Neumonía por Pneumocystis jirovecii asociada a hemorragia alveolar difusa en un paciente con trasplante renal

La neumonía por Pneumocystis jirovecii es una enfermedad fúngica oportunista descrita principalmente en pacientes con VIH, sin embargo, tras la introducción de la TARV, ha incrementado su incidencia en pacientes con inmunosupresión no asociada a VIH, como neoplasias hematológicas y trasplantes de órganos sólidos. Presentamos el caso de un varón de 17 años, receptor de un trasplante renal, con inmunosupresión prolongada con corticoesteroides, con cuadro clínico de tos, disnea y fiebre. La TC mostró micronódulos pulmonares centrolobulillares y vidrio esmerilado. El LBA fue compatible con hemorragia alveolar difusa (HAD), con RPC positiva para P. jirovecii. Se descartaron otras infecciones y enfermedades autoinmunes. Recibió tratamiento con cotrimoxazol con buena evolución clínica y mejoría radiológica. Si bien las causas más frecuentes de HAD son etiologías autoinmunes como enfermedades reumatológicas o vasculitis, es prioritario descartar causas infecciosas, incluyendo P. jirovecii, ya que el tratamiento dirigido puede tener un impacto significativo en la mortalidad en este grupo de pacientes.

Pneumocystis jirovecii pneumonia is an opportunistic fungal infection, described mainly in HIV patients, however, after the introduction of ART, its presentation has increased in patients with non-HIV immunosuppression, such as hematological cancers, solid or hematopoietic stem cell transplantation. We report the case of a 17-year-old male, kidney transplant patient, with prolonged immunosuppression with corticoesteroids, with history of cough, dyspnea, and fever. Chest CT evidences centrilobular pulmonary micronodules with ground glass. BAL was performed compatible with diffuse alveolar hemorrhage, with positive PCR for P. jirovecii. Other infections and autoimmune disease were ruled out. He received treatment with cotrimoxazole with clinical improvement of the patient, and follow up chest CT at the end of treatment showed decrease of pulmonary infiltrates. Although the most frequent causes of DAH are autoimmune etiologies such as rheumatic diseases or vasculitis, it is a priority to rule out infectious causes, including P. jirovecii, since targeted treatment could have a significant impact on mortality outcomes in this group of patients.

A rare case of post-ictal diffuse alveolar hemorrhage due to seizures

Diffuse alveolar hemorrhage is persistent or recurrent pulmonary hemorrhage that occurs due to a variety of causes. Here, we present the case of a 15-year-old male child who presented with chief complaints of involuntary jerking movements of the entire body in the morning followed by coughing out a massive amount of blood. At presentation, the patient’s blood glucose level was high. Bronchoscopy revealed bleeding in the middle and lower lobes of both lungs. Computed tomography (CT) brain was suggestive of cerebral edema and the CT chest was suggestive of diffuse opacities in bilateral lung fields. The patient was started on corticosteroids, antiplatelet drugs, antiepileptics, insulin, and oxygen inhalation which helped the patient to recover and was discharged in a week’s time.

Importancia de los macrófagos cargados con hemosiderina en el diagnóstico de la hemorragia alveolar difusa / Importance of hemosiderin-loaded macrophages in diagnosis of diffuse alveolar hemorrhage / A importância dos macrófagos carregados com hemosiderina no diagnóstico de hemorragia alveolar difusa / Reconocimiento a la trayectoria del Prof. Dr. Juan Miguel Castagnino†

Resumen La hemorragia alveolar difusa (HAD) es un síndrome clínico con una alta mortalidad que compromete la función respiratoria. Su diagnóstico se basa en pruebas clínicas, radiológicas y citológicas. El objetivo del trabajo fue ratificar el valor de referencia de hemosiderófagos en lavados broncoalveolares (BAL) (hemosiderófagos ≥20%), correlacionar con la etiología y definir las condiciones preanalíticas para que la reacción de Perls alcance valores elevados de sensibilidad. De 109 muestras de pacientes con sospecha de HAD, se analizaron 90 por cumplir los criterios de inclusión; 36 resultaron positivas para HAD, 3 falsamente negativas y 51 resultaron negativas. La sensibilidad fue de 92% y la especificidad de 100%. La mediana de hemosiderófagos para muestras con diagnóstico de HAD fue de 70%. Se agruparon según la etiología: procesos infecciosos puros (PI), enfermedades autoinmunes puras (EA), enfermedades neoplásicas puras (EN), enfermedades autoinmunes más procesos infecciosos (EA+PI), enfermedades neoplásicas más procesos infecciosos (EN+PI), misceláneas (MI). La mediana de hemosiderófagos para cada grupo fue: PI (n=7) 50%, EA (n=15) 58%, EN (n=6) 73%, EA+PI (n=5) 80%, EN+PI (n=4) 80%, MI (n=2) 45% (p=0,57). El porcentaje de pacientes fallecidos fue de 49% (n=19), con una mediana de hemosiderófagos de 70%, en comparación con la de pacientes no fallecidos de 64% (p=0,25). Se ratificó el valor de referencia para establecer el diagnóstico de HAD en muestras de BAL obtenidas luego de las 36 h de comenzados los síntomas utilizando la reacción de Perls, la cual demuestra una alta sensibilidad y especificidad para dicho diagnóstico.

