Screening criteria for interstitial lung disease associated to rheumatoid arthritis: Expert proposal based on Delphi methodology.

OBJECTIVE: To develop a joint proposal for screening criteria of interstitial lung disease (ILD) in patients with rheumatoid arthritis (RA) and vice versa, which serves as a guidelines in patient referral between the Rheumatology and Pneumology departments to early detection of these patients. METHODS: A systematic literature review was carried out on the risk factors for the development of ILD in RA patients, and for the referral criteria to Rheumatology for suspected early RA. Based on the available evidence, screening criteria were agreed using the Delphi method by a panel of pneumologists and rheumatologists with expertise in these pathologies. RESULTS: Screening criteria for ILD in patients with RA and for the early detection of RA in cases with ILD of unknown etiology have been developed. In both cases, a detection strategy was based on clinical risk factors. Recommendations also included the complementary tests to be carried out in the different clinical scenarios and on the periodicity that screening should be repeated. CONCLUSION: A selective screening strategy is recommended for the first time in the early diagnosis of patients with ILD-RA. This multidisciplinary proposal aims to solve some common clinical questions and help decision-making, although its usefulness to identify these patients with good sensitivity must be confirmed in a validation study.

Nódulos necrobióticos pulmonares: una manifestación excepcional de la enfermedad de Crohn / Pulmonary Necrobiotic Nodules: A Rare Manifestation of Crohn's Disease

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Pulmonary cysts in smoking-related interstitial fibrosis: a form of pseudocyst secondary to pulmonary interstitial emphysema / Quistes pulmonares en el contexto de la fibrosis intersticial pulmonar relacionada con el tabaco: una forma de seudoquiste secundario a enfisema intersticial pulmonar

Smoking-related interstitial fibrosis is a distinct form of fibrosis, found in smokers, which has striking histopathological features. We present a case of pulmonary interstitial fibrosis with cysts in a 58-year-old woman who was a significant active smoker, presenting with a 7 month history of progressive dyspnea. TAC revealed thin-walled pulmonary cysts. An open lung biopsy was performed and the histopathological study showed hyaline fibrous thickening of the alveolar septa, respiratory bronchiolitis and cysts in the thickness of the interlobar septa. Immunohistochemically, the absence of an epithelial, vascular or lymphatic endothelial lining of the cysts would suggest that the cysts had been caused by pulmonary interstitial emphysema. Immunohistochemistry is essential in the differential diagnosis that includes, in this case, true cysts, pseudocysts and pulmonary lymphangiectasia

La fibrosis intersticial relacionada con el tabaco es una forma especial de fibrosis con histología característica que ocurre en fumadores. Presentamos un caso de fibrosis intersticial pulmonar con quistes en una mujer de 58 años con historia de tabaquismo importante, que refería disnea progresiva en los últimos 7 meses. La TAC reveló quistes pulmonares de paredes delgadas. Se realizó una biopsia pulmonar abierta y el estudio histopatológico mostró engrosamiento fibroso hialino de los septos alveolares, bronquiolitis respiratoria y quistes en el espesor de los septos interlobares. Inmunohistoquímicamente, la ausencia de revestimiento epitelial, endotelial vascular y linfático de los quistes, apoya que estos son causados por enfisema intersticial pulmonar. La inmunohistoquímica es esencial en el diagnóstico diferencial que incluye en este caso, quistes verdaderos, seudoquistes y linfangiectasia pulmonar

Prognosis and Follow-Up of Idiopathic Pulmonary Fibrosis.

Idiopathic pulmonary fibrosis (IPF), a devastating progressive interstitial lung disease (ILD) with no known cause, is the most common and deadly of the idiopathic interstitial pneumonias. With a median survival of 3⁻5 years following diagnosis, IPF is characterized by a progressive decline in lung function and quality of life in most patients. Prognostic factors recognized classically that influence mortality include functional, clinical and radiological parameters. However, in recent years, there has also been progress in the knowledge of genetic factors and biomarkers that may be useful in the prognostic evaluation of these patients. On the other hand, the monitoring of the disease throughout its evolution is key to improving the prognosis of the patients, as it allows for taking therapeutic measures based on this evolution, even early remission for lung transplantation. This article reviews the main prognostic factors of the disease, as well as the most useful way to monitor the disease follow-up.

Síndrome platipnea-ortodeoxia cardiaco: una causa «misteriosa» de hipoxemia / Cardiac platypnea-orthodeoxia syndrome: A «mysterious» cause of hypoxemia

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