Pulmonary manifestation of VEXAS syndrome.

This case report presents the diagnostic journey of a man in his mid-70s who experienced shortness of breath, cough, recurrent episodes of fever, weight loss, pruritic erythroderma, uveitis and macrocytic anaemia. The initial diagnosis of cryptogenic organising pneumonia was made based on antibiotic refractory infiltrates seen in the lung CT scan. The patient initially responded favourably to immunosuppression but experienced a recurrence of symptoms when the corticosteroid dose was tapered. Despite ongoing systemic inflammation and refractory symptoms, it took nearly a year to establish the diagnosis of VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory and somatic) syndrome. This case highlights the challenges in diagnosing and managing VEXAS syndrome due to its recent discovery and limited awareness in the medical community, as well as the need to consider this syndrome as a rare differential diagnosis of therapy-refractory pulmonary infiltrates.

Why do a lung biopsy for benign lesions?

BACKGROUND: Transthoracic needle biopsy of lung lesions is a well-established procedure for the diagnosis of lung lesions. The literature focuses on the diagnosis of malignant lesions with an often reported accuracy rate of more than 90%. Experience showed that biopsy can identify sometimes incidentally, also benign lesions. There are many reasons why a biopsy is performed for a "benign lesion." First of all, it may be an unexpected diagnosis, as some benign pathologies may have misleading presentations, that are very similar to lung cancer, otherwise the reason is only to make a diagnosis of exclusion, which leads to the benign pathology already being considered in the differential diagnosis. METHODS: This study was designed as a retrospective single-center study. We selected from our database all the lung biopsies performed under CT guidance, from 2015 to 2019 and retrospectively analysed the histological data. We selected only benign lesions describing the imaging feature and differential diagnosis with lung malignancy. RESULTS: In our patient population, among the 969 of them that underwent biopsy, we identified 93 benign lesions (10%). Hamartomas, granulomas, slow-resolving pneumonia and cryptogenic organizing pneumonia are the pathologies that most frequently can misinterpratedas lung cancer. CONCLUSIONS: In this brief report we want to show the percentage and type of benign lesions that are found in our lung trans-thoracic biopsy population. Among these, we identified the three most frequent benign lesions that most frequently enter the differential diagnosis with lung malignant lesions describing the classic and atypical imaging findings.

Application of Innovative 3D Pathological Tactic for Diagnosis of Organizing Pneumonia.

BACKGROUND/AIM: The pathological diagnosis of organizing pneumonia (OP) relies on conventional traditional histopathological analysis, which involves examining stained thin slices of tissue. However, this method often results in suboptimal diagnostic objectivity due to low tissue sampling rates. This study aimed to assess the efficacy of tissue-clearing and infiltration-enhanced 3D spatial imaging techniques for elucidating the tissue architecture of OP. MATERIALS AND METHODS: H&E staining, 3D imaging technology, and AI-assisted analysis were employed to facilitate the construction of a multidimensional tissue architecture using six OP patient specimens procured from Taichung Veterans General Hospital, enabling a comprehensive morphological assessment. RESULTS: Specimens underwent H&E staining and exhibited Masson bodies and varying degrees of interstitial fibrosis. Furthermore, we conducted a comprehensive study of 3D images of the pulmonary histology reconstructed through an in-depth pathology analysis, and uncovered heterogenous distributions of fibrosis and Masson bodies across different depths of the OP specimens. CONCLUSION: Integrating 3D imaging for OP with AI-assisted analysis permits a substantially enhanced visualization and delineation of complex histological pulmonary disorders such as OP. The synergistic application of conventional histopathology with novel 3D imaging elucidated the sophisticated spatial configuration of OP, revealing the presence of Masson bodies and interstitial fibrosis. This methodology transcends conventional pathology constraints and paves the way for advanced algorithmic approaches to enhance precision in the detection, classification, and clinical management of lung pathologies.

A Case of Organizing Pneumonia Resembling Lung Cancer.

