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Chronic Interstitial Lung Disease


Presentation

  • The present report provides important clinically-relevant information on the current approach to diagnosis and management of chronic interstitial lung diseases in children.[ncbi.nlm.nih.gov]
  • Our patient presented with more of a chronic presentation as she had prior exposure to nitrofurantoin for more than 2 years.[link.springer.com]
Cough
  • We describe a case with chronic interstitial lung disease and chronic cough that was attributed to P. jirovecii.[ncbi.nlm.nih.gov]
  • Many patients with interstitial lung disease have their chronic cough mistakenly attributed to that condition when, in fact, the cough is due to more common disorders such as asthma, upper airway cough syndrome (previously referred to as postnasal drip[journals.lww.com]
  • Chronic presentation occurs over weeks to years and consists of a progressive dry cough and progressive dyspnea [ 18 , 19 , 20 ].[link.springer.com]
  • Most people with this condition also have a dry cough. A dry cough means you do not cough up any mucus or sputum. Over time, weight loss, fatigue, and muscle and joint pain are also present.[medlineplus.gov]
  • They are often symptomatic for shortness of breath and a dry cough. The diseases can be difficult to diagnose and vary from patient to patient.[academicdepartments.musc.edu]
Dry Cough
  • Chronic presentation occurs over weeks to years and consists of a progressive dry cough and progressive dyspnea [ 18 , 19 , 20 ].[link.springer.com]
  • CONCLUSION The diagnosis of ILD should be made after no more than 3 weeks during the evaluation of chronic exertional dyspnea and dry cough.[consultant360.com]
  • Most people with this condition also have a dry cough. A dry cough means you do not cough up any mucus or sputum. Over time, weight loss, fatigue, and muscle and joint pain are also present.[medlineplus.gov]
  • Symptoms of interstitial lung disease include: shortness of breath, and a dry cough . Treatment depends on the underlying cause of the inflammation.[emedicinehealth.com]
  • They are often symptomatic for shortness of breath and a dry cough. The diseases can be difficult to diagnose and vary from patient to patient.[academicdepartments.musc.edu]
Dyspnea
  • […] doi: 10.1097/01.mcp.0000174249.07762.37 Interstitial lung disease Abstract Author Information Authors Article Metrics Metrics Although dyspnea is the most common and distressing symptom in patients with chronic interstitial lung disease, chronic cough[journals.lww.com]
  • Chronic presentation occurs over weeks to years and consists of a progressive dry cough and progressive dyspnea [ 18 , 19 , 20 ].[link.springer.com]
  • For example, exertional dyspnea in ILD resembles dyspnea experienced by patients with more common ailments such as asthma, COPD, and heart disease.[consultant360.com]
  • Symptoms and Signs Early: cough, fatigue and dyspnea on exertion, chest exam may be unremarkable or reveal inspiratory crackles at lung bases. Late: dyspnea at rest, weight loss; clubbing, cyanosis, and right ventricular failure.[lumen.luc.edu]
  • Pneumothorax – Acute exacerbations of ILD: Defined as acute clinical deterioration (over – Miscellaneous: drug-induced pulmonary toxicity, diffuse alveolar hemorrhage Clinical features Acute respiratory failure may be characterized by: Symptoms: – worsening dyspnea[clinicaladvisor.com]
Exertional Dyspnea
  • TREATMENT Refrain from empiric drug treatment(s) until the cause of exertional dyspnea and dry cough is sufficiently clear and other more common causes of exertional dyspnea are eliminated, such as COPD and heart failure.[consultant360.com]
  • Case Report In early 2015, a 73-year-old female presented for evaluation of a persistent dry cough that started 2 years ago and had progressively worsened with exertional dyspnea over the past week.[link.springer.com]
  • Onset of the disease is insidious or subacute, with cough and exertional dyspnea. Some 50% of patients present constitutional symptoms and some 30% finger clubbing.[archbronconeumol.org]
Pulmonary Disorder
  • CONCLUSIONS: Chronic interstitial lung diseases in children are a group of rare pulmonary disorders, but a relevant one because of the possible progression to pulmonary fibrosis.[ncbi.nlm.nih.gov]
  • ILD includes many different pulmonary disorders, and some symptoms may vary based on an individual condition.[lunginstitute.com]
  • Since interstitial lung disease (ILD) is not a single disease but a group of more than 200 different pulmonary disorders, it can be confusing to understand exactly what you are dealing with, how a diagnosis is made, and who needs to be involved.[my.clevelandclinic.org]
Regurgitation
Dermatitis
  • Serum biomarkers such as cytokines and chemokines could be affected by the whole disease status, including myositis, dermatitis and ILD, in PM/DM.[academic.oup.com]
Encephalopathy
  • Hyperammonemia and encephalopathy developed in an 11-year-old girl with chronic interstitial lung disease and cholesterol casts in her lung biopsy specimen.[ncbi.nlm.nih.gov]
Altered Mental Status
  • mental status: confusion/delirium, somnolence Signs: – localizing findings on physical examination to suggest infection, volume overload, pneumothorax, DVT – new or worsening hypoxemia and/or hypercapnia – cyanosis – new or worsening radiographic infiltrates[clinicaladvisor.com]

