A case of acute severe cryptogenic organic pneumonia with secondary hemophilia / 中南大学学报(医学版)

Cryptogenic organic pneumonia (COP) refers to organic pneumonia that has not been identified a clear cause by current medical methods. A small proportion of COP can exhibit severe and progressive characteristics, while severe COP can cause systemic inflammatory storms and can be secondary to hemophilia. This article reported a case of acute severe COP secondary to hemophilia. A 67-year-old male patient was admitted to the hospital due to cough, shortness of breath, and fever. At first, he was misdiagnosed as severe pneumonia, but failed to receive anti infection treatments. Sputum pathogenetic examination and Macrogene testing of alveolar lavage fluid were performed, and no etiology was found to explain the patient's condition. The condition was gradually worsened and hemophilia occurred to explain, suggesting that acute severe COP was relevant. After receiving hormone treatment, the condition gradually relieved and the absorption of lung lesions improved. Hemophilia secondary to COP is rare, and the specific mechanism needs further study.

Organizing pneumonia due to anti-synthetase syndrome: Presentation of a clinical case

Introduction: Organizing pneumonia is a rare clinico-pathological syndrome. This crypto-genic or secondary condition is of unknown origin, and may be infectious, or associated with autoimmune diseases, cancer, drugs, or radiation. Case description: The case is presented of a 52-year-old patient who was diagnosed with organizing pneumonia secondary to anti-synthetase syndrome. Discussion: It is intended to make known that not all pulmonary consolidative clinical pictures correspond to infectious processes. In this case, an organizing pneumonia secondary to anti-synthetase syndrome is documented. Despite being a disorder that is classified as an idiopathic inflammatory myopathy, it manifests as an interstitial lung disease with predominantly respiratory symptoms.

Introducción: La neumonía organizativa es un síndrome clínico-patológico poco frecuente, dentro del cual se desconoce la etiología de la denominada neumonía criptogénica o secundaria, que puede ser infecciosa o asociada con enfermedades autoinmunes, cáncer, fármacos o radiación. Descripción del caso: Se presenta el caso de una paciente de 52 arios a quien se le diagnostica neumonía organizativa secundaria a síndrome antisintetasa. Discusión: Se busca dar a conocer que no todos los cuadros clínicos de consolidación pulmonar corresponden a procesos infecciosos. En este caso se documentó una neumonía organizativa secundaria a síndrome antisintetasa, la cual a pesar de ser una patología que se cataloga como una miopatía inflamatoria idiopática, se manifestó como una enfermedad pulmonar intersticial con síntomas predominantemente respiratorios.

Patrones tomográficos de neumonía en organización. Serie de casos y revisión de la literatura / Tomographic patterns of organizing pneumonia. Case series and review

Resumen La neumonía en organización es un tipo de enfermedad pulmonar intersticial difusa que puede ser idiopática (criptogénica) o secundaria a numerosas etiologías, y se asocia con hallazgos clínicos y de laboratorio inespecíficos. Su diagnóstico y tratamiento exigen un equipo interdisciplinario, en el que las imágenes desempeñan un papel indispensable. Se presenta una serie de nueve casos, haciendo énfasis en las características clínicas y profundizando en los diversos patrones imagenológicos identificados. También se aporta una revisión de las variantes recientemente descritas.

Abstract Organizing pneumonia is a type of diffuse interstitial lung disease that can be idiopathic (cryptogenic) or secondary to numerous etiologies, and is an entity associated with nonspecific clinical and laboratory findings. Its diagnosis and treatment require an interdisciplinary team in which images play an indispensable role. The presentation of a series of nine cases is made, emphasizing the clinical characteristics and delving into the various identified imaging patterns. A review of the recently described variants is also provided.

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.

Development of bronchiolitis obliterans organizing pneumonia during standard treatment of hepatitis C with Peg-IFNα2b

No abstract available.

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.

