Diffuse bronchiectasis and airflow obstruction in granulomatosis with polyangiitis.

Parenchymal lung nodes and diffuse intra-alveolar hemorrhage are the archetypal pulmonary manifestations of Granulomatosis with Polyangiitis (GPA). The occurrence of diffuse bronchiectasis and airflow obstruction during GPA is unusual. We report here 3 patients with GPA who developed diffuse bronchiectasis during follow-up. The airflow obstruction seemed then to evolve independently from the GPA itself and ultimately led to respiratory insufficiency. Bronchiectases promoted the occurrence of opportunistic infections, especially with atypical mycobacteria. (Sarcoidosis Vasc Diffuse Lung Dis 2018; 35: 81-84).

[Interleukin-1, inflammasome and autoinflammatory diseases]. / Interleukine-1, inflammasome et maladies auto-inflammatoires.

Interleukin-1 is a major cytokine of innate immunity and inflammation. It exerts various systemic effects during the inflammatory response, such as fever induction, thrombopoiesis and granulopoiesis, or leukocyte recruitment. Its involvement has been demonstrated in many inflammatory-mediated diseases, such as diabetes or gout. Moreover, interleukin-1 plays a pivotal role in some autoinflammatory diseases, such as cryopyrinopathies or familial Mediterranean fever. In these diseases, a constitutional defect of the inflammasome, a protein complex responsible for the activation of interleukin-1, explains the hypersecretion of interleukin-1. Other autoinflammatory diseases have a more complex pathophysiology involving deregulation of the interleukin-1 pathway, upstream or downstream of the inflammasome, or through more complex mechanisms. In this review, we are detailing the synthesis, the activation, the signalling, and the regulation of interleukin-1. We then describe the autoinflammatory diseases or related-diseases where the pathological role of interleukin-1 has been demonstrated.

[Inherited primitive and secondary polycythemia]. / Polyglobulies héréditaires primitives et secondaires.

Myeloproliferative disorders and secondary polycythemia cover most of the polycythemia cases encountered in daily practice. Inherited polycythemias are rare entities that have to be suspected when the classical causes of acquired polycythemia have been ruled out. Recent advances were made in the understanding of these pathologies, which are still little known to the physicians. This review reports the state of knowledge and proposes an algorithm to follow when confronted to a possible case of inherited polycythemia.

[Complicated course of eosinophilic gastroenteritis: a case report and literature review]. / Complications de la gastroentérite à éosinophiles: une observation et revue de la littérature.

INTRODUCTION: Eosinophilic gastroenteritis is an unusual disease characterized by an eosinophilic infiltration of the gastrointestinal tract. The esophageal location of this disorder is uncommon and is usually revealed by dysphagia. Diagnosis is obtained by histology during endoscopy after exclusion of differential diagnosis. Treatment is based on systemic corticosteroids, which improve dramatically symptoms and endoscopic lesions. CASE REPORT: We report an 88-year-old man who presented eosinophilic gastroenteritis with esophageal injury complicated by gastrointestinal haemorrhage and fistule. CONCLUSION: Eosinophilic gastroenteritis may have a potentially unfavourable outcome. The treatment of complicated forms is not codified and often empirical.

[Non-specific bronchoprovocation with metacholine in children before and during sugar cane burning in Catanduva-SP] / Broncoprovocação inespecífica com metacolina em crianças antes e durante a queima dos canaviais em Catanduva-SP.

OBJECTIVE: Evaluate the influence of sugar cane burning on BHR of asthmatic and "normal" control children by metacholine bronchoprovocation tests; and verify if interferes in pulmonary function tests.METHODS: Twenty-two asthmatic children (A) aged from 7 to 14 years old, and twelve "normal" control children (C), aged from 8 to 13 years, were submitted to non-specific bronchoprovocation test with methacholine before and during the sugar cane burning. The metacholine concentrations used were 0.025; 0.25; 1.0; 2.5; 10.0; 25.0 mg/ml, and the results were expressed in PC(20) FEV1 (concentration of metacholine that induces a fall of 20% or more in the forced expiratory volume in the first second).RESULTS: The PC(20) average for asthmatic children was significantly lower than the control group, before (A= 3.68; C= 25.62 mg/ml) and during the burning (A= 4.11; C= 25.25 mg/ml) (p < 0.05). However, there were no significant differences, when compared in each group PC(20) values before and during burning. The same was observed regarding FEV1, forced vital capacity (FVC) and forced expiratory flux between 25 and 75% of FVC (FEF (25-75%)).CONCLUSIONS: It was not possible to demonstrate that cane plantation burning influences the BHR, and pulmonary functions tests of the studied children.