Abstract Difusse alveolar hemorrhage (DAH) is a clinical syndrome with high mortality. Its diagnosis is based on clinical, radiological and cytological tests. The objective of this study was to ratify the reference value of hemosiderophages in bronchoalveolar lavages (BAL) (hemosiderophagues ≥20%), to correlate with the etiology and define the pre-analytical conditions for the Perls reaction to reach high sensitivity values. Out of the 109 samples from patients with suspected ADH, 90 were analysed for meeting the inclusion criteria; 36 were positive for HAD, 3 were false negatives, and 51 were negative (sensitivity 92%; specificity 100%). The median number of hemosiderophagues for samples with a diagnosis of ADH was 70%; they were grouped according to etiology: pure infectious processes (PI), pure autoimmune diseases (AD), pure neoplastic diseases (ND), autoimmune diseases plus infectious processes (AD + PI), and miscellaneous (MI). The median number of hemosiderophagues for each group was: PI (n=7) 50%, AD (n=15) 58%, ND (n=6) 73%, AD + PI (n=5) 80%, ND + PI (n=4) 80%, MI (n=2) 45% (p=0.57). The percentage of deceased patients was 49% (n=19), with a median hemosiderophague of 70%, compared with 64% of non-deceased patients (p=0.25). The reference value to establish the diagnosis of ADH in BAL simples obtained 36 hours after the beginning of symptoms using the Perls reaction was ratified, which shows a high sensitivity and specificity to make the diagnosis of ADH.

Resumo A hemorragia alveolar difusa (HAD) é uma síndrome clínica com alta mortalidade que compromete a função respiratória. Seu diagnóstico se baseia em testes clínicos, radiológicos e citológicos. O objetivo do trabalho foi ratificar o valor de referência de hemossiderófagos em lavagens broncoalveolares (LBA) (hemossiderófagos ≥20%), relacioná-los com a etiologia e definir as condições pré-analíticas para que a reação de Perls alcance valores elevados de sensibilidade. De 109 amostras de pacientes com suspeita de HAD, 90 foram analisadas para cumprir com os critérios de inclusão; 36 resultaram positivas para HAD, 3 foram falsos negativos e 51 resultaram negativas. A sensibilidade foi de 92% e a especificidade de 100%. A média de hemossiderófagos para amostras com diagnóstico de HAD foi de 70%, eles foram agrupados de acordo com a etiologia: processos infecciosos puros (PI), doenças autoimunes puras (DA), doenças neoplásicas puras (DN), doenças autoimunes mais processos infecciosos (DA+PI), doenças neoplásicas mais processos infecciosos (DN+PI), miscelâneas (MI). A média de hemossiderófagos para cada grupo foi: PI (n=7) 50%, DA (n=15) 58%, DN (n=6) 73%, DA+PI (n=5) 80%, DN+PI (n=4) 80%, MI (n = 2) 45% (p= 0,57). A porcentagem de pacientes falecidos foi de 49% (n=19), com uma média de hemossiderófagos de 70%, em comparação com 64% de pacientes não falecidos (p=0,25). Foi ratificado o valor de referência para estabelecer o diagnóstico de HAD em amostras LBA obtidas 36 horas após o início dos sintomas através da reação de Perls, que apresenta alta sensibilidade e especificidade para esse diagnóstico.

