Functional respiratory impairment and related factors in patients with interstitial pneumonia with autoimmune features (IPAF): Multicenter study from NEREA registry.

BACKGROUND: The objective of the present study is to describe the characteristics of interstitial pneumonia with autoimmune features (IPAF) patients, to assess the incidence rate of functional respiratory impairment over time and to evaluate the influence of therapeutic alternatives on the prognosis of these patients. METHODS: A longitudinal observational multicenter study was performed (NEREA registry). It was carried out by a multidisciplinary team in seven Hospitals of Madrid. Patients were included from IPAF diagnosis. MAIN OUTCOME: poor prognosis as functional respiratory impairment (relative decline in FVC % defined as ≥ 5% every 6 months). Covariates: therapy, sociodemographic, clinical, radiological patterns, laboratory and functional tests. STATISTICS: Survival techniques were used to estimate IR per 100 patients-semester with their 95% confidence interval [CI]. The influence of covariates in prognosis were analyzed through cox multivariate regression models (hazard ratio (HR) and [CI]). RESULTS: 79 IPAF were included, with a mean and a maximum follow-up of 3.17 and 12 years respectively. Along the study, 77.2% received treatment (52 glucocorticoids, 25 mycophenolate, 21 azathioprine, 15 rituximab and 11 antifibrotics). IR was 23.9 [19.9-28.8], and 50% of IPAF developed functional respiratory impairment after 16 months from its diagnosis. Multivariate analysis: usual interstitial pneumonia (UIP) had poorer prognosis compared to non-specific interstitial pneumonia (NSIP) (p = 0.001). In NSIP, positive ANA, increased the risk of poor prognosis. In UIP, glucocorticoids (HR: 0.53 [0.34-0.83]), age (HR: 1.04 [1.01-1.07]), and Ro-antibodies (HR: 0.36 [0.19-0.65]) influenced the prognosis. CONCLUSIONS: IPAF have functional impairment during the first years of disease. Factors predicting deterioration differ between radiographic patterns. Our real-life study suggests the potential benefit of particular therapies in IPAF.

Mycobacterium lentiflavum. Infección pulmonar en una paciente inmunocompetente / Mycobacterium lentiflavum. Pulmonary infection in an immunocompetent patient

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Simultaneous bilateral pneumothorax in an immunocompromised HIV patient with Pneumocystis jirovecii pneumonia.

Pneumocystis in humans is caused by a unicellular and eukaryotic organism called P. jirovecii. The overall incidence of P. jirovecii pneumonia (PCP) has decreased with the use of highly active antiretroviral therapy and the use of chemoprophylaxis with trimethroprim sulfametoxazole (TMP/SMX) in cases of immunosuppressed patients. However, approximately 85% of patients with advanced HIV infections continue to experience this disease with inadequate management. Pneumocystis infection can present with spontaneous pneumothorax in 2-6% of cases [8] which can be a potentially fatal complication. We report the case of a 32-year-old man presented with P. jirovecii pneumonia who developed cystic lesions and spontaneous bilateral pneumothorax in spite of TMP/SMX treatment. We consider it an interesting clinical case because few simultaneous bilateral pneumothorax cases have been described directly related to the PCP.

Value of Exhaled Nitric Oxide (FeNO) And Eosinophilia During the Exacerbations of Chronic Obstructive Pulmonary Disease Requiring Hospital Admission.

The aim of this study was to analyze whether FeNO levels in acute exacerbation of COPD (AECOPD) with hospital admission have better diagnostic value than eosinophilia in blood, and to evaluate its usefulness in predicting a better clinical response. An observational prospective study of patients with AECOPD was carried out. FeNO determinations were made on arrival at the emergency room (ER), at discharge and during stability 3-6 months after discharge. Co-morbidities, bronchodilators, inhaled (IGC) and systemic (SGC) glucocorticoids, eosinophils, systemic inflammation markers (procalcitonin, C-reactive protein), eosinophil cationic protein, and total IgE were collected. Fifty consecutive patients (92% men, mean age 75 ± 6 years) were included in this study. Phenotypes were 26% Asthma-COPD Overlap Syndrome (ACOS), 42% chronic bronchitis (CB) and 32% emphysema. ACOS patients showed significantly higher levels of FeNO (73 ppb) and eosinophils (508 cells/mm3) than the rest (CB: 23 ppb, 184 cells/mm3, emphysema: 27 ppb, 159 cells/mm3; p < 0.05). A significant correlation between FeNO levels measured in ER and eosinophils was observed (r = 0.7; p < 0.001), but not at discharge or in stable phase. No significant association was found with parameters of systemic inflammation and mean stay. In conclusion, the determination of FeNO in AECOPD does not offer advantages over the evaluation of eosinophilia. These parameters rise at arrival in ER, descend at discharge, and remain unchanged in the stable phase. Both present similar diagnostic utility and are able to better identify the ACOS phenotype, which helps select a population that could benefit from a glucocorticoids therapy.

[Pulmonary adenocarcinoma and Bazex syndrome (paraneoplastic acrokeratosis)]. / Adenocarcinoma de pulmón y síndrome de Bazex (acroqueratosis paraneoplásica).

Bazex syndrome, or paraneoplastic acrokeratosis, is a rare psoriasis-like paraneoplastic skin disease, characterized by erythema and scaling, which is accompanied by hyperkeratotic lesions. The tumors most frequently associated with Bazex syndrome are squamous cell carcinomas of the upper respiratory and digestive tracts, whereas lung cancers, particularly adenocarcinomas, are rarely associated. We present a case in which both pulmonary adenocarcinoma and Bazex syndrome were present.