scholarly journals Acute interstitial pneumonia (hamman-rich syndrome) - a life threatening respiratory disease

2018 ◽  
Vol 28 (2) ◽  
pp. 91-94
Author(s):  
Md Mahmudur Rahman Siddiqui ◽  
Md Mostafizur Rahman ◽  
Md Abdul Moyeed Siddiqui ◽  
Hasan Ali Masum ◽  
Rubayat Shekh Giasuddin
Keyword(s):  
Respiratory Failure ◽  
Lung Disease ◽  
Respiratory Disease ◽  
Interstitial Pneumonia ◽  
High Mortality ◽  
Poor Prognosis ◽  
Progressive Dyspnoea ◽  
Acute Interstitial Pneumonia ◽  
Medical Facilities ◽  
Life Threatening

Acute interstitial pneumonia (AIP) is an idiopathic lung disease characterized by rapidly progressive dyspnoea, respiratory failure developing over days to weeks in patients without pre-existing lung disease. AIP show a very poor prognosis, with high mortality and remains unfamiliar to physicians. In this report, we present a case of a mid-age male with AIP who, was treated with intensive medical facilities, still showed a rapid progressive clinical deterioration, and eventually died of respiratory failure.Bangladesh J Medicine Jul 2017; 28(2) : 91-94

scholarly journals Chronic respiratory disease and survival outcomes after extracorporeal membrane oxygenation

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Tak Kyu Oh ◽  
Hyoung-Won Cho ◽  
Hun-Taek Lee ◽  
In-Ae Song
Keyword(s):  
Respiratory Failure ◽  
Extracorporeal Membrane Oxygenation ◽  
Lung Disease ◽  
Respiratory Disease ◽  
Chronic Respiratory Disease ◽  
Newly Diagnosed ◽  
Membrane Oxygenation ◽  
Obstructive Sleep ◽  
All Cause Mortality ◽  
Ecmo Therapy

Abstract Background Quality of life following extracorporeal membrane oxygenation (ECMO) therapy is an important health issue. We aimed to describe the characteristics of patients who developed chronic respiratory disease (CRD) following ECMO therapy, and investigate the association between newly diagnosed post-ECMO CRDs and 5-year all-cause mortality among ECMO survivors. Methods We analyzed data from the National Health Insurance Service in South Korea. All adult patients who underwent ECMO therapy in the intensive care unit between 2006 and 2014 were included. ECMO survivors were defined as those who survived for 365 days after ECMO therapy. Chronic obstructive pulmonary disease (COPD), asthma, interstitial lung disease, lung cancer, lung disease due to external agents, obstructive sleep apnea, and lung tuberculosis were considered as CRDs. Results A total of 3055 ECMO survivors were included, and 345 (11.3%) were newly diagnosed with CRDs 365 days after ECMO therapy. The prevalence of asthma was the highest at 6.1% (185). In the multivariate logistic regression, ECMO survivors who underwent ECMO therapy for acute respiratory distress syndrome (ARDS) or respiratory failure had a 2.00-fold increase in post-ECMO CRD (95% confidence interval [CI]: 1.39 to 2.89; P < 0.001). In the multivariate Cox regression, newly diagnosed post-ECMO CRD was associated with a 1.47-fold (95% CI: 1.17 to 1.86; P = 0.001) higher 5-year all-cause mortality. Conclusions At 12 months after ECMO therapy, 11.3% of ECMO survivors were newly diagnosed with CRDs. Patients who underwent ECMO therapy for ARDS or respiratory failure were associated with a higher incidence of newly diagnosed post-ECMO CRD compared to those who underwent ECMO for other causes. Additionally, post-ECMO CRDs were associated with a higher 5-year all-cause mortality. Our results suggest that ECMO survivors with newly diagnosed post-ECMO CRD might be a high-risk group requiring dedicated interventions.

A Rare Case of Acute Interstitial Pneumonia Diagnosed at Autopsy

2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S153-S154
Author(s):  
E Conner ◽  
D Troxclair ◽  
H Khokhar ◽  
W Beversdorf
Keyword(s):  
Respiratory Failure ◽  
Interstitial Pneumonia ◽  
Diffuse Alveolar Damage ◽  
Ventilatory Support ◽  
Respiratory Illness ◽  
High Dose ◽  
Atypical Pneumonia ◽  
Acute Interstitial Pneumonia ◽  
Diagnostic Role ◽  
History Of