Diffuse alveolar hemorrhage complicating dengue haemorrhagic fever in a 15-year-old boy: A case report

Rationale: Dengue fever is a viral infection that is spread through the bites of infected female Aedes mosquitos. It can cause life threatening complications, including dengue haemorrhagic fever (DHF) and dengue shock syndrome. Patient concerns: A 15-year-old male presented with fever and petechiae and later developed hemoptysis. Diagnosis: Dengue fever with DHF with diffuse alveolar hemorrhage. Interventions: Invasive ventilation with high positive end expiratory pressure, multiple transfusions of packed red blood cells, fresh frozen plasma, single donor platelets and inotropic support Outcomes: The patient was stabilized and discharged on minimal supplemental oxygen. Lessons: Diffuse alveolar hemorrhage, although very rare, should be considered in a patient with dengue who presents with hemoptysis. The treatment is directed at providing respiratory and circulatory support, and preventing the progression of microcirculation damage.

Diffuse Alveolar Hemorrhage: A rare fatal complication of systemic Lupus Erythematosus (A report of two cases)

@#We report two Filipino women with systemic lupus erythematosus (SLE) who developed diffuse alveolar hemorrhage (DAH), a rare, life-threatening complication associated with a high mortality rate. DAH should be suspected in patients with SLE presenting with new pulmonary infiltrates, a decline in hemoglobin, hemoptysis, dyspnea, and persistent desaturation. The first patient is 23 years old and was diagnosed with SLE 8 years ago; initially presenting with malar rash, oral ulcers, nephritis, and positive antinuclear antibodies (ANA). She had a poorly controlled disease and was admitted for facial and bipedal edema due to lupus nephritis. She was given 1 gram of methylprednisolone intravenously (IV) for three consecutive days. She then became tachypneic producing bloody secretions, with desaturation and sudden decline in hemoglobin. She was given cyclophosphamide 1 gram IV and referred for plasmapheresis but eventually succumbed. The second patient is 56 years old with generalized body weakness. Laboratory workup showed nephritis, anemia, ANA, low C3, and high anti-dsDNA titers. Pulse methylprednisolone 1000 mg was initiated. However, there was new-onset hemoptysis and desaturation and the patient was intubated. Bronchoscopy revealed diffuse bleeding on the right middle lobe and she eventually expired. Both patients with active SLE nephritis presented in this study died within days of DAH diagnosis. Hence, aside from early recognition to improve outcomes we should anticipate its possible occurrence in patients with high disease activity.

Characteristics and Prognosis of Microscopic Polyangiitis Patients with Diffuse Alveolar Hemorrhage and Interstitial Lung Disease / 中国医学科学杂志(英文版)

Objective To evaluate the clinical characteristics and prognostic predictors of patients with diffuse alveolar hemorrhage (DAH) and/or interstitial lung disease (ILD) secondary to microscopic polyangiitis (MPA) in a Chinese general hospital. Methods We retrospectively reviewed the medical records of MPA patients admitted to internal medicine departments between the year 2002 and 2012. The patients were divided into the ILD, DAH, DAH combined with ILD (DAHILD), and no pulmonary involvement (NPI) groups according to pulmonary involvement patterns. The clinical characteristics at diagnosis were analyzed. The risk factors associated with short-term death and long-term death were identified with Logistic regression and Cox analysis.Results Of 193 newly diagnosed MPA patients, 181 patients were enrolled in the research, of which 19 had DAH alone, 96 had ILD alone, 18 had DAH and DAH concurrently, and 48 had NPI. The median of serum creatine level in the DAH group was 449 μmol/L, significantly higher than that in the ILD group (123 μmol/L, Nemenyi = -35.215, P = 0.045) and DAHILD group (359 μmol/L, Nemenyi = -43.609, P = 0.007). The median follow-up time was 67 (range: 1-199) months. Patients in the ILD group were older than those in the DAH group (median: 69 years vs. 57 years, Nemenyi = 43.853, P= 0.005). The patients with both DAH and ILD had combined features of the two subtypes, and the highest mortality (72.2% at the end of follow-up). The elevated white blood cell count was a risk factor for short-term death (OR = 1.103, 95%CI: 1.008-1.207, P = 0.032 for one month; OR = 1.103, 95%CI: 1.026-1.186, P = 0.008 for one year). Old age (HR= 1.044, 95%CI: 1.023-1.066, P < 0.001), cardiovascular system involvement (HR = 2.093, 95%CI: 1.195-3.665, P = 0.010), poor renal function (HR = 1.001, 95%CI: 1.000-1.002, P = 0.032) were risk factors for long-term death. Pulmonary infections (38/54) were the leading causes of death, especially for the patients with ILD. Besides, 49 patients suffered from pulmonary infections in the first year after diagnosis. Conclusions MPA patients who presented with different pulmonary involvement patterns have completely different clinical features. These subtypes probably have different pathogenesis and should be studied separately.

