Interstitial Lung disease In Childhood Rheumatic Disorders
Lourdes R. Laraya-Cuasay, Walter T. Hughes in Interstitial Lung Diseases in Children, 2019
Acute pneumonitis is characterized by the sudden appearance of pulmonary infiltrates and pleural effusions, and occurs in the setting of active lupus. It is often accompanied by fever, cough, chest pain, and tachypnea. Chronic interstitial lung disease, however, may occur with or without symptoms. The most common symptom is dyspnea on exertion. Physical findings associated with chronic interstitial disease may include poor diaphragmatic movement and basilar rales. Cyanosis and clubbing may occur. Radiographic findings include diffuse or localized infiltrates, plate-like atelectasis, unilateral elevation of the diaphragm, and small pleural effusions. When biopsied, early lesions may show inflammatory changes. Later lesions show thickening of alveolar walls with fibrous tissue, plasma cell infiltration of interstices, histiocyte desquamation, and necrosis of alveoli and bronchioles.3,6
What is asbestosis and what is not: Radiology and pathology correlates
Dorsett D. Smith in The Health Effects of Asbestos, 2015
The clinical/radiological diagnosis of interstitial lung disease is still imperfect. The sensitivity and positive predictive value for a HRCT diagnosis of idiopathic fibrosing alveolitis (IPF)/chronic fibrosing alveolitis (CFA) were 71% each, while specificity and negative predictive value were 67% each. For the ATS/ERS criteria, sensitivity, specificity, positive predictive value, and negative predictive value were 71%, 75%, 77%, and 69%, respectively. The inter-observer variability values, expressed as a kappa coefficient, for HRCT and the ATS/ERS criteria were 0.59 and 0.53, respectively. Both HRCT and the ATS/ERS clinical criteria may lead to misdiagnosis of patients with interstitial lung disease. (Peckham RM, Shorr AF, Helman DL Jr. Potential limitations of clinical criteria for the diagnosis of idiopathic pulmonary fibrosis/cryptogenic fibrosing alveolitis. Respiration 2004;71(2):165–9.)
Breathlessness in Pregnancy: Respiratory Causes
Tony Hollingworth in Differential Diagnosis in Obstetrics and Gynaecology: An A-Z, 2015
Usual interstitial pneumonia (UIP) and non-specific interstitial pneumonia (NSIP) (formerly known as ‘fibrosing alveolitis’) usually occur later in life, but may be associated with autoimmune diseases, which occur more frequently in young women, such as rheumatoid disease, scleroderma, and systemic lupus erythematosus, and should, therefore, be considered in the differential diagnosis of breathlessness in pregnancy. Other rarer forms of interstitial lung disease include acute interstitial pneumonia and respiratory bronchiolitis interstitial lung disease. Progressive breathlessness and cough are typical, with bilateral, fine, mid-late inspiratory crackles on auscultation. Finger clubbing may be present, but is often absent in earlier and milder disease. Chest X-ray usually shows peripheral bibasal interstitial shadowing, but HRCT scanning is necessary to define the type of disease and likely response to treatment. Lung function testing, as with the other interstitial lung diseases, reveals a reduced transfer factor (diffusion capacity).
Interstitial lung abnormalities – current knowledge and future directions
Published in European Clinical Respiratory Journal, 2021
Gisli Thor Axelsson, Gunnar Gudmundsson
Interstitial lung abnormalities have many parallels with advanced forms of interstitial lung disease. They are associated with some of the cardinal epidemiologic, environmental, and genetic risk factors of ILD as well as some proposed biomarkers and histopathological features of ILD [5,25,31,34,37,39]. Associations have been found to differ among different imaging patterns, with stronger genetic associations and worse outcomes observed for imaging abnormalities that are extensive enough to meet criteria for ILD diagnosis among symptomatic people [5]. ILA progression has been observed and the odds of progression have been found to be increased with genetic risk factors of ILD and imaging features suggestive of more advanced fibrosis [50,74]. Aside from their relationship with ILD, ILA seem to give rise to adverse clinical outcomes, most notably lung malignancies and increased mortality [8,10,65]. So, the proposition that some or all of the changes that the ILA term encompasses represent possible precursors, or early stages, of ILD [91] are strengthened by the body of research reviewed here. In addition, the outcomes associated with ILA suggest that they can by themselves be a relevant clinical finding.
The pre-clinical discovery and development of osimertinib used to treat non-small cell lung cancer
Published in Expert Opinion on Drug Discovery, 2021
Florian Wittlinger, Stefan A. Laufer
Most common adverse events (> 20%) which were observed in clinical trials under treatment with osimertinib were diarrhea, rash, musculoskeletal pain, paronychia, dry skin, stomatitis, fatigue, and cough[94]. Occurrence of Interstitial lung disease (ILD)-like events was reported in 3.7% of patients, and permanent discontinuation of therapy is suggested if symptoms appear. ILD is a collective term for lung diseases affecting the parenchyma, which are triggered by an initial inflammation of the alveoli that expand to the interstitium and usually result in pulmonary fibrosis[95]. QTc interval prolongation was observed, fortunately without any fatal cases. Yet, in patients with a preexisting heart condition, strict monitoring with ECG should be performed. Cardiomyopathies appeared in 3% of patients, and cardiac monitoring should be conducted in patients with cardiac risk factors. Keratitis was reported in 0.7% of patients in clinical trials. An ophthalmologist should be consulted in the presence of typical symptoms. Severe skin reactions such as Stevens-Johnson syndrome and erythema multiforme major have been reported under treatment with osimertinib, which should be discontinued at signs of early symptoms. Osimertinib should be withheld in the case of symptoms of a cutaneous vasculitis, and therapy should be discontinued if coherence between systemic treatment and disease is proven after consultation of a dermatologist[94].
Pyoderma gangrenosum with pulmonary involvement: a pulmonary special report and literature review
Published in Expert Review of Respiratory Medicine, 2022
Fanfan Xing, Kelvin Hei-Yeung Chiu, Jin Yang, Haiyan Ye, Lijun Zhang, Chenjing Liu, Kwok-Yung Yuen
Concerning further investigation among the 51 patients included in this review, 46 patients (90.2%) received a CT of the thorax for further delineation of underlying pulmonary pathology. The most common radiological findings were pulmonary infiltrates (84.8%, 39/46), followed by cavitary lesions (39.1%, 18/46), pleural effusion (13.0%, 6/46), and consolidation (10.9%, 5/46). Other radiological findings included interstitial lung disease and interstitial pneumonia. Only 27 patients (52.9%, 27/51) received bronchoscopy or open/radiological guided lung biopsy for further confirmation of the diagnosis as well as exclusion of other similar etiologies. Neutrophil infiltration or inflammation (85.2%, 23/27) was a common finding in the histopathology of the respiratory specimen, with 2 patients (11.1%, 3/27) showing pulmonary fibrosis and 5 patients (22.2%, 6/27) showing granulomatous inflammation, and with negative bacterial and fungal staining excluding the possibility of opportunistic infection in these patients.
Related Knowledge Centers
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- Endothelium
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