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]
RB-ILD usually presents with mild symptoms and is associated with a good prognosis. [pediatrics.aappublications.org]
Capillaritis is not present. Specific features of WG are not present. The findings are simply those of alveolar hemorrhage of an unknown cause. FIGURE 7 Wegener’s granulomatosis. [nature.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]
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]
Oxygen therapy should be prescribed if hypoxemia is detected with exercise or persists at rest. [consultant360.com]
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]
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]
Prednisolone was tapered down to 5 mg but the patient began deteriorating clinically with symptoms of cough and breathlessness, despite remaining a non-smoker. [thorax.bmj.com]
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]
- 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]
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]
For example, exertional dyspnea in ILD resembles dyspnea experienced by patients with more common ailments such as asthma, COPD, and heart disease. [consultant360.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]
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]
- 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]
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]
- 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]
“Velcro rales” are common in most forms of ILD. They are less likely to be heared in sarcoidosis. Inspiratory squeaks typical of Boop. Clubbing: most commonly seen in IPF but non-specific. [slideshare.net]
Cardiovascular
- 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]
Chest pain and weight loss were less frequent and hemoptysis and fever were reported incidentally [[ 8 ]], and might have resulted from underlying disease including lower respiratory tract infection [[ 26 ]]. [ojrd.biomedcentral.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]
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
[…] 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]
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]
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]
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]
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]
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]
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]
Epidemiology The prevalence and incidence of RB-ILD is unknown and has remained difficult to assess for many years. [ojrd.biomedcentral.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]
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]
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]
Pathophysiology of Sjögren's syndrome Like other autoimmune diseases, the aetiopathogenesis of Sjögren's syndrome combines environmental factors such as viruses (cytomegalovirus, HIV, Human T-cell leukaemia virus, and hepatitis C virus) [ 9 ] or solvents [err.ersjournals.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]