BACKGROUND: Organizing pneumonia (OP) is a pathologic diagnosis with clinical and imaging manifestations that often resemble other diseases, such as infections and cancers, which can lead to delays in diagnosis and inappropriate management of the underlying disease. In this article, we present a case of organized pneumonia that resembles lung cancer. METHODS: We report a case of initial suspicion of pulmonary malignancy, treated with anti-inflammatory medication and then reviewed with CT suggesting no improvement, and finally confirmed to be OP by pathological biopsy taken via transbronchoscopy. A joint literature analysis was performed to raise clinicians' awareness of the diagnosis and treatment of OP. RESULTS: Initially, because of the atypical auxiliary findings, we thought that the disease turned out to be a lung tumor, which was eventually confirmed as OP by pathological diagnosis. CONCLUSIONS: The diagnosis and treatment of OP requires a combination of clinical information and radiological expertise, as well as biopsy to obtain histopathological evidence. That is, clinical-imaging-pathological tripartite cooperation and comprehensive analysis.

[Clinical analysis of 4 children with cryptogenic organizing pneumonia].

Objective: To analyze the clinical features of children with cryptogenic organizing pneumonia (COP) confirmed by pathology. Methods: The clinical manifestations, imaging, pathology, treatment and outcome data of 4 children with COP confirmed by thoracoscopic lung biopsy were retrospectively analyzed, who were hospitalized at Respiratory Department of Shenzhen Children's Hospital from January 2004 to December 2022. Results: All of the 4 patients were male, the age ranged from 1 year 3 months to 14 years. The time from onset to diagnosis was 3 months to 3 years. The follow-up duration was 6 months to 6 years. All the 4 cases had cough, 2 cases had tachypnea and wheezing, 1 case had expectoration, 1 case had chest pain, 1 case had decreased activity tolerance and weight loss. Rales in 2 cases and hypoxemia in 1 case. Pulmonary high resolution CT showed diffuse distribution, involvement of both lungs in 3 cases, and single lung combined migration in 1 case.Three cases showed ground-glass opacity, consolidation, patchy or fibrous strips, and 1 case presented air bronchogram and "reversed halo sign". All the 4 cases were performed thoracoscopic lung biopsy, and the pathological findings showed cellulose exudate or small nodules filled with granulation tissue or fibroblasts in the alveolar cavity and small airways, and 1 case was Masson corpuscle positive. Three patients achieved remission after glucocorticoid therapy. Spontaneous remission without treatment was seen in 1 patient.Two cases were followed up for 17 months and 6 years, respectively, who had excellent outcome. Conclusions: The manifestations of COP in children include cough, expectoration and chest pain. Infants and young children may have tachypnea and wheezing. The most common chest CT findings are diffuse distribution of ground-glass opacity, patchy and consolidation in both lungs. Diagnosis should depend on pathological examination. The effect of glucocorticoid therapy is good.

Neumonía criptogénica organizada como diagnóstico diferencial de neumonía que no responde a tratamiento / Cryptogenic organizing non-resolving pneumonia.Report of one case

Non-resolving pneumonia is a common clinical problem that prolongs morbidity and increases hospitalization costs. We report an 82 year-old non-smoking female who was admitted with chronic diarrhea and later developed nosocomial pneumonia. Lung infiltrates did not resolve despite sequential antibiotic treatments. Infectious causes such as resistant nosocomial pathogens, respiratory viruses, tuberculosis, Legionellosis, cytomegalovirus or agents associated with HIV infection were discarded. Non-infectious causes such as thromboembolic lung disease, neoplasms and rheumatic disorders were also ruled out. An exudative pleural effusion was detected, but the study was unremarkable. Fiberoptic bronchoscopy and a transbronchial biopsy, revealed nonspecific findings. The patient persisted febrile, required non-invasive mechanical ventilation and displayed a migratory pattern of lung infiltrates that motivated a second biopsy, this time by open thoracotomy, showing a cryptogenic organizing pneumonia. The patient's conditions improved after treatment with adrenal steroids. In patients with non-resolving pneumonia, a dedicated and comprehensive study should be done using invasive procedures and considering both infectious and non-infectious causes. Cryptogenic organizing pneumonia is one of the alternatives that is potentially treatable, but often underdiagnosed.