Clinical Analysis of 25 Cases of Cryptogenic Organizing Pneumonia / 沈阳医学院学报

Objective:To analyze clinical features and imaging characteristics of cryptogenic organizing pneumonia (COP) confirmed by pathological examination, and improve clinicians understanding of the disease and reduce the disease misdiagnosis. Methods:General information, clinical manifestations,laboratory examination and radiographic features of 25 patients with COP confirmed by pathological examination of lung biopsy were collected, and a comprehensive analysis of these data was made. Results:There were 15 males and 10 females in 25 patients.The mean age was (61.4±13.1) years.And 6 cases (24??0%) had smoking history. All patients had no obvious allergic and industrial dust related exposure history. The clinical presentations were nonspecific, and the main clinical symptoms were cough with a small amount of sputum. The most common laboratory tests was counting normal white blood cells and faster blood sedimentation.The main features of chest CT were space occupying lesions and patchy consolidation or ground-glass opacification.Conclusion:The clinical manifestation and laboratory examination of COP lack of characteristic, and the chest CT performance is similar to tumor and lung infection, and the diagnosis of COP depends on pathological examination.In order to reduce misdiagnosis,clinical doctors should improve the awareness and vigilance of the disease.

Organizing pneumonia: chest HRCT findings / Pneumonia em organização: achados da TCAR de tórax

OBJECTIVE: To determine the frequency of HRCT findings and their distribution in the lung parenchyma of patients with organizing pneumonia. METHODS: This was a retrospective review of the HRCT scans of 36 adult patients (26 females and 10 males) with biopsy-proven organizing pneumonia. The patients were between 19 and 82 years of age (mean age, 56.2 years). The HRCT images were evaluated by two independent observers, discordant interpretations being resolved by consensus. RESULTS: The most common HRCT finding was that of ground-glass opacities, which were seen in 88.9% of the cases. The second most common finding was consolidation (in 83.3% of cases), followed by peribronchovascular opacities (in 52.8%), reticulation (in 38.9%), bronchiectasis (in 33.3%), interstitial nodules (in 27.8%), interlobular septal thickening (in 27.8%), perilobular pattern (in 22.2%), the reversed halo sign (in 16.7%), airspace nodules (in 11.1%), and the halo sign (in 8.3%). The lesions were predominantly bilateral, the middle and lower lung fields being the areas most commonly affected. CONCLUSIONS: Ground-glass opacities and consolidation were the most common findings, with a predominantly random distribution, although they were more common in the middle and lower thirds of the lungs. .

OBJETIVO: Determinar a frequência dos achados tomográficos e sua distribuição no parênquima pulmonar de pacientes com pneumonia em organização. MÉTODOS: Estudo retrospectivo das imagens de TCAR de 36 pacientes adultos (26 mulheres e 10 homens) com pneumonia em organização confirmada por exame histopatológico. A faixa etária dos pacientes incluídos foi de 19 a 82 anos (média: 56,2 anos). As imagens foram avaliadas por dois observadores, de forma independente, e os casos discordantes foram resolvidos por consenso. RESULTADOS: O achado tomográfico mais comum foi o de opacidades em vidro fosco, presentes em 88,9% dos casos. O segundo achado mais comum foi o de consolidação (em 83,3% dos casos), seguido de opacidades peribroncovasculares (em 52,8%), reticulação (em 38,9%), bronquiectasias (em 33,3%), nódulos intersticiais (em 27,8%), espessamento de septos interlobulares (em 27,8%), padrão perilobular (em 22,2%), sinal do halo invertido (em 16,7%), nódulos do espaço aéreo (em 11,1%) e sinal do halo (em 8,3%). As lesões foram predominantemente bilaterais, e os terços médios e inferiores dos pulmões foram as regiões mais afetadas. CONCLUSÕES: Os achados mais frequentes foram opacidades em vidro fosco e consolidações, com distribuição predominantemente aleatória, embora tenham sido mais comuns nos terços médios e inferiores dos pulmões. .

Successful Immunoglobulin Treatment in Severe Cryptogenic Organizing Pneumonia Caused by Dermatomyositis / 대한구급학회지

In connective tissue diseases, autoantibodies cause pulmonary interstitial inflammation and fibrosis, and patients require treatment with an immunosuppressive agent such as a steroid. Dermatomyositis is an incurable, uncommon form of connective tissue disease that occasionally causes diffuse pulmonary inflammation leading to acute severe respiratory failure. In such cases, the prognosis is very poor despite treatment with high-dose steroid. In the present case, a 46-year-old man was admitted to our hospital with dyspnea. He was diagnosed with dermatomyositis combined with cryptogenic organizing pneumonia (COP) with respiratory failure and underwent treatment with steroid and an immunosuppressive agent, but the COP was not improved. However, the respiratory failure did improve after treatment with intravenous immunoglobulin, which therefore can be considered a treatment option in cases where steroids and immunosuppressive agents are ineffective.