Síndrome hemorrágico pulmonar grave por leptospira / Leptospirosis-Associated Severe Pulmonary Hemorrhagic Syndrome

La leptospirosis es una zoonosis con expresión clínica muy variable. El síndrome pulmonar hemorrágico grave se trata de una forma infrecuente de la enfermedad con una elevada mortalidad. Se presenta caso de una mujer joven, residente de zona urbana que consultó por fiebre, expectoración hemoptoica y disnea evolucionando con insuficiencia ventilatoria y sangrado en la vía aérea. Se obtuvo el diagnóstico mediante pruebas serológicas y antecedente epidemiológico

Leptospirosis is a zoonosis of very variable clinical expression. The severe pulmonary hemorrhagic syndrome is an uncommon form of the disease, with high mortality rates. We report the case of a young woman who lives in an urban area and consulted with the physician for fever, hemoptoic expectoration and dyspnea, and developed ventilatory failure and bleeding of the airway. The diagnosis was obtained by means of serologic tests and epidemiological history.

A case of diffuse alveolar hemorrhage following synthetic cathinone inhalation / 世界急诊医学杂志（英文）

@#Dear editor, Diffuse alveolar hemorrhage (DAH) sometimes causes a life-threatening condition; thus, prompt diagnosis and treatment for DAH is crucial. However, a variety of diseases (e.g., systemic autoimmune diseases, infectious diseases, drugs) are associated with the development of DAH, which occasionally causes diffi culty with identifying the specifi c etiology.[1

Hemorragia alveolar difusa secundaria a vasculitis asociada a ANCA. Recaída durante hemodiálisis / Diffuse alveolar hemorrhage secondary to ANCA-associated vasculitis. Relapse during hemodialysis

Resumen La hemorragia alveolar difusa (HAD) masiva es una complicación inusual de los pacientes con vasculitis ANCA, frecuentemente amenaza la vida y está asociada con una mortalidad de hasta el 100%. La información en la literatura acerca del tratamiento en casos refractarios y cuando el paciente se encuentra en diálisis es escasa. Se presenta el caso de un paciente con vasculitis p-ANCA con compromiso renal y pulmonar en el escenario de síndrome pulmón-riñón, con múltiples recaídas de hemorragia alveolar a pesar de tratamiento con corticoide, azatioprina, ciclofosfamida y terapia de recambio plasmático. Se instauró manejo con anticuerpo monoclonal anti CD20 e inmunoglobulina, logrando resolución del episodio de hemorragia alveolar y permaneciendo sin actividad. Se resalta la utilidad del rituximab como estrategia terapéutica en casos refractarios. (Acta Med Colomb 2019; 44: 111-114).

Abstract Massive diffuse alveolar hemorrhage (DAH) is an unusual complication of patients with ANCA vasculitis that frequently threatens life and is associated with mortality up to 100%. In formation in the literature about treatment in refractory cases and when the patient is on dialysis is scarce. The case of a patient with p-ANCA vasculitis with renal and pulmonary involvement in the lung-kidney syndrome scenario, with multiple relapses of alveolar hemorrhage despite treatment with corticosteroid, azathioprine, cyclophosphamide and plasma exchange therapy is presented. Management with anti-CD20 monoclonal antibody and immunoglobulin was estab lished, achieving resolution of the episode of alveolar hemorrhage and remaining without activity. The usefulness of rituximab as a therapeutic strategy in refractory cases is highlighted. (Acta Med Colomb 2019; 44: 111-114).

Diffuse alveolar hemorrhage syndrome secondary to bocavirus and rhinovirus infection in a pediatric patient / Síndrome de hemorragia alveolar difusa secundaria a coinfección por Rhinovirus y Bocavirus en un paciente pediátrico

Abstract The diffuse alveolar hemorrhage syndrome is characterized by the presence of blood in the pulmonary alveolus from arterioles, venules and pulmonary capillaries, as a consequence of the lesion of the alveolar wall and without an endobronchial alteration. Its presentation includes a classic triad of hemoptysis, anemia and diffuse alveolar infiltrates. It's a rare but potentially fatal entity and there are no clear data on its real incidence in the pediatric population. We present the case of a previously healthy pediatric patient, immunocompetent, who presented diffuse alveolar hemorrhage syndrome with secondary ventilatory failure. After discarding all possible etiologies, coinfection by Rhinovirus and human Bocavirus was detected through the polymerase chain reaction, determining them as causal factors of the event. Recently, viral infections have been postulated as causing serious lung disease, especially coinfection in immunocompromised patients, in this case Rhinovirus and human Bocavirus; however there are no reports on the syndrome caused by these viruses.

