Síndrome de Hamman - rich: Reporte de caso / Hamman-Rich Syndrome: case report

Resumen La neumonía intersticial aguda, también conocida como síndrome de Hamman-Rich, se caracteriza por la presencia de insuficiencia respiratoria aguda, hallazgo imagenológico de infiltrados difusos bilaterales del parénquima pulmonar y daño alveolar difuso en histopatología pulmonar. Es una patología con una mortalidad mayor al 50% debido a la falta de una terapia específica más allá del manejo sintomático y soporte ventilatorio. Se expone el caso de un paciente masculino de 56 años quien se presenta con síntomas respiratorios inespecíficos, deterioro progresivo de la oxigenación y hallazgo de opacidades en vidrio esmerilado difusas bilaterales. Además, biopsia pulmonar en la que se evidencia daño alveolar difuso en fase fibroproliferativa en quien se descarta proceso infeccioso, enfermedades autoinmunes y toxicidad medicamentosa, por lo que se hace el diagnóstico. Recibió manejo con pulsos de metilprednisolona con recuperación completa del cuadro. MÉD.UIS.2021;34(3): 103-8.

Abstract Acute interstitial pneumonitis, also known as Hamman Rich syndrome, characterised by acute respiratory failure, bilateral lung infiltrates on radiographs and diffuse alveolar damage (DAD) on lung histopathology, is an exclusion diagnosis which requires a complete study of other possible etiologies of DAD. It is a pathology with a mortality greater than 50% due to not having a specific therapy beyond symptomatic management and ventilatory support. We present a case of a 56 year old male with respiratory symptoms, who has progressive worsening oxygen levels and bilateral ground glass opacities on chest images. Lung biopsy with diffuse alveolar damage, infection, autoimmune diseases, and drug adverse reaction were ruled out. Managed with mechanical ventilation and high dose systemic corticoids with complete recovery. MÉD.UIS.2021;34(3): 103-8.

The value of imaging features in the diagnosis and treatmen of acute interstitial pneumonia / 实用放射学杂志

Objective To investigate the imaging features of acute interstitial pneumonia and to provide the reference for early diagnosis and treatment.Methods The imaging and clinical data of 50 patients with clinically diagnosed acute interstitial pneumonia in our hospital were analyzed retrospectively.Results X-ray film showed the fan-shaped and lightly patchy shadows around the lung hilum distributing along the lung markings in multiple lung fields,accompanied by thickening of interlobular septa with varying degrees and thickened bronchial vascular shadows running through the above-mentioned light patchy shadows.CT revealed the fan-shaped ground glass opacity in the pulmonary area with rich blood supply,interspersed with thickened clustered pulmonary arteries and veins.Conclusion The imaging features of acute interstitial pneumonia have certain characteristics.The suggestive diagnosis based on imaging plays an important guiding role in early clinical diagnosis and treatment.

Nationwide surveillance of acute interstitial pneumonia in Korea / 소아과

PURPOSE: Acute interstitial pneumonia (AIP) is a rare disease, but its prognosis is fatal because of lack of efficient treatment modality. Recently, it has been reported that there was epidemic AIP in Korea. This study aims to investigate the past and current status of AIP in Korea. METHODS: We performed a nationwide survey and a prospective study. From August 6 to 15, 2008, a questionnaire survey was conducted to identify the prevalence, local distribution, and response to current treatments. The questionnaire was answered by pediatrician working in 23 referral centers in Korea. In addition, 5 referral centers in Seoul performed a preliminary prospective observational study by obtaining clinical data and specimens from appropriate patients. The Korea Centers for Disease Control and Prevention analyzed the samples for possible pathogens. RESULTS: The survey showed 78 AIP cases had occurred and 36 patients had died. Lung biopsy was performed only on 20 patients. In 2008, 9 AIP cases developed. In a prospective study, 9 (M:F=5:4) patients developed AIP in spring and 7 (78%) died, with the mean rate of death occurring 46 days after diagnosis. Human corona virus 229 E, cytomegalovirus, influenza A virus, influenza B virus, and parainfluenza virus were isolated from the respiratory specimens. CONCLUSION: This study showed nationwide prevalence of AIP in Korea. In addition, because of the high mortality rate and rapid progress, pediatricians need to be aware of the disease. Further studies and a nationwide network are required for reducing the morbidity and mortality rates related to AIP.