Different Responses to Clarithromycin in Patients with Cryptogenic Organizing Pneumonia / 결핵및호흡기질환

Cryptogenic organizing pneumonia (COP) is an idiopathic interstitial pneumonia characterized by a subacute course and favorable prognosis with corticosteroids. However, some patients show resistance to steroids. Macrolides have been used with success in those patients showing resistance to steroids. A few reports showed treatment failure with macrolides in patients with COP who were resistant to steroids. In this report, we described two cases of COP who showed different responses to clarithromycin. One recovered completely, but the other gradually showed lung fibrosis with clarithromycin.

A Case of Cryptogenic Organizing Pneumonia after Transarterial Chemoembolization for the Treatment of Hepatocellular Carcinoma / 결핵및호흡기질환

Cryptogenic organizing pneumonia (COP) is an inflammatory lung disease involving the distal bronchioles, respiratory bronchioles, bronchiolar ducts, and alveolae. The etiology is usually unknown; however, there are several known causes and associated systemic diseases. Corticosteroid therapy is the best treatment option and the prognosis of COP is good, with recovery in up to 80% of patients. We described a patient with in-operable hepatocellular carcinoma (HCC) undergoing chemoembolization with doxorubicin in a drug-eluting bead (DEB). COP developed in the patient after chemoembolization but resolved spontaneously in several months.

Predictors of Relapse in Patients with Organizing Pneumonia / 결핵및호흡기질환

BACKGROUND: Although organizing pneumonia (OP) responds well to corticosteroid therapy, relapse is common during dose reduction or follow-up. Predictors of relapse in OP patients remain to be established. The aim of the present study was to identify factors related to relapse in OP patients. METHODS: This study was retrospectively performed in a tertiary referral center. Of 66 OP patients who were improved with or without treatment, 20 (30%) experienced relapse. The clinical and radiologic parameters in the relapse patient group (n=20) were compared to that in the non-relapse group (n=46). RESULTS: Multivariate analysis demonstrated that percent predicted forced vital capacity (FVC), PaO2/FiO2, and serum protein level were significant predictors of relapse in OP patients (odds ratio [OR], 0.82; 95% confidence interval [CI], 0.70-0.97; p=0.018; OR, 1.02; 95% CI, 1.00-1.04; p=0.042; and OR, 0.06; 95% CI, 0.01-0.87; p=0.039, respectively). CONCLUSION: This study shows that FVC, PaO2/FiO2 and serum protein level at presentation can significantly predict relapse in OP patients.

Successful Immunoglobulin Treatment in Severe Cryptogenic Organizing Pneumonia Caused by Dermatomyositis / 대한중환자의학회지

In connective tissue diseases, autoantibodies cause pulmonary interstitial inflammation and fibrosis, and patients require treatment with an immunosuppressive agent such as a steroid. Dermatomyositis is an incurable, uncommon form of connective tissue disease that occasionally causes diffuse pulmonary inflammation leading to acute severe respiratory failure. In such cases, the prognosis is very poor despite treatment with high-dose steroid. In the present case, a 46-year-old man was admitted to our hospital with dyspnea. He was diagnosed with dermatomyositis combined with cryptogenic organizing pneumonia (COP) with respiratory failure and underwent treatment with steroid and an immunosuppressive agent, but the COP was not improved. However, the respiratory failure did improve after treatment with intravenous immunoglobulin, which therefore can be considered a treatment option in cases where steroids and immunosuppressive agents are ineffective.

