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]
It is the authors’ belief that RB-ILD was the cause of the initial illness at presentation and she showed improvement after smoking cessation alone. [thorax.bmj.com]
The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment. [orpha.net]
A contemporary overview of these conditions with special emphasis on diagnosis is presented. [books.google.com]
Entire Body System
- 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]
Some patients are sensitive to light and have red, painful eyes, while others may be systemically ill, with symptoms including fevers, chills, and unintentional weight loss. Causes A variety of microbes cause retinitis. [asrs.org]
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]
Late: dyspnea at rest, weight loss; clubbing, cyanosis, and right ventricular failure. Chest Roentgenography Early: normal to ground-glass pattern and nodular, reticular or reticulonodular patterns. [lumen.luc.edu]
- 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]
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]
Oxygen therapy should be prescribed if hypoxemia is detected with exercise or persists at rest. [consultant360.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]
- Congestive Heart Failure
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]
Cancer in some cases spreads throughout the lymph system in the lungs and appears as interstitial disease Congestive heart failure, renal failure both can present with similar findings due to excess fluid in the spongy tissue of the lung Examples of idiopathic [medicinenet.com]
- Malnutrition
The physical examination revealed malnutrition (5th to 15th BMI percentile) and clubbing. Resting oxygen saturation (96%) was normal, and there was no desaturation ( Fig 1 ). [pediatrics.aappublications.org]
Respiratoric
- 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]
せき(咳) croupクループ cryptogenic特発性 cuffed tracheal tubeカフ付き気管チューブ d deformity奇形 desensitization脱感作 diaphragm 横隔膜 difficulty in breathing呼吸困難 diffuse びまん性の diffuse panbronchiolitis びまん性汎細気管支炎 diffusion拡散 discontinuous sound[s]断続[性ラ]音 dry cough乾性咳[嗽]、痰を伴わないせき [tokyo-med.ac.jp]
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]
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]
- 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]
For example, exertional dyspnea in ILD resembles dyspnea experienced by patients with more common ailments such as asthma, COPD, and heart disease. [consultant360.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]
A 60-year-old woman was seen in a pulmonary clinic because of increasing dyspnea. Chest imaging revealed bronchiectasis and mild, diffuse bronchial-wall thickening. A diagnostic procedure was performed. [resident360.nejm.org]
The onset is usually insidious, with exertional dyspnea and progressive persistent cough, which may be nonproductive. [pediatrics.aappublications.org]
- 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]
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]
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]
- Aspiration
Pneumonia (see Aspiration Pneumonia, [[Aspiration Pneumonia]]) Physiology: due to aspiration of foreign material (vegetable fragments, excipients from oral pills, etc) Bronchocentric Granulomatosis (see Bronchocentric Granulomatosis, [[Bronchocentric [mdnxs.com]
Acid-blocking medicines can help kids with acid reflux avoid aspiration as well. Surgery Children with severe chILD may need a lung transplant if other treatments haven't worked. [childrens.com]
[…] chILD Foundation: Acute interstitial pneumonia Alveolar capillary dysplasia with misalignment of pulmonary veins Alveolar hemorrhage syndromes: Pulmonary capillaritis Acute idiopathic pulmonary hemorrhage of infancy Idiopathic pulmonary hemosiderosis Aspiration [child-foundation.org]
吸引 aspiration pneumonia燕(えん)下性肺炎 asthma喘息 atelectasis無気肺 atopyアトピー atypical非定型の b bacillus菌、細菌、桿菌 barrel chest 樽状胸 bifurcation分岐 bifurcation of trachea (carina)気管分岐部 bilateral hilar lymphadenopathy (BHL)両側肺門リンパ節腫脹 blood gas血液ガス bloody sputum血痰 breath [tokyo-med.ac.jp]
[…] pulmonary alveolar proteinosis Neuroendocrine hyperplasia of infancy, pulmonary interstitial glycogenosis Lung disorders related to the body’s response to infection, drugs and environmental toxins (hypersensitivity pneumonitis, bronchiolitis obliterans) Aspiration [mottchildren.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 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]
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]
The onset is usually insidious, with exertional dyspnea and progressive persistent cough, which may be nonproductive. [pediatrics.aappublications.org]
Cardiovascular
- Heart Failure
This form of hypertension can develop in cases of long-standing interstitial lung disease and can lead to enlargement of the heart and right-sided (the side of the heart that pumps blood back to the lungs to receive oxygen) heart failure (known as cor [medicinenet.com]
In advanced stages of disease ILD can result in pulmonary insufficiency and respiratory heart failure with right ventricular insufficiency. [amboss.com]
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]
Definition (CSP) chronic inflammation and progressive fibrosis of the pulmonary alveolar walls with steady progressive dyspnea; finally, resulting in death from lack of oxygen or right heart failure. [fpnotebook.com]
Cardiovascular findings were normal and there was no evidence of pulmonary hypertension or heart failure. [racgp.org.au]
- Chest Pain
Clinical manifestations Most commonly in individuals 20-50 years of age; associated with smoking Cough, dyspnea, chest pain. Pneumothorax (10-30%); rarely diabetes insipidus. [lumen.luc.edu]
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]
• Other unusual chest symptoms: – Haemoptysis e.g. alveolar hemorrhage syndromes, pulmonary vascular diseases – Pleuritic chest pain e.g. collagen vascular illness. – Wheezing e.g. [slideshare.net]
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]
Pain Sarcoidosis (substernal pain is common) SLE-associated ILD (pleuritic) RA-associated ILD (pleuritic) MCTD-associated ILD (pleuritic) Symptoms Preceding Onset of a Connective Tissue Disease ILD may precede other manifestations of disease by months-yrs [mdnxs.com]
- Heart Disease