Resumen El síndrome de hemorragia alveolar difusa se caracteriza por la presencia de sangre los alveolos pulmonar procedente de arteriolas, vénulas y capilares pulmonares, como consecuencia de la lesión de la pared alveolar y sin identificársele una alteración endobronquial. Su presentación incluye una triada clásica de hemoptisis, anemia e infiltrados alveolares difusos. Es una entidad poco frecuente aunque potencialmente fatal y no hay datos claros de su real incidencia en la población pediátrica. Se presenta el caso de un paciente pediátrico previamente sano, inmunocompetente, quien presentó síndrome de hemorragia alveolar difusa con falla ventilatoria secundaria. Después de descartar todas las posibles etiologías, se detectó, a través de reacción en cadena de polimerasa, coinfeccion por Rhinovirus y Bocavirus humano, determinándolos como causales del evento. Recientemente se postula las infecciones virales como causantes de enfermedad pulmonar grave, en especial la coinfeccion en pacientes inmunocomprometidos, en este caso Rhinovirus y Bocavirus humano, sin embargo no existen reportes sobre el síndrome causada por estos virus.

A case of life-threatening intraoperative diffuse alveolar hemorrhage during a femur fracture operation with suspected fat embolism: A case report

Diffuse alveolar hemorrhage (DAH) is a rare manifestation of trauma or long bone fracture. A relationship between fat embolism and DAH has been reported. A 73-year-old female developed sudden cardiac arrest during a femur fracture operation. Cardiopulmonary resuscitation (CPR) was repeated for about 130 minutes. During CPR, blood was detected in the endotracheal tube. Diffuse patch ground glass opacity appearance and increased pulmonary artery with bulging of interventricular septum toward the left ventricle were detected on a chest computed tomography scan. After full supportive care including ventilator therapy, the patient's condition became stabilized and she was extubated after 7 days. We report a case of DAH in the course of a suspected fat embolism during femur fracture operation. Although DAH is a rare manifestation of fat embolism, early diagnosis and aggressive treatment likely can decrease morbidity and mortality.

Clinical analysis of 34 cases with diffuse alveolar hemorrhage in systemic lupus erythematosus / 实用医学杂志

Objective To identify the clinical characteristics and therapeutic effect of systemic lupus erythematosus (SLE) patients with diffuse alveolar hemorrhage (DAH).Methods Clinical characteristcs,diagnosis,treatment and outcome in 34 patients hospitalized in The First Affiliated Hospital of Guangxi Medical University from January 2006 to December 2016 were retrospectively analyzed.Results The incidence of DAH involvement of SLE was about 0.39％.Median age was 19 (interquartile range (IQR) 11.75 ～ 32) years.The duration of SLE before clinical DAH was 6 months (IQR 2 ～ 15.75) months.The main clinical manifestations of DAH were cough,dyspnea and fever.The SLE disease activity index (SLEDAI) score was 19.5 (16 ～ 25)points and anti-dsDNA antibody titer elevated markedly (38.2％).The overall mortality rate was 73.5％,however patients who chose department of rheumatism have lower mortality (52.9％).And treatment of CTX was associated with mortality (OR =0.059,95％ CI 0.006 ～ 0.554,P =0.013),as well as steroids and immunosuppressant combination therapy.Conclusions The clinical symptoms of SLE with DAH is often atypical.There is manifestation of cough,fever,dyspnea and etc.Imaging and broncoscopy can assist the diagnosis and its prone to the pulmonary infection and high mortality.Early diagnosis and application of CTX combined with conventional dose of hormone theraphy can in early diagnosis and reduce the mortality.

Pulmonary metastatic angiosarcoma presenting with diffuse alveolar hemorrhage: 9 case reports / 中华内科杂志