A Case of Organizing Pneumonia Associated with FOLFIRI Chemotherapy / 결핵및호흡기질환

The combination chemotherapy of irinotecan with 5-fluorouracil and leucovorin (FOLFIRI regimen) was recently proven to be beneficial in patients with advanced colorectal cancer. Pulmonary toxicity is very rare in adverse effects of irinotecan. No case of organizing pneumonia (also known as bronchiolitis obliterans organizing pneumonia) associated with FOLFIRI chemotherapy has been reported. We experienced a case of a 62-year-old man who presented persistent dry cough and progressive dyspnea after receiving chemotherapy with FOLFIRI regimen. After surgical lung biopsy, the patient was diagnosed with FOLFIRI chemotherapy-induced organizing pneumonia which was successfully treated with steroid therapy.

Pneumonia em organização secundária ao uso de abciximab: relato de caso / Secondary organizing pneumonia after Abciximab use: case report

Relata-se o caso de paciente octogenária, com infarto agudo do miocárdio (IAM) em evolução, que desenvolveu quadro inflamatório pulmonar agudo, compatível com diagnóstico de pneumonia em organização secundária ao uso de abciximab intracoronariano, em angioplastia percutânea coronariana (APC). Esse diagnóstico foi firmado por meio de alterações clínicas, radiográficas e tomográficas típicas e pela regressão dessas alterações após terapia com corticosteroide.

This report presents the case of an octogenarian female patient with acute myocardial infarction who developed an acute inflammatory pulmonary condition compatible with a diagnosis of secondary organizing pneumonia after intracoronary abciximab during percutaneous coronary angioplasty. This diagnosis was grounded on typical clinical, radiographic and tomographic alterations and the regression of these alterations after corticoid treatment.

Clinical Significance of Serum Autoantibodies in Idiopathic Interstitial Pneumonia

Although autoantibodies are routinely screened in patients with idiopathic interstitial pneumonia, there are no reliable data on their clinical usefulness. The aim of this study was to investigate the prognostic value of autoantibodies for predicting the development of new connective tissue disease in these patients and also mortality. We conducted retrospective analysis of the baseline, and follow-up data for 688 patients with idiopathic interstitial pneumonia (526 with idiopathic pulmonary fibrosis, 85 with nonspecific interstitial pneumonia, and 77 with cryptogenic organizing pneumonia) at one single tertiary referral center. The median follow-up period was 33.6 months. Antinuclear antibody was positive in 34.5% of all subjects, rheumatoid factor in 13.2%, and other specific autoantibodies were positive between 0.7%-6.8% of the cases. No significant difference in patient survival was found between the autoantibody-positive and -negative groups. However, the presence of autoantibodies, especially antinuclear antibody with a titer higher than 1:320, was a significant predictor for the future development of new connective tissue diseases (relative risk, 6.4), although the incidence was low (3.8% of all subjects during follow-up). In conclusion, autoantibodies are significant predictors for new connective tissue disease development, although they have no prognostic value.

Insuficiência respiratória aguda causada por pneumonia em organização secundária à terapia antineoplásica para linfoma não Hodgkin / Acute respiratory failure caused by organizing pneumonia secondary to antineoplastic therapy for non-Hodgkin's lymphoma

Doenças difusas do parênquima pulmonar pertencem a um grupo de doenças de evolução geralmente subaguda ou crônica, mas que podem determinar insuficiência respiratória aguda. Paciente masculino, 37 anos, em terapia para linfoma não Hodgkin, admitido com tosse seca, febre, dispneia e insuficiência respiratória aguda hipoxêmica. Iniciadas ventilação mecânica e antibioticoterapia, porém houve evolução desfavorável. Tomografia computadorizada de tórax mostrava opacidades pulmonares em "vidro fosco" bilaterais. Devido ao paciente ter feito uso de três drogas relacionadas à pneumonia em organização (ciclofosfamida, doxorrubicina e rituximabe) e quadros clínico e radiológico serem sugestivos, iniciou-se pulsoterapia com metilprednisolona com boa resposta. Pneumonia em organização pode ser idiopática ou associada a colagenoses, drogas e neoplasias, e geralmente responde bem a corticoterapia. O diagnóstico é anatomopatológico, mas condições clínicas do paciente não permitiam a realização de biópsia pulmonar. Pneumonia em organização deve ser diagnóstico diferencial em pacientes com aparente pneumonia de evolução desfavorável ao tratamento antimicrobiano.