Epidemic acute interstitial pneumonia in children occurred during the early 2006s / 소아과

PURPOSE: This study was aimed to analyze the clinical characteristics of patients with acute interstitial pneumonia who had presented similar clinical patterns from March to June, 2006 and to describe our experience of treatment and to identify risk factors associated with prognosis. METHODS: The clinical characteristics, radiologic and histopathologic findings and response to steroids of 15 patients (non-survival group [n=7] and survival group [control, n=8]) with acute interstitial pneumonia were investigated through the review of medical records. RESULTS: The mean age of the patients was 26 (range: 3-48) months. Cough, cyanosis and fever were frequent symptoms. The most frequent radiologic findings on admission were pneumomediastium and extensive ground glass opacity. Surgical lung biopsy was performed on 8/15 (53.3%) patients and diffuse alveolar damage was found. Mechanical ventilation was applied for 9/15 (60.0%) patients for 40 (range: 1-99) days. Five patients in survival group received steroid treatment and 7 patients in non-survivial group (P=0.20). One patient in survival group received steroid pulse treatment and 4 patients in non-survival group (P=0.12). Seven patients died all of respiratory failure. The survival rate was 53.4%. CONCLUSION: The patients with acute interstitial pneumonia which occurred on spring 2006 showed high mortality because of rapidly and extensively progressing pulmonary fibrosis and air leakage. Therefore, we should consider surgical lung biopsy and steroid application earlier. We should recognize this acute interstitial pneumonia occurring on spring in domestics and need to investigate the cause and treatment in large scale.

Pneumonia intersticial aguda / Acute Interstitial Pneumonia

A pneumonia intersticial aguda é entidade clínicopatológica incomum, caracterizada pela presença de dano alveolar difuso, e clinicamente por insuficiência respiratória que desenvolve de forma aguda, cursando em dias ou semanas e sem origem clara, tendo, infelizmente, na maioria das vezes, a fatalidade. Neste artigo de revisão, dissertamos sobre etiologia, características clínicas, patologia, diagnóstico e tratamento.

The acute interstitial pneumonia is a rare pathological clinic entity characterized by the presence of diffuse alveolar damage, and clinically by a respiratory deficiency which evolves to the acute form within days or weeks with no clear origin, and most of times, unfortunately evolving to death. In this review, we discuss its etiology, clinical features, pathology, diagnosis, and treatment.

Two Cases of Acute Interstitial Pneumonia During the Second Trimester of Pregnancy / 대한주산의학회잡지

We present two cases of acute interstitial pneumonia (AIP) during the second trimester of pregnancy managed by mechanical ventilation, high dose corticosteroids. We described clinical course, laboratory data and imaging studies. Case 1) A 29-year-old woman, G1P1, was referred for aggravated continuous coughing, sputum and dyspnea, pleuritic chest pain and mild fever for 3 weeks at 24(+3) weeks of gestation. There were coarse breathing sounds with crackles on the both lung field. Her chest X-ray showed diffuse haziness in both lungs with suspicious nodular opacities and suggested pneumonia. HRCT showed diffuse ground glass opacities with interstitial thickening and suspicious fine nodular infiltration in both lungs suggesting acute interstitial pneumonia combined with ARDS. Because her symptoms were more aggravated, she was performed mechanical ventilation treatment. After that, she was performed cesarean hysterotomy and delivered a dead male 850grams. After her symptoms were much more improved. All antibiotics were stopped and reduced the doses of steroids, she was discharged with t-tube capped state. Case 2) A 33-year-old woman, G1P1, was referred for continuous coughing, sputum and dyspnea for 3 months and low abdominal discomfort at 24(+4) weeks of gestation. There were coarse breathing sounds with rales on the both lung field. Her chest X-ray showed diffuse haziness in both lungs with suspicious nodular opacities and suggested interstitial pneumonia. HRCT showed diffuse ground glass opacities with some intralobular and interlobular interstitial thickening and suspicious fine nodular infiltration in both lungs suggesting acute interstitial pneumonia or miliary TBc combined with ARDS. She was treated with antibiotics, oxygen, high dose corticosteroids, and tocolytics. There was no evidence of TBc in the bronchoscopy. She showed decreased mentality and decreased O2 saturation below 90% in spite of mechanical ventilation, high dose steroids and IVGV therapy. She delivered a boy of 870 g (Apgar score 1/5). After delivery, she was expired due to combined aggravating DIC. Her baby was expired at the next day, too. The outcome of AIP is fatal, reporting 59~100% mortality rate. The clinical course, laboratory data and treatment are not well established due to rarity of this disease entity. Chest X-ray and HRCT may be helpful in diagnosis and high dose steroid and immunosuppressive agents usually used but the efficacy is not guaranteed.

Efficacy of Early Steroid Therapy in Acute Interstitial Pneumonia / 결핵

BACKGROUND: Steroid therapy has been shown to improve the clinical outcome in acute respiratory distress syndrome (ARDS) patients wit histological evidence of fibroproliferation in the lung tissue and no identifiab le soure of infection. Because the histopathological features of acute interstitial pneumonia (AIP) are identical with that of ARDS, early steroid therapy was used in AIP patients who had histological evidence of fibroproliferation in the lung tissue and no identifiable source of infection. We analyzed seven years of our experience to evaluate the efficacy of early steroid therapy in AIP. METHODS: A retrospective review was performed on AIP patients who received steroid therapy within 7 days of mechanical ventilatory support in Dankook University Hospital between May 1995 and May 2002. AIP was diagnosed clinically by ARDS without a known cause of the etiology and pathologically by a lung biopsy showing a fibroproliferative stage of diffuse alveolar damage. The clinical response and physiologic parameters were evaluated during steroid therapy. RESULTS: Five AIP patients received intravenous methylprednisolone (1-2mg/kg every 6 hours) after 0.6+/-1.7 days of mechanial ventilatory support. Lung biopsies were performed after 1.8+/-0.4 days of mechanical ventilatory support. Four patients(80%) survived and were extubated after 2.8+/-0.4 days of steroid therapy with improvement in the PaO2 ration (127.4+/-10.1 at day 0 to 223.8+/-37.6 at day 7) by steroid therappy. However, one patient(20%) died of respiratory failure after 15 days of steroid therapy. CONCLUSION: Early steroid therapy sppears to be beneficial in AIP patients without evidence of infection. However, as our study group was too small, futher large scale studies to define the effectiveness of steroids are required.

