Neumonia crónica organizada en un adolescente: presentación de un caso / Chronic organized pneumonia in an adolescent: presentation of a case

We present a case of organized chronic pneumonia (OCP), a rare interstitial disease, which usually affects people over 50 years of age and is very unusual in pediatrics, so we thought it was of interest to communicate it. Is a 14-year-old male in whom in the study of a febrile condition, a pneumonia of LII was detected. The images persist after 2 months of being treated and being asymptomatic. Infectious causes and rheumatic diseases were ruled out due to the history of discoid lupus. Chest CT, bronchoalveolar lavage and lung biopsy showing Masson's bodies were performed and OCP was diagnosed. He received prednisone 1 mg / kg day and Clarithromycin for 2 months with rapid improvement. Relapses with new radiological images typical of OCP twice, when treating on alternate days. It is indicated prednisone 30 mgr / day for 3 months and gradual reduction. At 18 months, being with prednisone 5 mgr day, has a relapse of rapid progression that was treated with pulses of methylprednisolone. The collagen study shows positive lupus anticoagulant and ANA and Systemic Lupus is diagnosed. The bad evolution would be explained because it was a secondary OCP. Mycophenolate associated with prednisone was indicates, which has been used in the OCP and in Lupus. This clinical case shows the importance of radiological follow-up of patients with OCP and the search for secondary OCP causes due to the poor response to corticosteroids.

Se presenta un caso de neumonía crónica organizada (NCO), enfermedad intersticial rara, que afecta habitualmente a mayores de 50 años y muy inusual en pediatría, por lo que creímos de interés comunicarlo. Se trata de un varón de 14 años en el que en el estudio de un cuadro febril se detecta una neumonía de LII cuyas imágenes persisten luego de 2 meses de haber sido tratado y estando asintomático. Se descartaron causas infecciosas y enfermedades reumatológicas por el antecedente de lupus discoide. Se realizó TC de tórax, lavado broncoalveolar y biopsia pulmonar que mostró cuerpos de Masson, con lo que se diagnosticó NCO. Recibió prednisona 1 mgr/ kg día y Claritromicina por 2 meses con una rápida mejoría. Recae con nuevas imágenes radiológicas típicas de NCO dos veces, al pasar de tratamiento diario a días alternos. Se indica 30 mgr/día por 3 meses de prednisona y reducción gradual posterior. A los 18 meses de tratamiento, estando con prednisona 5 mgr día, tiene una recaída de rápida progresión que se trató con pulsos de metilprednisolona. El estudio de colagenosis muestra anticoagulante lúpico y ANA positivos y se diagnóstica Lupus Sistémico. La mala evolución se explicaría porque se trató de una NCO secundaria, ante lo cual indicó micofenolato asociado a prednisona, que se ha usado en la NCO y en el Lupus. Este caso clínico muestra la importancia del seguimiento radiológico de los pacientes con NCO y de la búsqueda de causas secundarias de NCO ante la mala respuesta a corticoides

Signo del halo invertido como manifestación inhabitual de neumonía organizada criptogénica: reporte de un caso / Reversed halo sign as an unusual manifestation of cryptogenic organized pneumonia: report of one case

We report a 64 years old female admitted with fever, cough, dyspnea and lung opacities in the chest X ray. A chest tomography scan (CTS) showed multiple-bilateral ring-shaped opacities and the reversed halo sign (RHS). The patient did not improve with antimicrobial therapy (AT). Infection and rheumatologic causes were excluded, therefore Cryptogenic organizing pneumonia (COP) was suspected with compatible percutaneous biopsy. Systemic steroids were started with a good clinical response. The patient was discharged four weeks after admission in good general conditions and practically no lungs opacities.