Interstitial lung diseases belong to a group of diseases that typically exhibit a subacute or chronic progression but that may cause acute respiratory failure. The male patient, who was 37 years of age and undergoing therapy for non-Hodgkin's lymphoma, was admitted with cough, fever, dyspnea and acute hypoxemic respiratory failure. Mechanical ventilation and antibiotic therapy were initiated but were associated with unfavorable progression. Thoracic computed tomography showed bilateral pulmonary "ground glass" opacities. Methylprednisolone pulse therapy was initiated with satisfactory response because the patient had used three drugs related to organizing pneumonia (cyclophosphamide, doxorubicin and rituximab), and the clinical and radiological symptoms were suggestive. Organizing pneumonia may be idiopathic or linked to collagen diseases, drugs and cancer and usually responds to corticosteroid therapy. The diagnosis was anatomopathological, but the patient's clinical condition precluded performing a lung biopsy. Organizing pneumonia should be a differential diagnosis in patients with apparent pneumonia and a progression that is unfavorable to antimicrobial treatment.

Neumonía organizada criptogénica como presentación inicial de la artritis reumatoide / Cryptogenic organizing pneumonia as initial presentation of rheumatoid arthritis

Se presenta el caso de una mujer de 65 años a quien se diagnostica neumonía organizada criptogénica (NOC), como primera manifestación de artritis reumatoide (AR). La paciente presentó tos y posteriormente disnea. La tomografía computarizada de tórax mostró opacidades parenquimatosas con broncograma de aire en los lóbulos superiores. Ocho semanas después presenta criterios clínicos y serológicos para AR, se diagnosticó NOC por biopsia abierta de pulmón, con excelente respuesta clínica y radiológica al tratamiento con corticosteroides. (Acta Med Colomb 2012; 37: 27-30).

The case of a 65 year old woman who was diagnosed with cryptogenic organizing pneumonia (NOC) as a first manifestation of rheumatoid arthritis (RA). The patient subsequently developed cough and dyspnea. Chest computed tomography showed parenchymal opacities with air bronchogram in the upper lobes. Eight weeks later, he presented clinical and serological criteria for RA, NOC diagnosis by open lung biopsy, with excellent clinical and radiological response to treatment with corticosteroids. (Acta Med Colomb 2012; 37: 27-30).

A Case of Bronchilolitis Obliterans Organizing Pneumonia in a Patient with Ulcerative Colitis / 결핵및호흡기질환

The ulcerative colitis is a chronic inflammatory bowel disease with an unknown etiology. The major symptoms of ulcerative colitis are diarrhea, abdominal pain and hematochezia. However, arthritis, skin disorders, hepatobiliary inflammation and uveitis are occasionally recognized as systemic complications. Although there are few reports of coexistent pulmonary and inflammatory bowel disease, the lung is not generally considered to be a target organ in ulcerative colitis. We report a patient with ulcerative colitis-related bronchilolitis obliterans organizing pneumonia confirmed by video-assisted thoracoscopic surgery, who responded to corticosteroid therapy.

Bronchiolitis Obliterans Organizing Pneumonia in the Patient with Non-Small Cell Lung Cancer Treated with Docetaxel/Cisplatin Chemotherapy: A Case Report / 결핵및호흡기질환

A 60-year-old man was diagnosed with stage IV squamous cell carcinoma of lung and treated with weekly doses of docetaxel and cisplatin. Tumor mass and mediastinal lymphadenopathy disappeared after 4.5 cycles of chemotherapy. At one week post final chemotherapy, the patients developed sudden shortness of breath. New, multifocal infiltrations developed on both lungs without definitive evidence of infection. Despite administration of broad spectrum antibiotics, the lung lesion did not improve, so bronchoalveolar lavage and computed tomography-guided lung biopsy were performed. The proportion of lymphocytes was increased markedly and histopathology revealed squamous cell carcinoma combined with bronchiolitis obliterans organizing pneumonia. After high dose corticosteroid therapy, dyspnea and the newly developed consolidation had decreased slightly. However, dyspnea and hypoxemia increased again because of aggravated lung cancer since chemotherapy had stopped. Chemotherapy couldn't be restarted due to the poor performance status of the patient. Later, patient died of respiratory failure from poor general condition and progression of lung cancer.