Objective@#Pulmonary metastatic angiosarcoma is a rare, fatal disease that often presents as diffuse alveolar hemorrhage(DAH). In this report, clinical characteristics of pulmonary metastatic angiosarcoma were retrospectively reviewed.@*Methods@#A total of 9 patients with angiosarcoma who presented as DAH were enrolled. Clinical data included age, gender, symptoms, smoking status, physical exam findings, pulmonary function tests, and radiology.@*Results@#All patients were male with median age 41 years(range, 22 to 57 years). The most common symptom was hemoptysis(9/9). Other symptoms included dyspnea (5/9), cough(3/9), chest pain(3/9), fever(2/9,) and edema of the lower extremity and oliguria(4/9).The common misdiagnoses were tuberculosis(4/9), vasculitis(3/9) and other infection(1/9). Chest computed tomography showed bilateral,random distributed different-sized nodules(9/9),as well as ground-glass areas (9/9).The hearts, mainly right atrium, were the most common primary locations(7/9).Cardiac mass was the first manifestation in five patients by echocardiography(5/8).Two atrial masses were identified by computer tomography pulmonary angiography and magnetic resonance imaging respectively. Transbronchial lung biopsy failed to find malignancy. Computer tomography guided transthoracic needle biopsy was difficult to perform in most patients. Eight patients were diagnosed by surgical biopsy, either by lung biopsy(4/8) or cardiac biopsy(4/8).The median survival period was only 3 months after surgery.@*Conclusion@#Metastatic pulmonary angiosarcoma should be considered in patients with DAH and multiple glass ground opacity and nodules on chest CT. Careful cardiologic monitoring is necessary. Surgical biopsy is reliable for diagnosis.

Síndrome pulmón riñón, reporte de caso y revisión de la literatura / Pulmonary-renal syndrome, case report and literature review / Síndrome Pulmão-Rim, relato de um caso e revisão da literatura

Resumen: El complejo del síndrome pulmón riñón (SPR) se define como la combinación de hemorragia alveolar difusa acompañada de glomerulonefritis rápidamente progresiva, dentro del protocolo de diagnóstico se requieren niveles de anticuerpos anticitoplasma de neutrófilos (ANCA), broncoscopia, tomografía de tórax y estudio de sedimento urinario, es más común que se manifieste en el sexo masculino entre la edad de 40 y 55 años, siendo infrecuente en población joven. Se han descrito tres formas principales, la granulomatosis con poliangeítis, síndrome de Churg Strauss y síndrome de Goodpasture (SGP). En cualquiera de sus presentaciones se asocia a una elevada tasa de mortalidad que requiere manejo multidisciplinario con soporte ventilatorio, sustitución de la función renal, manejo específico con esteroide sistémico y terapia inmunosupresora. La introducción de la ciclofosfamida en combinación con esteroides anunció una alternativa en el tratamiento de las vasculitis observando disminución en la mortalidad en comparación con los glucocorticoides como monoterapia. El objetivo de este artículo es presentar el caso de un paciente con SPR y revisar la patogénesis, el abordaje diagnóstico y tratamiento, haciendo énfasis en la importancia del manejo multidisciplinario en la unidad de cuidados intensivos.

Abstract: The complex syndrome lung-kidney or pulmonary renal syndrome (PRS) is defined as the combination of diffuse alveolar hemorrhage accompanied by rapidly progressive glomerulonephritis, within the diagnostic protocol levels of anti-cytoplasm (ANCA), bronchoscopy, chest tomography and study required urinary sediment, occurs more commonly in males between 40 and 55 years, being rare in young people, described three main forms granulomatosis with polyangiitis, Churg Strauss syndrome Goodpasture (SGP), in any of its presentations is associated with a high mortality rate requiring multidisciplinary management with ventilatory support, replacement of renal function, specific management with systemic steroid and immunosuppressive therapy. The introduction of cyclophosphamide in combination with steroids announced an alternative in the treatment of vasculitis finding decrease mortality compared with glucocorticoids as monotherapy. The aim of this paper is to present the case of a patient with PSR, review the pathogenesis, diagnosis and treatment approach, emphasizing the importance of multidisciplinary management in the Intensive Care Unit.

Resumo: A complexa síndrome do Pulmão-Rim (SPR) é definida como a combinação de hemorragia alveolar difusa e glomerulonefrite rapidamente progressiva, dentro do protocolo de diagnóstico é necessário níveis de anticorpos anti citoplasmáticos de neutrófilos (ANCA), broncoscopia, tomografia torácica e estudo do sedimento urinário. A síndrome é mais frequente em homens entre 40 e 55 anos, sendo raro na população jovem. Descreveu-se três formas principais: Granulomatose com Poliangeíte, Síndrome de Churg Strauss e síndrome de Good-Pasture (SGP), em qualquer das suas apresentações é associada com uma alta taxa de mortalidade, requerendo uma abordagem multidisciplinar com suporte respiratório, substituição da função renal, tratamento específico com esteróide sistêmico e terapia imunossupressora. A introdução da ciclofosfamida combinada com esteróides é uma alternativa no tratamento da vasculite, diminuindo a mortalidade, em comparação aos glucocorticóides como monoterapia. O objetivo deste artigo é apresentar o caso de um paciente com SPR, revisar a patogênese, a abordagem diagnóstica e o tratamento, enfatizando a importância do tratamento multidisciplinar na unidade de terapia intensiva.