Use cautiously with liver disease or alcoholism. Smokers who consume alcohol and beta-carotene may be at an increased risk for lung cancer or heart disease. [columbiaeye.org]
For example, exertional dyspnea in ILD resembles dyspnea experienced by patients with more common ailments such as asthma, COPD, and heart disease. [consultant360.com]
Musculoskeletal
- Fracture
Vitamin A intake and the risk of hip fracture in postmenopausal women: the Iowa Women’s Health Study. Osteoporos Int. 2004;15:552-559. Lips P. Hypervitaminosis A and fractures. N Engl J Med. 2003;348:347-349. Loktev AV, Zhang Q, Beck JS, et al. [rarediseases.org]
Potential implications Management Gastro-oesophageal reflux disease Lung injury, acceleration of lung fibrosis Proton pump inhibitor H 2 -receptor antagonist Prokinetic agents (mainly scleroderma) Surgery in selected cases Osteoporosis* Vertebral and rib fractures [racgp.org.au]
- Contusion
Primary pulmonary risk factors include aspiration, pneumonia, toxic inhalation and pulmonary contusion. Extrapulmonary risk factors are sepsis, pancreatitis, multiple blood transfusions, trauma and the use of drugs such as heroin. [radiologyassistant.nl]
Neurologic
- Confusion
Over time, the diagnostic criteria for RBILD have become progressively more confused. OBJECTIVE: To review the diagnostic criteria for RBILD. DESIGN: The review was based on the literature and personal experience. [ncbi.nlm.nih.gov]
Citation: Consultant. 2015;55(2):120-124 Interstitial lung disease (ILD) has a history of baffling clinicians for several reasons—2 of those being confusing terminology and classification, which continue to thwart clinicians’ ability to establish a clear [consultant360.com]
The acute or subacute processes are often confused with atypical pneumonias, since many have diffuse radiographic opacities, fever, or relapses of disease activity. [quizlet.com]
Discussion Bronchiolar abnormalities and their terminology have been confusing for both clinicians and pathologists. [nature.com]
Diagnostic criteria In the last 30 years there has been a great deal of confusion concerning the diagnostic criteria for SS, with competition between different more or less restrictive definitions, resulting in great variations in the prevalences reported [em-consulte.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]
[…] 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]
In our opinion, screening for SS should be performed in the etiological workup for diffuse bronchiectasis. Bronchiolar involvement can reveal SS. There are three cases in the literature of diffuse bronchiolitis occurring in patients with primary SS. [em-consulte.com]
X-Ray
- 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]
HRCT may show ground glass appearance or honeycomb cysts. BAL: Predominance of neutrophils. Diagnosis: By exclusion, usually need open lung biopsy to confirm. [lumen.luc.edu]
The radiographic appearance can suggest NSIP with ground glass opacities, mainly subpleural and posterior, with signs of fibrosis variable in extent (subpleural reticulations, sometimes honeycomb cysts). [em-consulte.com]
Pulmonary Function Test
- Lung Function Testing Abnormal
Bronchoalveolar lavage (BAL) is abnormal in 50% of patients with SS, often in the absence of clinical, radiological or lung function test abnormalities [ 78 ]. [em-consulte.com]
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]
The decision for a longer duration of treatment was therefore made. Notwithstanding, smoking cessation is very important for the course of the disease, as shown in this case. [thorax.bmj.com]
[…] and prognosis Usually, no treatment is required other than giving up smoking. [radiopaedia.org]
Prognosis
Conclusions UIP in pSS–ILD was not related to poorer prognosis than NSIP. Assessment of detailed clinical–radiologic–pathologic findings is more important than distinguishing UIP to evaluate prognosis in this disease. [journals.plos.org]
What is the prognosis for retinitis pigmentosa? The prognosis varies widely among RP patients, with some retaining 20/40 or better vision in at least one eye and others eventually losing all useful central vision. [medicinenet.com]
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]
Fibrotic disease generally carries a worse prognosis. [mdedge.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]
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]
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]
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]
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]
Epidemiology The prevalence and incidence of RB-ILD is unknown and has remained difficult to assess for many years. [ojrd.biomedcentral.com]
Other causes of the usual interstitial pneumonia pattern include domestic and occupational environmental exposures, connective tissue disease, and drug toxicity. 28 An epidemiologic association between smoking and usual interstitial pneumonia is well [mdedge.com]
The epidemiology of idiopathic pulmonary fibrosis and interstitial lung diseases at risk of a progressive-fibrosing phenotype. Eur Respir Rev 2018; 27 (150): 180077. [ Links ] 19. Hutchinson J, Fogarty A, Hubbard R, McKeever T. [scielo.cl]
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]
Murine models for SS shed light on the important role played by B lymphocytes in the pathophysiology of the disease. [em-consulte.com]
New insights into the pathophysiology of various entities of SR-ILD have paved the way for specific treatments. [aerzteblatt.de]
Bardet-Biedl syndrome: Genetics, molecular pathophysiology, and disease management. Indian J Ophthalmol. 2016;64(9):620-7. Radtke ND, Aramant RB, Seiler MJ, Petry HM, Pidwell D. [rarediseases.org]
Indeed, there can be considerable inter-individual variability in genetic abnormalities that have predisposed an individual to develop the disease, in pathophysiologic characteristics of the disease process, and in responses to specific drugs. [transrespmed.springeropen.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]
To help prevent blindness, doctors both treat the retinitis and work to strengthen your immune system. You may need an antiviral medication such as ganciclovir. You might take pills by mouth or receive an injection into a vein or eye. [webmd.com]
An eye test can spot some eye conditions and, if caught early, treatment may prevent further deterioration. [fightforsight.org.uk]