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Respiratory Bronchiolitis Interstitial Lung Disease

RB-ILD


Presentation

  • Symptomatic smokers presenting with symptoms mimicking interstitial lung disease are diagnosed with respiratory bronchiolitis–interstitial lung disease ( RB-ILD ).[radiologykey.com]
  • This group of RB-ILD patients had the usual characteristic clinical presentation described previously in other series.[elsevier.pt]
  • RB-ILD usually presents with mild symptoms and is associated with a good prognosis.[pediatrics.aappublications.org]
  • Presentation tends to be with progressive shortness of breath and chronic cough 4 . Inspiratory crackles are heard in up to half of patients. Clubbing is rare 1 .[radiopaedia.org]
  • On BAL, the lavage fluid itself often shows yellow-brownish discoloration, the alveolar macrophages seen in increased numbers have yellow, gold, brown or black pigments; eosinophils, neutrophils and lymphocytes may be present to a slightly increased extent[pulmonary-fibrosis.net]
Weight Loss
  • Many affected individuals also experience a loss of appetite and gradual weight loss. Some people with idiopathic pulmonary fibrosis develop widened and rounded tips of the fingers and toes (clubbing) resulting from a shortage of oxygen.[icdlist.com]
  • As the disease progresses, weight loss, muscle and joint pain, and fatigue may also occur.[radiologyinfo.org]
  • Weight loss, most often in people with COP or BOOP. In most forms of interstitial lung disease, the shortness of breath develops slowly (over months).[webmd.com]
  • Some people may also experience unexplained weight loss. Anyone who is having trouble breathing should see their doctor for a diagnosis.[medicalnewstoday.com]
  • Fevers, fatigue, and weight loss are non-specific, but can suggest an infectious process. It is often difficult to make a diagnosis from symptoms alone, since so many lung diseases can present with shortness of breath and cough.[medicinenet.com]
Hypoxemia
  • […] in patients with interstitial lung disease is V/Q mismatch, with DLCO defect contributing to hypoxemia with activity increased expiratory flow rates due to increased radial traction (result of increased elastic recoil) on airway walls prevents small[medbullets.com]
  • With disease progression, chronic hypoxemia, cyanosis, and finger clubbing occur. The precise diagnosis is often difficult and frequently requires invasive procedures such as bronchoscopy, and in most cases, open lung biopsy.[pediatrics.aappublications.org]
  • Hypoxemia is common during exercise and may be present at rest. HRCT, which distinguishes airspace from interstitial disease, is the most useful test and is always done.[merckmanuals.com]
  • Our patients with RB and associated diseases demonstrated only slight impairment of their LFT - resembling small-airways disease - and rarely presented with hypoxemia.[karger.com]
Death in Infancy
  • Passive smoking has been causally linked to increased risks of respiratory tract infections, middle ear infections, sudden unexplained death in infancy, and asthma, but the relation between smoking and ILD is not clear in childhood. 5 In the literature[pediatrics.aappublications.org]
Cough
  • The onset is usually insidious with exertional dyspnea and persistent cough, which may be non-productive, developing over a course of weeks or months.[ncbi.nlm.nih.gov]
  • The onset is usually insidious, with exertional dyspnea and progressive persistent cough, which may be nonproductive.[pediatrics.aappublications.org]
  • Most patients (73.4%) have mild symptoms, dyspnea on exertion ( n 8/53.3%) and cough ( n 7/46.6%) were the only symptoms identified.[elsevier.pt]
  • CASE 117 Clinical Presentation 41-year-old man with long-standing history of tobacco abuse since age 11, complaining of worsening cough and progressive dyspnea over the past 18 months Radiologic Findings HRCT ( Figs. 117.1A, 117.1B, 117.1C, 117.1D ) demonstrates[radiologykey.com]
  • Presentation tends to be with progressive shortness of breath and chronic cough 4 . Inspiratory crackles are heard in up to half of patients. Clubbing is rare 1 .[radiopaedia.org]
Dyspnea
  • The onset is usually insidious with exertional dyspnea and persistent cough, which may be non-productive, developing over a course of weeks or months.[ncbi.nlm.nih.gov]
  • The onset is usually insidious, with exertional dyspnea and progressive persistent cough, which may be nonproductive.[pediatrics.aappublications.org]