Abstract Introduction/Objective Acute interstitial pneumonia (AIP) is a rare disease clinically characterized by rapidly progressing respiratory failure in individuals with no history of respiratory illness or other inciting factors. While most often diagnosed in middle-aged adults, it may present in any age group. Initial presentation is described as influenza- like, and respiratory failure requiring ventilatory support often progresses within weeks to months. Prognosis is poor, with an estimated mortality rate approaching 80% without treatment. Methods We present the case of a 44-year-old male nonsmoker with no significant medical history, who presented in 2018 with 1.5 months of dyspnea and headache initially diagnosed as atypical pneumonia. Chest imaging revealed bilateral opacities; however, microbial workup revealed no evidence of infectious etiology. Autoimmune serology studies were likewise unrevealing. Despite aggressive supportive and medical management, he deteriorated to respiratory failure and succumbed. Results At autopsy, the lungs were symmetrically congested and edematous (combined weight 2,340 g) but free of evident consolidation or discrete lesions. Microscopic examination revealed diffuse alveolar damage with extensive hyaline membrane formation, interstitial edema, and fibroblastic proliferation. The vasculature was severely congested, and the alveoli contained hemorrhage and scattered macrophages. No fungal or mycobacterial elements were identified by staining. Based on the histologic features and clinical context, the diagnosis of AIP was made. Conclusion AIP is a rare, aggressive, and diagnostically challenging disease that includes a broad range of both clinical and histologic differentials. Timely recognition and intervention with aggressive respiratory support and high- dose glucocorticoids are the mainstays of clinical management. The diagnostic role of histology is significant, but hinges on early clinical consideration of AIP as disease progression may later preclude the biopsy procedure. We share this case to raise awareness of this rapidly progressive and diagnostically troubling interstitial lung disease while emphasizing the importance of clinicopathologic correlation.

Extracorporeal Membrane Oxygenation for Refractory Severe Respiratory Failure in Acute Interstitial Pneumonia

2018 ◽  
Vol 42 (5) ◽  
pp. 569-574 ◽  
Author(s):  
Gabriela Gonçalves-Venade ◽  
Nuno Lacerda-Príncipe ◽  
Roberto Roncon-Albuquerque ◽  
José Artur Paiva
Keyword(s):  
Respiratory Failure ◽  
Extracorporeal Membrane Oxygenation ◽  
Interstitial Pneumonia ◽  
Severe Respiratory Failure ◽  
Membrane Oxygenation ◽  
Acute Interstitial Pneumonia

scholarly journals Radiological diagnosis of interstitial lung diseases

2013 ◽  
Vol 66 (suppl. 1) ◽  
pp. 29-33
Author(s):  
Ruza Stevic ◽  
Vucinic Mihailovic ◽  
Dragana Jovanovic ◽  
Nada Vasic
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Lung Diseases ◽  
Usual Interstitial Pneumonia ◽  
Interstitial Lung Diseases ◽  
Ground Glass ◽  
Cryptogenic Organizing Pneumonia ◽  
Acute Interstitial Pneumonia ◽  
Nonspecific Interstitial Pneumonia

Introduction. Interstitial lung diseases include the entities of idiopathic pulmonary fibrosis, nonspecific interstitial pneumonia, cryptogenic organizing pneumonia, acute interstitial pneumonia, respiratory bronchiolitis-associated interstitial lung disease, desquamative interstitial pneumonia, and lymphocytic interstitial pneumonia. Recent introduction of high-resolution computed tomography has made the diagnosis of interstitial lung disease much easier. Usual interstitial pneumonia A predominantly subpleural reticulation and honeycombing at the basal posterior part of the lung with a progression to anterior and superior parts are characteristic of usual interstitial pneumonia/ idiopathic pulmonary fibrosis. Nonspecific interstitial pneumonia Typical findings of nonspecific interstitial pneumonia are bilateral, relatively symmetrical subpleural ground glass opacifications and irregular linear opacities. Desquamative interstitial pneumonia is characterized by diffuse symmetrical ground glass opacifications. Respiratory bronchiolitisassociated interstitial lung disease Centrilobular nodules and irregular ground glass opacifications are present. Cryptogenic organizing pneumonia Subpleural and peribronchial consolidations are prominent findings that are not present in other idiopathic interstitial pneumonias. Acute interstitial pneumonia. Bilateral ground-glass opacifications are present and areas of peripheral consolidations may also be seen in acute interstitial pneumonia. Lymphocytic interstitial pneumonia. Diffuse or patchy areas of ground glass opacification with centrilobular nodules and occasionally well-defined cysts are seen. Conclusion. Imaging plays a crucial role in identifying interstitial lung diseases but precise diagnosis requires a dynamic interdisciplinary approach that correlates clinical, radiological and pathologic features.

scholarly journals Acute Interstitial Pneumonia (Hamman-Rich Syndrome) as a Cause of Idiopathic Acute Respiratory Distress Syndrome