Bronquiolite obliterante com pneumonia em organização / Bronchiolitis obliterans organizing pneumonia

Uma paciente 48 anos apresentou-se, assintomática, mas com achado radiológico de condensações alveolares, algumas constituindo nódulos em lobo superior direito, lobo médio e língula com predomínio perihilar. Foi considerado o diagnóstico diferencial de neoplasia, sarcoidose, doenças granulomatosas e tuberculose, entre outras doenças. O diagnóstico de bronquiolite obliterante com pneumonia em organização foi estabelecido através de videotoracoscopia e foi iniciado o tratamento com esteróides. Houve boa evolução com resolução radiológica.

Recidiva en bronquiolitis obliterante con neumonía en organización: BOOP / Relapsein bronchholitis obliterans organizing pneumonia: boop

Although relapses are frequent in bronchiolitis obliterans organizing pneumonia (BOOP), there is scant information regarding the causes underlying its occurrence. We report a 63 year old woman with clinical and radiological features compatible with BOOP. No underlying cause was identified so she was thought to have cryptogenic BOOP or cryptogenic organizing pneumonia (COP). A transbronchial lung biopsy demonstrated chronic organizing pneumonia and features of proliferative bronchiolitis. She was successfully treated with prednisone. On the eighth month of steroid therapy, while tapering the dose, she begun with cough and dyspnea and developed new lung infiltrates on the chest x-ray film. The infiltrates cleared rapidly after increasing the dose of steroids. We discuss the possible causes of relapse in BOOP and its relation to steroid therapy.

Las recaídas son frecuentes en la bronquiolitis obliterante con neumonía en organización (BOOP), sin embargo, existe poca información con respecto a su causa. Se presenta el caso de una mujer de 63 años con un cuadro clínico radiológico compatible con BOOP. No se identificó una causa subyacente por lo que se planteó el diagnóstico de neumonía en organización criptogénica o COP. Se realizaron biopsias transbronquiales que demostraron una neumonía crónica organizada y bronquiolitis proliferativa. Se trató con prednisona con buena respuesta. Al octavo mes de tratamiento, mientras se disminuía la dosis de esteroides, comenzó con disnea y tos y aparición de nuevas opacidades pulmonares radiológicos, las que regresaron rápidamente al aumentar la dosis de esteroides. Discutimos las causas posibles de la recidiva de esta patología y su relación con la disminución de la dosis de esteroides.

O espectro clínico e radiológico da pneumonia em organização: análise retrospectiva de 38 casos / Clinical and radiographic spectrum of organizing pneumonia: retrospective analysis of 38 cases

A pneumonia em organização, acompanhada ou não de bronquiolite obliterante, é uma condição anatomopatológica pulmonar específica com uma grande variedade de apresentações clínicas e radiológicas. A pneumonia em organização pode ser idiopática ou secundária a várias doenças, incluindo infecções e drogas. Objetivos: Descrever as manifestações clínicas, dados radiológicos e espirométricos de pacientes com pneumonia em organização. Métodos: Análise retrospectiva de pacientes com o diagnóstico de pneumonia em organização. Resultados: 38 pacientes foram incluídos na análise. Quatorze apresentavam também condições clínicas relacionadas à pneumonia em organização, e estes mais freqüentemente apresentaram infiltração pulmonar difusa (três de quatro casos) e bronquiolite obliterante associada (57 por cento vs. 20 por cento, p = 0,05). Dos 13 pacientes com bronquiolite obliterante associada apenas um era assintomático e dois apresentavam lesões localizadas (15 por cento). De oito pacientes assintomáticos com lesões localizadas, nenhum possuía bronquiolite obliterante associada, e a apresentação radiológica freqüentemente mimetizou carcinoma brônquico. A espirometria de modo geral não contribuiu sobremaneira para o diagnóstico, provavelmente devido à alta prevalência de tabagismo na amostra. Conclusões: A pneumonia em organização possui apresentação clínica e radiológica variada. Fatores que sugerem a ausência de bronquiolite obliterante são a ausência de sintomas e lesões radiológicas localizadas, e esta forma da doença mais freqüentemente deve ser diferenciada de carcinoma brônquico na prática clínica

Neumonía intersticial no específica / Diffuse idiopathic interstitial lung disease

