved a clinical and functional remission in a higher percentage (81%), that was maintained after a mean five year follow-up period (p < 0.001), as compared to control group. In twelve subjects with new-onset Churg-Strauss disease, IVIg was added to standard treatment. In these patients, IVIg permitted to achieve a long-term stable remission with a good functional recovery with lower incidence of the steroid-associated side effects. In conclusion, IVIg can be safely employed in subjects with immune-mediated diseases, even in those with severe and refractory disease.

Usefulness of Medical Thoracoscopy in the Management of Pleural Effusion Caused by Chronic Renal Failure.

INTRODUCTION: Although pleural effusion (PE) can be caused by several pathologies like congestive heart failure, infections, malignancies, and pulmonary embolism, it is also a common finding in chronic kidney disease (CKD). Diagnostic thoracentesis is of limited value in the differential diagnosis, and the role of more invasive investigations like medical thoracoscopy (MT) is still unclear. AIM: To evaluate the usefulness of MT in unexplained PE in CKD. MATERIALS AND METHODS: In the electronic database of our Institution, we retrospectively searched for patients with CKD who underwent MT for unexplained PE between January 2008 and August 2016. RESULTS: Ten patients were included in the present study. The average age was 72.4 years, the male:female ratio 9:1 and the average blood creatinine value 5.96 mg/dL. The average follow-up was 18 months.A thoracentesis showed an exudate was found in 9 patients and in 1 case pleural fluid characteristics were not recorded for technical reasons; in none of them the cytologic or microbiological analyses were considered diagnostic.The clinical suspicion was a neoplastic (5) or an infectious disease (5). In 4 patients with recurrent PE, MT was performed to obtain talc pleurodesis.No immediate procedure-related complications were recorded; 1 patient developed empyema after 2 months. In 6 cases final diagnosis was chronic uremic pleuritis, hydrothorax in 2, and chronic lymphocytic pleurisy in 2. CONCLUSIONS: MT represents a safe and effective diagnostic and therapeutic procedure in patients with CKD, that itself is a common cause of exudative effusion, and those patients may not require MT.