Manifestaciones clínicas de la neumonía en organización / Clinical manifestations of organizing pneumonia

La neumonía en organización es una entidad clínica asociada a síntomas y hallazgos radiológicos inespecíficos y anomalías en las pruebas de función pulmonar. El patrón histopatológico característico se define por tapones intra-alveolares de tejido de granulación. Puede presentarse sin una etiología determinada -neumonía en organización criptogénica (COP, cryptogenic organizing pneumonia)- o en otra condición clínica -neumonía en organización secundaria (SOP: secondary organizing pneumonia). Es poco frecuente y las manifestaciones clínico-radiológicas son poco específicas. Para su confirmación se requieren procedimientos invasivos, siendo habitual el tratamiento empírico con esteroides sistémicos ante la sospecha clínica. Se presentan 13 casos con diagnóstico histológico de neumonía en organización describiendo las características clínicas. La mediana de edad fue 76 años y la de tiempo al diagnóstico desde el inicio de los síntomas: 31 días. En 10 casos el diagnóstico fue por biopsia transbronquial (BTB). Ocho pacientes requirieron internación, 4 de ellos recibieron pulsos de esteroides y soporte ventilatorio. Uno falleció por una causa atribuible a la entidad y 5 presentaron recaídas. Disnea, tos y fiebre fueron los síntomas más frecuentes. La mayoría presentó más de un patrón tomográfico, siendo los más habituales vidrio esmerilado y consolidación alveolar. En 9 se realizó el diagnóstico de COP y en 4 de SOP. La causa secundaria en todos los casos fue toxicidad por drogas. Las características clínicas de los casos comunicados son consistentes con las series previamente publicadas. Cabe destacar la necesidad de tratamiento con dosis altas de esteroides y soporte ventilatorio en un grupo de pacientes.

Organizing pneumonia is a clinical entity asociated with nonspecific symptoms and radiological findings and abnormalities in pulmonary function tests. It is defined by the characteristic histopathological pattern: filling of alveoli and respiratory bronchioles by plugs of granulation tissue. It can be idiopathic (COP) or secondary to other causes (SOP). It is an unusual finding and the clinical and radiographic findings are nonspecific. For specific diagnosis an invasive procedure has to be done, but often empirical treatment is started when there’s a clinical suspicion. We describe the clinical characteristics of 13 patients with histological diagnosis of organizing pneumonia. Data was obtained from their medical records. The median age was 76 years and the median time to diagnosis from the onset of symptoms was 31 days. In 10 cases the diagnosis was made by transbronchial biopsy. 8 patients required hospitalization, 4 of them received high doses of steroids and 3 required ventilatory support. One patient died from a cause attributable to this entity and 5 relapsed. Dyspnea, cough and fever were the most frequent symptoms. Most patients had more than one tomographic pattern being the most common ground glass opacities and alveolar consolidation. Nine patients were diagnosed with COP and 4 with SOP. The most frequent underlying cause of SOP was drug toxicity. The clinical characteristics of the reported cases are consistent with previously published series. As an interesting feature, there was a group of patients that needed high doses of steroids and ventilatory support.

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.

Bronchiolitis Obliterans Organizing Pneumonia:Clinicopathologic Review of a Series of 45 Korean Patients Including Rapidly Progressive form

Bronchiolitis obliterans organizing pneumonia (BOOP) is a clinicopathological syndrome associated with a variety of disease entities. The aim of this study was to review cases with initial diagnosis of BOOP applying uniform histopathologic criteria, and analyze the clinical characteristics of proven cases of BOOP including rapidly progressive form. A total of 81 cases, initially diagnosed as BOOP and with available tissue sections, was collected. Thirty six cases (44.4%) were excluded from the study, more than two thirds of which were given a revised diagnosis of interstitial pneumonitis/fibrosis other than BOOP. Thirty one patients were classified as idiopathic BOOP, 8 patients as secondary BOOP, and 6 patients as rapidly progressive BOOP. Open lung biopsy specimen from all six cases with lethal outcome showed more severe interstitial inflammation and septal fibrosis and/or alveolar exudate with a varying degree than those with good prognosis. There was no difference by the sexes. The two most frequent presenting symptoms were cough and dyspnea. Bilateral multifocal consolidation was a common radiological finding. More than 70% cases of idiopathic BOOP experienced clinical improvements. The diagnosis of BOOP is usually suggested by clinicoradiologic findings, but needs to be confirmed histopathologically, preferably through surgical open or video-assisted thoracoscopic biopsy.