Se presenta un paciente, clínicamente calificado como broncoquiolitis obliterante con neumonía demostró una neumonía intersticial inespecífica, entidad poco frecuente, recientemente descrita, cuyo reconocimiento es importante por responder a tratamiento corticoidal, al contrario de la fibrosis intersticial usual, afección más frecuente con la cual ha sido confundida bajo la categoría clínica de fibrosis pulmonar idiopática

Biopsia pulmonar de enfermos críticos en la Unidad de Terapia Intensiva / Open lung biopsy in severely ill patient at the Intensive Care Unit

Antecedentes: El diagnóstico y tratamiento de las neumopatías asociadas al respirador requiere habitualmente el uso de técnicas cruentas en enfermos graves. Objetivo: Determinar la seguridad y la confiabilidad de la biopsia de pulmón a cielo abierto en la cama de la Unidad de Terapia Intensiva (UTI) y su influencia sobre la evolución de los enfermos. Lugar de aplicación: Unidad de Terapia Intensiva de Adultos de un Hospital Universitario. Población: Once enfermos sometidos a asistencia respiratoria mecánica, con rápido deterioro de su función respiratoria e inestabilidad hemodinámica. Diseño: Análisis retrospectivo evaluando parámetros respiratorios y hemodinámicos, diagnóstico y tratamiento previos y posteriores al procedimiento. Métodos: Biopsia de pulmón a cielo abierto en la cama del enfermo de UTI empleando suturas mecánicas por toracotomía mínima (5 cm). Procesado del material en forma inmediata (microbiología y anatomía patológica). Resultados: En 10 de los 11 enfermos hubo cmabio de diagnóstico y tratamiento y 3 enfermos sobrevivieron y pudieron ser dados de alta. Solamente un enfermo presentó una complicación relacionada con el procedimiento. Conclusión: La biopsia de pulmón en la Unidad de Terapia Intensiva es segura y confiable; cambia el diagnóstico y el tratamiento en la mayoría de los enfermos, con bajo índice de complicaciones relacionadas

Bronquiolitis obliterante con neumonia organizada asociada a purpura trombocitopenica idiopatica / Bronchiolitis obliterans with organizing pneumonia associated with idiopathic thrombocytopenic purpura

Se presenta el caso de una mujer de 35 años portadora de una púrpura trombocitopénica idiopática (PTI), esplenectomizada y en tratamiento con corticoides y ciclosporina, que desarrolla una neumopatía intersticial bilateral, con incapacidad ventilatoria mixta e insuficiencia respiratoria parcial. Durante su enfermedad produce una seroconversión para el virus de Epstein Barr con alteraciones en el hepatograma. Se diagnostica, por examen anatomopatológico, una bronquiolitis obliterante con neumonía organizada (BOOP). El cuadro remite sin mediar medicación alguna, coincidiendo con la suspensión de la ciclosporina. No existen publicaciones previas de BOOP asociada a PTI, ni en donde se mencionen como desencadenantes de este cuadro a la ciclosporina o a la infección por el virus Epstein Barr.

Patología de la pequeña vía aérea / High resolution CT in adult bronchiolitis

Las anormalidades bronquiales pueden ser detectadas por aumento o disminución de la densidad del parénquima pulmonar. Cuando ésta disminuye se manifiesta por la existencia de lesiones de tipo destructivo; enfisema, bullas, quistes, bronquioloectasias, o indirectamente por disminución de la perfusión y atrapamiento aéreo. El aumento de densidad puede ser detectado cuando hay engrosamiento de la pared del bronquiolo, inflamación, fibrosis y obstrucción de la luz. Las enfermedades representantes son: la bronquiolitis obliterante, bronquiolitis obliterante con neumonía en organización, bronquiolitis respiratoria y la panbronquiolitis. La Tomografía Computada con alta resolución es la mejor modalidad de imagen para estudiar el parénquima y la vía aérea pulmonar, a través del conocimiento de la anatomía del lóbulo secundario. Si bien es cierto que muchos de los signos para el diagnóstico son indirectos y poco específicos, poder detectar las lesiones favorece el tratamiento médico y orienta la realización de una biopsia pulmonar que dará el diagnóstico definitivo