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.

Idiopathic bronchiolitis obliterans organizing pneumonia: case review

Bronchiolitis obliteans organizing pneumonia [BOOP] is characterized clinically by a subacute or chronic respiratory illness. The purpose of this study was to describe clinical and radiologic features of Idiopathic [cryptogenic] bronchiolitis obliterans organizing pneumonia. We retrospectively reviewed 11 patients with biopsy proven BOOP at Masih Daneshvari Hospital, for whom well documented clinical and radiographic data were available. The final diagnosis of BOOP was validated if the followings were present. 1] Negative sputum or bronchoalveolar lavage [BAL] analysis for Mycobacterium tuberculosis 2] Open lung biopsy [OLB] or trans-bronchial lung biopsy [TBLB] findings characteristic of BOOP. 3] Negative findings for systemic disorders or associated primary pulmonary lesions such as cancer. 4] Prompt response to steroid therapy. The mean age of patients with BOOP in this case series was 46.3 +/- 24.6 yrs. [range 32-70]; the male/female ratio was 7/4. The clinical pattern in BOOP presentation was more similar to classic sub-acute infectious process: dyspnea in 9 patients [81.8%], fever in 5 [45.4%], and cough in 6 [54.5%]. The symptoms were usually mild. Physical examination showed sparse crackles in 5 patients [45.4%] and wheezing in 7 [63.6%]. The most frequent radiologic patterns were ground glass appearances [63.6%] and diffuse infiltration associated with reticular pattern [27.2%]. In 6 patients chest images showed bilateral distribution. The clinical and radiological manifestation of BOOP in our patients did not differ from other reports. BOOP cases may present a distinct entity like pneumonia. Physicians in charge of these patients were all surprised of BOOP diagnosis by tissue examination. Trans- bronchial lung biopsy specimens along with strongly suggestive clinical and radiologic findings in many cases were adequate for making the diagnosis. We suggest that the diagnosis of BOOP must be considered in any immunocompetent patient with pneumonia with poor or no response to antibiotic therapy