2011 ◽  
Vol 2011 ◽  
pp. 1-4 ◽  
Author(s):  
Jackrapong Bruminhent ◽  
Shahla Yassir ◽  
James Pippim
Keyword(s):  
Acute Respiratory Distress Syndrome ◽  
Respiratory Distress Syndrome ◽  
Respiratory Distress ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Distress Syndrome ◽  
Glucocorticoid Therapy ◽  
Unknown Etiology ◽  
Upper Respiratory Tract ◽  
Acute Interstitial Pneumonia

Hamman-Rich syndrome, also known as acute interstitial pneumonia, is a rare and fulminant form of idiopathic interstitial lung disease. It should be considered as a cause of idiopathic acute respiratory distress syndrome. Confirmatory diagnosis requires demonstration of diffuse alveolar damage on lung histopathology. The main treatment is supportive care. It is not clear if glucocorticoid therapy is effective in acute interstitial pneumonia. We report the case of a 77-year-old woman without pre-existing lung disease who initially presented with mild upper respiratory tract infection and then progressed to rapid onset of hypoxic respiratory failure similar to acute respiratory distress syndrome with unknown etiology. Despite glucocorticoid therapy, she did not achieve remission and expired after 35 days of hospitalization. The diagnosis of acute interstitial pneumonia was supported by the histopathologic findings on her lung biopsy.

scholarly journals A Cross-Sectional Study of Radiological Profile of Interstitial Lung Disease Using High Resolution Computed Tomography of Patients Attending Medical College at Ahmedabad

2021 ◽  
Vol 8 (17) ◽  
pp. 1127-1132
Author(s):  
Nikunj C. Desai ◽  
Nilesh P. Parkar ◽  
Asutosh N. Dave
Keyword(s):  
Computed Tomography ◽  
High Resolution ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Usual Interstitial Pneumonia ◽  
Cross Sectional Study ◽  
High Resolution Computed Tomography ◽  
Cross Sectional ◽  
Acute Interstitial Pneumonia

BACKGROUND Interstitial lung disease (ILD) is an unpredictable diffuse parenchymal lung disease, which involves interstitium of lung (tissue around the alveoli of the lungs). High resolution computed tomography (HRCT) is one of the confirmatory, easily accessible methodology for the conclusion and follow up assessment of interstitial lung disease. We wanted to study the normal HRCT patterns found with interstitial lung disease and contrastingly different HRCT designs and clinical information in differential determination of pulmonary fibrosis. We also wanted to study the different patterns of interstitial lung disease on high resolution computed tomography and thereby provide accurate diagnosis and management to the patients. METHODS The study was a hospital based prospective, cross sectional study. In the present study, total fifty patients referred from Department of Medicine and Department of Pulmonary Medicine of GCS Medical College having suspicion of interstitial lung disease were studied from April 2019 to September 2019. All patients underwent HRCT thorax on 16 slice Siemens computerised tomography (CT) scan machine in recumbent position utilising usual HRCT protocol. Lung abnormalities were noticed and classified for explicit diagnosis of interstitial lung pathologies. RESULTS Most of the patients (N = 25) were found to be in the age group of 50 - 80 years (17 female & 8 male). Progressive dyspnoea (N = 47; 94 %) was the most common chief complaint. The most common form of interstitial lung disease was usual interstitial pneumonia (UIN) (N = 18; 36 %) in our study. Acute interstitial pneumonia (AIP) (N = 7; 14 %) and non-specific interstitial pneumonia, NSIP (N = 7; 14 %) were the next common interstitial lung diseases. CONCLUSIONS The most well-known interstitial lung disease seen in our examination was usual interstitial pneumonia. Cases of interstitial lung disease are on the rise. Interstitial lung disease should be ruled out in all patients with progressive dyspnoea, particularly when there are no obvious or known causes of dyspnoea. Clinical and laboratory findings, pulmonary function tests, history of exposure along with HRCT workup is indispensable for the identification or exclusion of interstitial lung disease. HRCT is also useful for the follow-up study. KEYWORDS Acute Interstitial Pneumonia (AIP), High Resolution Computed Tomography (HRCT), Interstitial Lung Disease (ILD), Nonspecific Interstitial Pneumonia (NSIP), Usual Interstitial Pneumonia (UIP)

scholarly journals Respiratory failure in a patient with dermatomyositis

2013 ◽  
Vol 8 ◽  
Author(s):  
Ivano Salimbene ◽  
Ilaria Leli ◽  
Salvatore Valente
Keyword(s):  
Respiratory Failure ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Usual Interstitial Pneumonia ◽  
Ventilatory Support ◽  
Pulmonary Complications ◽  
Gas Analysis ◽  
Lower Limbs ◽  
Arterial Blood