  • Most patients (73.4%) have mild symptoms, dyspnea on exertion ( n 8/53.3%) and cough ( n 7/46.6%) were the only symptoms identified.[elsevier.pt]
  • CASE 117 Clinical Presentation 41-year-old man with long-standing history of tobacco abuse since age 11, complaining of worsening cough and progressive dyspnea over the past 18 months Radiologic Findings HRCT ( Figs. 117.1A, 117.1B, 117.1C, 117.1D ) demonstrates[radiologykey.com]
  • The main symptoms are exertional dyspnea and a dry cough. Bibasilar inspiratory crackles or rales are usually heard on auscultation. Treatment is based on the underlying cause.[amboss.com]
Dry Cough
  • We describe an 11-year-old boy with failure to thrive, dry cough, and exertional dyspnea for 1 year who was diagnosed with RB-ILD due to heavy passive smoking exposure.[pediatrics.aappublications.org]
  • Symptoms The clinical symptoms consist of slowly developing dyspnoea and dry cough, and approximately half the patients have finger clubbing. On lung function tests, restrictive ventilatory impairment and abnormal diffusion are prominent findings.[pulmonary-fibrosis.net]
  • The main symptoms are exertional dyspnea and a dry cough. Bibasilar inspiratory crackles or rales are usually heard on auscultation. Treatment is based on the underlying cause.[amboss.com]
  • The symptoms will vary from person to person, but the most common symptoms of an ILD are: Shortness of breath, especially with exertion or exercise Dry cough that does not produce phlegm Labored breathing Although rapid worsening can happen, ILD's symptoms[uchospitals.edu]
  • The most common symptoms of diffuse interstitial lung disease are shortness of breath and dry cough. As the disease progresses, weight loss, muscle and joint pain, and fatigue may also occur.[radiologyinfo.org]
Exertional Dyspnea
  • The onset is usually insidious with exertional dyspnea and persistent cough, which may be non-productive, developing over a course of weeks or months.[ncbi.nlm.nih.gov]
  • The onset is usually insidious, with exertional dyspnea and progressive persistent cough, which may be nonproductive.[pediatrics.aappublications.org]
  • The main symptoms are exertional dyspnea and a dry cough. Bibasilar inspiratory crackles or rales are usually heard on auscultation. Treatment is based on the underlying cause.[amboss.com]
  • The most important clinical manifestations reported in RB-ILD are dry cough, shortness of breath, exertional dyspnea, and weight loss, eventually dyspnea at rest and rarely thoracic pain.[karger.com]
Rales
  • Bibasilar inspiratory crackles or rales are usually heard on auscultation. Treatment is based on the underlying cause. Immune modulators and corticosteroids are used in cases of unknown etiology.[amboss.com]
  • Children with ILD usually present with failure to thrive, dry cough, dyspnea, and tachypnea and rales. With disease progression, chronic hypoxemia, cyanosis, and finger clubbing occur.[pediatrics.aappublications.org]
  • 肺症 pneumonia肺炎 productive cough 湿性せき、痰を伴うせき psittacosisおうむ病 pulmonary 肺の pulmonary adventitious sound[s]ラ音 pulmonary carcinoma肺癌 pulmonary function test肺機能検査 pulmonary hilum肺門 pulmonary marking[s]肺紋理 pulse oximetryパルスオキシメトリー purulent膿性 r radiation放射線 rale[tokyo-med.ac.jp]
  • -Crackles (velcro rales): may hear even if the chest film was normal -Inspiratory squeaks -Cor pulmonale - left parasternal systolic lift, loud S2 exaggerated in the pulmonic region -Cyanosis - late stage -Clubbing - late stage -Extrapulmonary findings[quizlet.com]
  • Hemoptysis and velcro rales on auscultation were rarely present, and clubbing, reported with an incidence of 12.5-33% in patients with RB-ILD [ 21, 30 ], was only seen in 2 of our cases (11%).[karger.com]
Failure to Thrive
  • We describe an 11-year-old boy with failure to thrive, dry cough, and exertional dyspnea for 1 year who was diagnosed with RB-ILD due to heavy passive smoking exposure.[pediatrics.aappublications.org]
Chest Pain
  • -Progressive exertional dyspnea -Persistent, usually nonproductive cough -Hemoptysis, wheezing, chest pain less common What are the incidental findings that can be seen on a chest x-ray of a patient with an ILD -Interstitial opacities in a reticular,[quizlet.com]
  • The illness develops rapidly in less than five days with respiratory failure and fever, shortness of breath, chest pain and achy joints. These symptoms prompt an admission to the hospital or to the ICU (intensive care unit).[eplerhealth.com]

