scholarly journals CHILDHOOD-ONSET INTERSTITIAL LUNG DISEASE: A RARE CASE OF IDIOPATHIC, NONSPECIFIC INTERSTITIAL PNEUMONIA IN A TEENAGER

CHEST Journal ◽  
2020 ◽  
Vol 158 (4) ◽  
pp. A1086-A1087
Author(s):  
Giuliana Cerro ◽  
Tim Shen ◽  
Jack Green ◽  
Gary Goulin ◽  
Michael Lewis
2013 ◽  
Vol 66 (suppl. 1) ◽  
pp. 29-33
Author(s):  
Ruza Stevic ◽  
Vucinic Mihailovic ◽  
Dragana Jovanovic ◽  
Nada Vasic

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.


2013 ◽  
Vol 2013 ◽  
pp. 1-13 ◽  
Author(s):  
Lorenzo Cavagna ◽  
Sara Monti ◽  
Vittorio Grosso ◽  
Nicola Boffini ◽  
Eva Scorletti ◽  
...  

Interstitial lung disease (ILD) is a relevant extra-articular manifestation of rheumatoid arthritis (RA) that may occur either in early stages or as a complication of long-standing disease. RA related ILD (RA-ILD) significantly influences thequoad vitamprognosis of these patients. Several histopathological patterns of RA-ILD have been described: usual interstitial pneumonia (UIP) is the most frequent one, followed by nonspecific interstitial pneumonia (NSIP); other patterns are less commonly observed. Several factors have been associated with an increased risk of developing RA-ILD. The genetic background plays a fundamental but not sufficient role; smoking is an independent predictor of ILD, and a correlation with the presence of rheumatoid factor and anti-cyclic citrullinated peptide antibodies has also been reported. Moreover, bothexnovooccurrence and progression of ILD have been related to drug therapies that are commonly prescribed in RA, such as methotrexate, leflunomide, anti-TNF alpha agents, and rituximab. A greater understanding of the disease process is necessary in order to improve the therapeutic approach to ILD and RA itself and to reduce the burden of this severe extra-articular manifestation.


2021 ◽  
pp. 239719832110233
Author(s):  
Bhavya Chintala ◽  
Lalit Duggal ◽  
Neeraj Jain

Ascending aorta aneurysm in scleroderma can be ascribed to its macrovascular involvement which is very less elucidated. We here describe a 56-year-old female with rapidly progressive diffuse cutaneous scleroderma. She presented with skin thickening involving all four limbs, thorax and abdomen. She had other features like arthritis, Raynaud’s phenomena, dyspnoea, heaviness of chest, and dysphagia. On investigation, she was strongly positive for antinuclear antibody and Scl 70. Imaging revealed interstitial lung disease (nonspecific interstitial pneumonia pattern) and a fusiform dilatation of ascending aorta of 6.5 cm. Patient was offered surgical correction for aneurysm, for which she refused. To the best of our knowledge, our case report adds up to the few cases of ascending aorta aneurysm in scleroderma available in world literature.


2018 ◽  
Vol 5 (4) ◽  
pp. 16
Author(s):  
Jack Xu ◽  
Steven Verga ◽  
Jonathan Stoll ◽  
Lauren Pioppo ◽  
Eileen Shanahan ◽  
...  

Introduction: Statins have been shown to effectively prevent both cardiovascular morbidity and mortality by inhibiting the hydroxymethylglutaryl coenzyme A (HMG-CoA) reductase enzyme. Pulmonary complications are very rare, and can include pneumonitis, pleural effusion, and nonspecific interstitial pneumonia (NSIP). There have been very few previously documented cases of statin-induced fibrotic NSIP.Case report: We present a female with a history of hyperlipidemia on atorvastatin who presented with shortness of breath. Computed tomography scan of the chest revealed interstitial infiltrates with bilateral ground-glass opacities. She underwent a surgical lung biopsy which showed uniform fibrous alveolar septal thickening, scattered collections of alveolar macrophages and inflammation, along with areas of fibrosis. The findings were most suggestive of fibrotic NSIP. Atorvastatin was stopped and she was started on mycophenolic acid with improvement of her symptoms.Discussion: Although rare, the clinician should be aware of possible pulmonary complications of statin therapy. The exact mechanism of injury is unclear, however immunological or toxicological mechanisms are implicated. One case series of statin induced interstitial lung disease showed some improvement of dyspnea with systemic glucocorticoids and termination of statin therapy. Statins, specifically pravastatin, lovastatin, and simvastatin have been associated with drug induced pneumonitis and interstitial lung disease.