Workup

  • In a prospective analysis of consecutive patients with early stage IPF undergoing initial workup with RHC and pulmonary function testing, H amada et al. [ 18 ] observed that the prevalence of PH in this patient population was as low as 8.1%.[err.ersjournals.com]
  • A proper and detailed history looking for the occupational exposures, and for signs of conditions listed above is the first and probably the most important part of the workup in patients with interstitial lung disease.[en.wikipedia.org]
  • […] can also be found in other diseases such as CTDs, which is another ILD classification, but is idiopathic when the cause is unknown despite a comprehensive workup.[consultant360.com]
  • BAL cell count and differential may be useful in the diagnostic workup of the hospitalized patient with ILD.[bmcmedicine.biomedcentral.com]
Pulmonary Infiltrate
  • . - Pulmonary infiltrates in immunodeficient patients. Pulmonary infiltrates in patients with lowered immunity, primarily those caused by opportunistic pathogens, produce interstitial patterns in chest radiographs.[archbronconeumol.org]
  • Eosinophilic Pneumonia Pulmonary infiltrates and blood/tissue eosinophilia may occur in several distinct diseases: Loffler's syndrome, acute tropical pneumonia, chronic eosinophilic pneumonia, drug-induced eosinophilic pneumonia, Churg-Strauss syndrome[lumen.luc.edu]
  • Usefulness of open lung biopsy in mechanically ventilated patients with undiagnosed diffuse pulmonary infiltrates: influence of comorbidities and organ dysfunction. Crit Care. 2007;11:R93.[bmcmedicine.biomedcentral.com]
Kerley A Lines
  • Lymphangitic Carcinomatosis Progressive dyspnea; CXR showing Kerley B lines, restrictive defect on PFTs; biopsy showing malignant cells.[lumen.luc.edu]
Decreased Lung Compliance
  • Characterized by reduced lung volumes/decreased lung compliance. Give some examples of restrictive lung disorders (6).[quizlet.com]

Treatment

  • Early diagnosis and a long-term, specialized treatment and follow-up are important for the patient outcome. Pediatricians should be aware of these diseases because in many cases diagnosis and treatment are overlooked.[ncbi.nlm.nih.gov]
  • Despite several efforts to improve efficacy of treatment in IPF, only permanent treatment with varying dosages of giucocorticosteroids has been shown to partially improve lung function in IPF.[jhu.pure.elsevier.com]
  • Talmadge King, Harold Collard, and Luca Richeldi, bring you up to date with increased understanding, new treatment protocols, and recent advances in the field.[books.google.com]
  • Specific Treatment The treatment of acute respiratory failure in patients with ILD has two components. The first is supportive; the second is aimed at treating identifiable underlying etiologies.[clinicaladvisor.com]

Prognosis

  • The prognosis is often poor.[ncbi.nlm.nih.gov]
  • Prognosis The prognosis of acute respiratory failure in patients with ILD in general, and IPF specifically, is poor, especially for those who require ICU admission and/or mechanical ventilation.[clinicaladvisor.com]
  • The prognosis of patients with PLCH and PH is still not well known.[err.ersjournals.com]
  • Prognosis. In the case of pneumonitis with a good response to treatment, the prognosis is favorable. The prognosis for fibrosis is variable.[archbronconeumol.org]