A Case of Acute Interstitial Pneumonia with Invasive Pulmonary Aspergillosis / 결핵

Acute interstitial pneumonia (AIP) is a rare fulminant form of lung injury that presents acutely; usually in a previously healthy individual. It corresponds to a subset of cases of idiopathic adult respiratory distress sy ndrome (ARDS). Invasive pulmonary aspergillosis is a disease occuring predominantly with defects in immunity such as hematologic malignancy, influenza infection, postchemotherapy, long-term corticosteroid treatment. Invasive aspergillosis has worse prognosis and most cases are diagnosed at postmortem autopsies. We experienced a case of acute interstitial pneumonia with an invasive aspergillosis during corticosteroid treatment. Acute interstitial pneumonia with invasive aspergillosis was diagnosed by an open lung biopsy using thoracoscopy, showing fungal hyphae with sepsis and an acute angle branching invasion of the lung tissue and blood vessels. The patient was treated with IV amphotericin-B, but died due to septic shock.

A Case of Nonspecific Interstitial Pneumonia with Clinical Course of Rapid Aggravation / 결핵

Nonspecific interstitial pneumonia (NSIP) was first described as a new category of idiopathic interstitial pneumonia in 1994. This is a disease with a more insidious onset and has a chronic course. The histological findings are unusual for other idiopathic interstitial pneumonia cases (usual interstitial pneumonia, diffuse interstitial pneumonia, and acute interstitial pneumonia). In contrast to NSIP, acute interstitial pneumonia (AIP) has an acute onset and a fulminant course with the rapid development of respiratory failure. A pathological examination demonstrated characteristic diffuse interstitial fibrosis, hyaline membranes, thrombi, and architectural derangement. Here we report a 48-year-old woman who was diagnosed pathologically NSIP, but with a rapid progressive course similar to AIP.

Clinicopathologic features of Acute Interstitial Pneumonia / 결핵

BACKGROUND: Acute interstitial pneumonia is a relatively rare form of interstitial pneumonia, since the vast majority of interstitial pneumonia have a more chronic course. It corresponds to the lesion described by Hamman and Rich, as Hamman-Rich disease in 1944. Another name in the clinical literature is accelerated interstitial pneumonia, idiopathic acute respiratory distress syndrome (idiopathic ARDS), and the organizing stage of diffuse alveolar damage. Acute interstitial pneumonia differs from chronic interstitial pneumonia by clinical and pathologic features. Clinically, this disease is characterized by a sudden onset and a rapid course, and reversible disease. METHOD AND PURPOSE: Five cases of pathologically proven acute interstitial pneumonia were retrospectively studied to define the clinical, radiologic, and pathologic features. RESULTS: 1) The five cases ranged in age from 31 to 77 years old. The onset of illness was acute in all patients, it began with viral-like prodrome 6~40 days prior to shortness of breath, and respiratory failure eventually developed in all patients. In 2 cases, generalized skin rash was accompanied with flu-like symptoms. Etiologic agent could not be identified in any case. 2) All patients had leukocytosis and severe hypoxemia. Pulmonary function test of 3 available cases shows restrictive ventilatory defect, and one survived patient(case 5) has a complete improvement of pulmonary function after dismissal. 3) Diffuse bilateral chest infiltrates were present radiologically. Theses were the ground-glass, consolidation, and reticular densities without honeycomb fibrosis in all patients. The pathologic abnormalities were the presence of increased numbers of macrophages and the formation of hyaline membranes within alveolar spaces. There was also interstitial thickening with edema, proliferation of immature fibroblast, and hyperplasia of type II pneumocyte. In the survived patient(case5), pathologic findings were relatively early stage of acute interstitial pneumonia, such as hyaline membrane with mild interstitial fibrosis. 4) Of the 5 patients, four patients died of respiratory failure 14~90 days after onset of first symptom, and one survived and recovered in symptoms, chest X ray, and pulmonary function test CONCLUSION: These results emphasize that acute interstitial pneumonia is clinically, radiologically, and pathologically distinct form of interstitial pneumonia and should be separated from the group of chronic interstitial pneumonia. Further studies will be needed to evaluate the pathogenesis and the treatment of acute interstitial pneumonia.