Cytoplasmic Anti-Neutrophil Cytoplasmic Antibody Positive Diffuse Alveolar Hemorrhage Associated with Methimazole

Diffuse alveolar hemorrhage (DAH) is a life-threatening condition associated with many disorders. Here, we report a case of 59-year-old female who had diffuse alveolar hemorrhage associated with methimazole. She had been treated with methimazole for two weeks due to the recurrence of Grave's disease, before visiting the emergency room. She had to be intubated on the 3rd day of hospitalization because of unabated massive hemoptysis and rapid progression of diffuse alveolar infiltration on chest radiographs. Since her clinical condition improved substantially after cessation of methimazole and steroid pulse therapy, she was extubated on the 9th day of hospitalization and then discharged. After discharge, DAH did not recur with cessation of steroid and she had radioactive iodine therapy for her Grave's disease. This was a rare and interesting case of life-threatening DAH associated with cytoplasmic-antineutrophil cytoplasmic antibody and methimazole.

Advances in diagnosis and treatment of diffuse alveolar hemorrhage / 中华实用儿科临床杂志

Diffuse alveolar hemorrhage (DAH) is a life threatening clinical syndrome caused by a variety of causes.Early identification and etiological diagnosis of DAH in children are challenging.Despite some advances have been made in the identification and management of DAH,the mortality rate is still high.This article aims to raise the cognition of clinicians on DAH by providing a general review of some recent researches.

Treatment and risk factors of diffuse alveolar hemorrhage in 62 children / 临床儿科杂志

Objective To investigate the possible risk factors for prognosis of diffuse alveolar hemorrhage (DAH) in children and to improve the recognition of the disease.Methods The study included 62 DAH pediatric patients hospitalized from January, 2006 to January, 2016. Clinical data were retrospectively analyzed. According to the basic diseases, children were divided into immune associated DAH and non-immune associated DAH to explore the effect of early glucocorticoid treatment on the two groups of DAH. Based on the prognosis, the patients were divided into the death group and the survival group to analyze its related risk factors.Results Of the 62 patients, 20 were of immune associated DAH, 42 of non-immune associated DAH. There was no signiflcant difference of early treatment with glucocorticoid between the two groups (P>0.05). In our cohort, 30 patients died, the total mortality was 48.4% (30/62). Pediatric critical illness score may be the independent risk factor for DAH mortality.Conclusions DAH is an acute, life-threatening event, the lower the pediatric critical illness score, the higher risk of death.

Hemorragia alveolar difusa: Diez años de experiencia / Diffuse Alveolar Hemorrhage. Ten Year Experience

Introducción: Las manifestaciones registradas de la hemorragia alveolar difusa (HAD) están influenciadas por las características de los pacientes y la experiencia de los centros de referencias donde son asistidos. Objetivos: Describir los hallazgos clínicos y de laboratorio, las etiologías y los factores de riesgo de mortalidad en pacientes con HAD. Métodos: Análisis retrospectivo de los informes de flbrobroncoscopias en pacientes con diagnóstico de HAD (período 2003-2013), según etiologías inmunológicas y no inmunológicas. Resultados: Se identiflcaron 20 pacientes que presentaron los signos clínicos y patológicos de HAD sobre un total de 6839 flbrobroncoscopias (0,3%). La edad mediana fue 57 años (rango 20-88). Los signos más frecuentes de presentación fueron disnea e inflltrados radiológicos pulmonares en el 100% de los casos y anemia en el 95%. La clásica tríada semiológica (inflltrados radiológicos pulmonares, anemia y hemoptisis) de la HAD se encontró solo en 4 casos, 20%. La etiologías no inmunológicas fueron las más frecuentes (15 casos, 75%), especialmente infecciosas, oncohematológicas y cardiovasculares. La mediana de estadía hospitalaria fue de 17.5 días (rango 2-90 días). Doce pacientes fueron tratados en la UTI. La letalidad fue 60% (12/20 pacientes). Los principales riesgos de muerte fueron pacientes inmunocomprometidos (OR 27.50; IC 95%: 1.99 - 378.00, p = 0.013), necesidad de asistencia respiratoria mecánica (OR 18.00; IC 95%: 1.49-216.00, p = 0.023) y estadía en UTI (OR 7.50; IC 95%: 0.92-61.00, p = 0.049). La mortalidad a los 14 días de internación en el grupo no inmunológico fue superior a la del grupo inmunológico (p = 0.007) pero la mortalidad global no fue diferente (p = 0.066). Conclusiones: En nuestra serie, los principales signos clínicos fueron disnea, anemia e inflltrados pulmonares; pero la tríada clásica fue de rara observación. En todos nuestros pacientes fue posible adscribir la HAD a una etiología deflnida. Las principales etiologías fueron no inmunológicas. La estadía en UTI y la necesidad de ARM se asociaron a mayor riesgo de mortalidad. La mortalidad a mediano plazo no fue diferente entre ambos grupos.