Since its original description in 1956 the association between interstitial lung disease and polymyositis (PM) and dermatomyositis (DM) has become well established. Interstitial lung disease (ILD) can be a significant complication in rheumatic diseases (RDs). Although most patients with RD do not develop clinically evident ILD, these systemic autoimmune disorders are estimated to be responsible for approximately 25% of all ILD deaths and 2% of deaths due to all respiratory causes. Radiologic abnormalities in DM are characterized by a high incidence of airspace consolidation. Non-Specific Interstitial Pneumonia (NSIP) is the most common form of lung disease, with a frequency in biopsies 4-fold greater than that of Usual Interstitial Pneumonia (UIP) in PM and a slightly smaller predominance in DM. We report a case of a female patient, 57 years old, no former smoker, whose clinical history was onset in November 2008 with asthenia with muscle and osteoarticular pain especially located in the upper limbs and then also expanded to the lower limbs. The EMG was compatible with dermatomyositis in the acute phase. The patient received therapy with steroids and tacrolimus, also making several rounds of treatment with immunoglobulin. Given the recurrence of myositis in association with signs of poorly controlled interstitial lung disease, immunosuppressive therapy with Rituximab was administered. The Computed Tomography (CT) scans showed "bronchiectasis and traction bronchiolectasis, hypodense areas consistent with the phenomena of air trapping. The pattern of interstitial lung disease with fibrotic evolution seems consistent with NSIP. The arterial blood gas analysis showed a pattern of hypoxic-hypercapnic respiratory failure (pH: 7,34, PaO2: 67 mmHg; PaCO2: 55 mmHg). As a result of an episode of marked desaturation unresponsive to supplemental oxygen at high flows we proceeded to noninvasive mechanical ventilation with Helmet for 24 hours/24. This ventilatory support was maintained for a week, with resolution of the respiratory failure. In this brief case report we want to highlight various pulmonary complications as a result of dermatomyositis. The progression of respiratory complications may also lead to a situation of respiratory failure, as in our patient, and require a noninvasive ventilatory treatment.

Diffuse Lung Disease

2012 ◽  
Author(s):  
Timothy R. Aksamit
Keyword(s):  
Differential Diagnosis ◽  
Respiratory Failure ◽  
Lung Disease ◽  
Lung Diseases ◽  
Clinical Course ◽  
Diffuse Lung Disease ◽  
Wide Range ◽  
Life Threatening ◽  
Diffuse Lung ◽  
Parenchymal Lung

Diffuse lung disease includes a wide range of parenchymal lung diseases that have infectious, inflammatory, malignant, drug, occupational or environmental, and other causes. Although many identifiable causes are recognized, the cause of most cases of diffuse lung disease in many published series is idiopathic. The clinical course may be acute or prolonged and may progress rapidly to life-threatening respiratory failure with death, or it may be indolent over many years. In most instances, a differential diagnosis can readily be formulated by obtaining the medical history, with emphasis on the nature of the symptoms, duration, and pertinent environmental, occupational, drug, and travel exposures.

scholarly journals Acral Cutaneous Ulcerations and Livedo Reticularis with Rapidly Progressive Interstitial Lung Disease in Anti-MDA5 Antibody-Positive Classical Dermatomyositis

2020 ◽  
Vol 12 (1) ◽  
pp. 57-63
Author(s):  
Rachot Wongjirattikarn ◽  
Suteeraporn Chaowattanapanit ◽  
Charoen Choonhakarn ◽  
Apichart So-ngern ◽  
Ajanee Mahakkanukrauh ◽  
...  
Keyword(s):  
Respiratory Failure ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Muscle Weakness ◽  
Blood Test ◽  
Poor Prognosis ◽  
Livedo Reticularis ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Prognosis Prediction

Rapidly progressive interstitial lung disease (RP-ILD) and its distinctive cutaneous features are highly associated with the presence of anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody in patients with dermatomyositis (DM), leading to a poor prognosis. We describe the case of a 25-year-old man who developed progressive proximal muscle weakness with RP-ILD and had unusual cutaneous findings (cutaneous ulcerations and livedo reticularis) accompanied by classical cutaneous features (heliotrope rash, Gottron’s papules, Gottron’s sign, and flagellate erythema). Blood test was positive for anti-MDA5 antibody. He was treated with intravenous corticosteroids and immunoglobulin, but passed away due to respiratory failure within 1 month after admission. Our case highlights that the presence of cutaneous ulcerations and livedo reticularis, in addition to RP-ILD, are useful clinical clues that may aid in the detection of anti-MDA5 antibody, early initiation of combined immunosuppressants, and prognosis prediction in patients with classical DM.

Sign in / Sign up

Export Citation Format

Share Document