Etiology

  • The first is supportive; the second is aimed at treating identifiable underlying etiologies.[clinicaladvisor.com]
  • This review summarizes the pathologic spectrum of idiopathic interstitial pneumonias together with other inflammatory process with known or suggestive etiologies simulating interstitial pneumonias.[jpatholtm.org]
  • However, most cases of ILD are of uncertain classification or etiology (1).[cdc.gov]
  • […] such as connective tissue diseases and hypersensitivity pneumonia IPF was previously called chronic interstitial pneumonitis or cryptogenic fibrosing alveolitis Sites Bottom of lower lobes of bilateral (or "bibasilar") lung Pathophysiology / etiology[pathologyoutlines.com]
  • […] recovery. interstitial lung disease Diffuse interstitial pulmonary fibrosis Pulmonology A group of disorders characterized by scarring of deep lung tissue, leading to SOB and loss of functional alveoli, limiting O 2 exchange; ILD is more common in smokers Etiology[medical-dictionary.thefreedictionary.com]

Epidemiology

  • CDC has initiated a series of toxicologic studies and is considering additional epidemiologic investigation within the flocking industry.[cdc.gov]
  • Pathophysiology NA Epidemiology The overall incidence of acute respiratory failure in patients with ILD has not been well established. Infection and acute exacerbations of underlying ILD appear to be the most common precipitating etiologies.[clinicaladvisor.com]
  • Based on the Bernalillo County, NM, USA registry data published in 1994, the overall estimated incidence is approximately 30 cases per 100,000 persons per year. [2] Rates of interstitial lung disease are somewhat higher in men than in women, and the epidemiology[emedicine.medscape.com]
  • The epidemiology of PH in IPF is not well described due to several factors. First, IPF is an insidious disease, so PH is usually diagnosed at an advanced stage of the disease, thus making the incidence of PH in the setting of IPF difficult to study.[err.ersjournals.com]
  • Epidemiology Epidemiological data on DILD are sparse. The incidence and prevalence reported in the literature are highly variable because the epidemiological methods used differ from one study to another.[archbronconeumol.org]
Sex distribution
Age distribution

Pathophysiology

  • In addition, recommendations for the management of paediatric interstitial lung diseases, as well as new insights into interstitial lung diseases pathophysiology during childhood are discussed.[ncbi.nlm.nih.gov]
  • Serum IL-8 levels and the balance between IL-4 and IFN-γ may contribute to the differences in pathophysiology between anti-ARS-ILD and anti-MDA5-ILD.[academic.oup.com]
  • Pathophysiology NA Epidemiology The overall incidence of acute respiratory failure in patients with ILD has not been well established. Infection and acute exacerbations of underlying ILD appear to be the most common precipitating etiologies.[clinicaladvisor.com]
  • […] seen in other etiologies such as connective tissue diseases and hypersensitivity pneumonia IPF was previously called chronic interstitial pneumonitis or cryptogenic fibrosing alveolitis Sites Bottom of lower lobes of bilateral (or "bibasilar") lung Pathophysiology[pathologyoutlines.com]

Prevention

  • As no ILD primary prevention studies have been previously conducted, we propose that research studies that promote implementation of primary prevention strategies could, over time, make a subset of ILD preventable.[vivo.med.cornell.edu]
  • Prevention Since the late 1970s, many regulations have been enacted to protect workers from asbestos exposure. Reducing or eliminating asbestos exposure is the best way to prevent asbestosis.[asbestos.com]
  • Is there a way to prevent chILD? There’s no way to prevent chILD but you can take steps to avoid diseases that may make it worse. Frequent hand-washing and keeping your child away from pollutants like cigarette smoke can improve her quality of life.[childrens.com]
  • Getting a flu shot every year can help prevent both the flu and pneumonia. In addition, pneumococcal bacteria can cause minor problems such as ear infections.[cedars-sinai.edu]

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