Introduction: The registered manifestations of Diffuse Alveolar Hemorrhage (DAH) are influenced by the characteristics of the patients and the experience of the referral centers where they were assisted. Objectives: To describe clinical and laboratory findings, etiologies and risk factors for mortality of patients with DAH. Methods: A retrospective analysis of fibrobronchoscopy reports in patients with DAH diagnosis was carried out for the period 2003-2013, according to to immunological and non-immunological etiologies. Results: Twenty patients with clinical and pathologic signs of DAH were identified, mean age 57 years old (range 20-88). The most common signs of presentation were dyspnea and radiologic pulmonary infiltrates (100%), and anemia (95%). The classical clinical semiology of three signs (radiologic pulmonary infiltrates, anemia and hemoptisis) was present only in 4 cases (20%). The most frequent etiologies were no immunological (75%), especially infectious, oncohematologic and cardiovascular etiologies. The median hospital stay was 17.5 days (range 2-90 days). Twelve patients were admitted into the Intensive Care Unit. Case fatality was 60% (12/20 patients). The main risk factors for death were immunocompromised patients (OR 27.50; IC 95%: 1.99-378.00, p = 0.013), need for prescription of mechanical ventilation (OR was 18.00; IC 95%: 1.49-216.00, p = 0.023) and intensive care unit admission (OR 7.50 (IC 95%: 0.92-61.00, p = 0.049). Mortality at 14 days in the group not immunological was lower (p=0.007) but the overall mortality was not different (p=0.066). Conclusion: The main clinical manifestation was dyspnea, anemia and pulmonary infiltrates, while the classic triad was infrequent. In all the cases it was possible to attribute the DAH to a defined etiology. The main etiologies were no immunological. The stay in the intensive care unit, the necessity for mechanical ventilation and immunological etiologies were associated with a higher risk of mortality. The medium-term mortality was not different between the two groups.

Diffuse alveolar hemorrhage and recombinant factor VIIa treatment in pediatric patients / 소아과

Diffuse alveolar hemorrhage (DAH) is a life-threatening pulmonary complication in patients with hematologic malignancies or autoimmune disorders. The current treatment options, which include corticosteroids, transfusions, extracorporeal membrane oxygenation (ECMO), and immunosuppressants, have been limited and largely unsuccessful. Recombinant activated factor VII (rFVIIa) has been successfully administered, either systemically or bronchoscopically, to adults for the treatment of DAH, but there are few data on its use in pediatric patients. The current literature in the PubMed database was reviewed to evaluate the efficacy and risk of rFVIIa treatment for DAH in pediatric patients. This review discusses the diagnosis and treatment of DAH, as well as a new treatment paradigm that includes rFVIIa. Additionally, the risks and benefits of off-label use of rFVIIa in pediatric patients are discussed.

Diffuse alveolar hemorrhage and recombinant factor VIIa treatment in pediatric patients / 소아과

Diffuse alveolar hemorrhage (DAH) is a life-threatening pulmonary complication in patients with hematologic malignancies or autoimmune disorders. The current treatment options, which include corticosteroids, transfusions, extracorporeal membrane oxygenation (ECMO), and immunosuppressants, have been limited and largely unsuccessful. Recombinant activated factor VII (rFVIIa) has been successfully administered, either systemically or bronchoscopically, to adults for the treatment of DAH, but there are few data on its use in pediatric patients. The current literature in the PubMed database was reviewed to evaluate the efficacy and risk of rFVIIa treatment for DAH in pediatric patients. This review discusses the diagnosis and treatment of DAH, as well as a new treatment paradigm that includes rFVIIa. Additionally, the risks and benefits of off-label use of rFVIIa in pediatric patients are discussed.