Workup

  • The finding of RB should prompt further diagnostic workup, and in case of RB-ILD, entail regular screening for lung cancer. 2015 S.[karger.com]
Ground Glass Appearance
  • On HRCT, there is a ground glass appearance, being most prominent in the lower fields and periphery and are sometimes patchy and sometimes homogeneous. There are also centrilobular nodes and condensations of the central and peripheral airways.[pulmonary-fibrosis.net]
Ground Glass Appearance
  • On HRCT, there is a ground glass appearance, being most prominent in the lower fields and periphery and are sometimes patchy and sometimes homogeneous. There are also centrilobular nodes and condensations of the central and peripheral airways.[pulmonary-fibrosis.net]

Treatment

  • The majority of published reports consider RB-ILD to be a nonprogressive ILD that clinically improves with smoking cessation and antiinflammatory treatment.[ncbi.nlm.nih.gov]
  • Some patients respond favorably to corticosteroids and/or smoking cessation, but often there is no functional improvement and the disease progresses despite smoking cessation and treatment.[ncbi.nlm.nih.gov]
  • […] and prognosis Usually, no treatment is required other than giving up smoking.[radiopaedia.org]
  • Steroid treatment was tapered starting at the second month of treatment and was stopped at the seventh month. At his last outpatient visit, 3 years after elimination of smoke exposure, he is a 14-year-old without any complaints.[pediatrics.aappublications.org]

Prognosis

  • This article will give a broad and current overview of these two rarer forms of IIP, including issues that relate to diagnosis, imaging, histopathology, treatment, and prognosis.[thieme-connect.com]
  • […] subpleural regions and more so in the lower lung zones patchy areas of hypoattenuation ( 40%) with a lower lung predominance 10 other changes related to smoking bronchial wall thickening : can be central /- peripheral centrilobular emphysema Treatment and prognosis[radiopaedia.org]
  • RB-ILD usually presents with mild symptoms and is associated with a good prognosis.[pediatrics.aappublications.org]
  • Additionally, they showed a favorable clinical course, even those who continued to smoke, which confirms a good prognosis associated with this disease. Conflict of interest The authors declare no conflict of interest.[elsevier.pt]
  • The prognosis for these conditions is variable depending on the intrinsic behavior of each condition.[journals.lww.com]

Etiology

  • Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology.[icd10coded.com]
  • The etiology of many entities is different from that of adults. Some of these diseases are very rare, and sometimes etiology cannot be identified. Children with ILD usually present with failure to thrive, dry cough, dyspnea, and tachypnea and rales.[pediatrics.aappublications.org]
  • Etiology The cause and pathogenesis are unknown, but RB and RB-ILD are strongly related to tobacco abuse. Clinical Findings Only gold members can continue reading.[radiologykey.com]
  • TABLE 233-1 Etiologies of Interstitial Lung Disease (ILD) by Category View Table Favorite Table Download (.pdf) TABLE 233-1 Etiologies of Interstitial Lung Disease (ILD) by Category Autoimmune Scleroderma Rheumatoid arthritis Systemic lupus erythematosis[accessmedicine.mhmedical.com]
  • Immune modulators and corticosteroids are used in cases of unknown etiology. In advanced stages of disease ILD can result in pulmonary insufficiency and respiratory heart failure with right ventricular insufficiency.[amboss.com]

Epidemiology

  • The exact epidemiology is difficult to determine due to misidentification of patients as having more common disorders such as congestive heart failure or chronic obstructive pulmonary disease (COPD). The epidemiology varies based on the ILD subtype.[accessmedicine.mhmedical.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]
  • Describe the epidemiology of sarcoidosis (2).[quizlet.com]
Sex distribution
Age distribution

Pathophysiology

  • Introduction Definition restricted lung expansion results in reduced lung volumes (TLC and FVC) Pathophysiology poor breathing mechanics with normal lung function muscle weakness polio and myasthenia gravis chest wall limitation kyphosis, scoliosis, and[medbullets.com]

Prevention

  • 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]
  • Can interstitial lung disease be prevented? Interstitial lung disease can be prevented only to the extent that its individual causes may be prevented.[medicinenet.com]
  • […] resting hypoxemia in patients with interstitial lung disease is V/Q mismatch, with DLCO defect contributing to hypoxemia with activity increased expiratory flow rates due to increased radial traction (result of increased elastic recoil) on airway walls prevents[medbullets.com]
  • When interstitial disease is present, the lung becomes inflamed and stiff, preventing the alveoli from fully expanding. This limits both the delivery of oxygen to the blood stream and the removal of carbon dioxide from the body.[radiologyinfo.org]
  • Antifibrotic drugs such as pirfenidone (Esbriet) and nintedanib (Ovef) may prevent further scarring in the lungs. These drugs are both approved by the U.S. Food and Drug Administration for the treatment of idiopathic pulmonary fibrosis.[healthline.com]

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