Author(s):  
Giorgia Borio ◽  
Chiara Terracciano ◽  
Federico Buttafava ◽  
Andrea Vercelli ◽  
Laura Pagani ◽  
...  

We report the case of a 62-year-old male patient fully vaccinated for COVID-19, admitted to our emergency room for persistent fever associated with exertional dyspnoea, skin lesions, diffuse myalgias and arthralgias not responsive to broad-spectrum antibiotic and antiviral therapy, who developed a rapidly progressive refractory to treatment interstitial lung disease due to anti-melanoma differentiation-associated gene 5 (MDA5) antibodies, that required mechanical ventilation and ECMO. Here, we highlight the importance of always considering alternative diagnoses, i.e. viral and autoimmune diseases, including anti-MDA5 antibody screening, when dealing with patients with a skin rash, seronegative polyarthralgias and interstitial pneumonia, or acute respiratory distress syndrome of unknown origin.


2018 ◽  
Vol 314 (6) ◽  
pp. L998-L1009 ◽  
Author(s):  
Elizabeth F. Redente ◽  
Martin A. Aguilar ◽  
Bart P. Black ◽  
Benjamin L. Edelman ◽  
Ali N. Bahadur ◽  
...  

Rheumatoid arthritis (RA)-associated interstitial lung disease (RA-ILD) develops in ~20% of patients with RA. SKG mice, which are genetically prone to development of autoimmune arthritis, develop a pulmonary interstitial pneumonia that resembles human cellular and fibrotic nonspecific interstitial pneumonia. Nintedanib, a tyrosine kinase inhibitor approved for treatment of idiopathic pulmonary fibrosis, has been shown to reduce the decline in lung function. Therefore, we investigated the effect of nintedanib on development of pulmonary fibrosis and joint disease in female SKG mice with arthritis induced by intraperitoneal injection of zymosan (5 mg). Nintedanib (60 mg·kg−1·day−1 via oral gavage) was started 5 or 10 wk after injection of zymosan. Arthritis and lung fibrosis outcome measures were assessed after 6 wk of treatment with nintedanib. A significant reduction in lung collagen levels, determined by measuring hydroxyproline levels and staining for collagen, was observed after 6 wk in nintedanib-treated mice with established arthritis and lung disease. Early intervention with nintedanib significantly reduced development of arthritis based on joint assessment and high-resolution μ-CT. This study impacts the RA and ILD fields by facilitating identification of a therapeutic treatment that may improve both diseases. As this model replicates the characteristics of RA-ILD, the results may be translatable to the human disease.


2021 ◽  
Vol 5 (1) ◽  
pp. 035-041
Author(s):  
Zhou Zexuan ◽  
Zhang Guangfeng ◽  
Xu Ting ◽  
Lin Haobo ◽  
Zhang Xiao

Background: The aim of the study was to evaluate radiographic features of systemic sclerosis-associated interstitial lung disease. Patients and methods: 116 patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD) from 2010 to 2019 comprised our retrospective study. All patients were subject to high resolution computed tomography (HRCT). ILD patterns were classified into 7 patterns as IIPs and analyzed with pathology. We chose two staging method and two semi-quantitative score methods to evaluate the HRCT performance and analyzed with pulmonary function tests. Results: Ground-glass opacities were the most common presentation on HRCT, followed by interlobular septal thickening, reticular opacities, intralobular interstitial thickening; honeycombing, traction bronchiectasis and nodules can also be observed. The most common pattern of SSc-ILD was nonspecific interstitial pneumonia (NSIP), secondly was UIP. There was no difference in ILD pattern between HRCT and pathology, and revealed a high congruence. The four HRCT evaluating methods presented in this study all had significant relationships with PETs. Conclusion: The most common pattern of SSc-ILD was nonspecific interstitial pneumonia (NSIP). The ILD patterns of HRCT coincide very well with histology, and will replace pathology as the gold standard for diagnosis and evaluation of SSc-ILD.


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