CASE PRESENTATION A 47-year-old Indian woman, who was a non-smoker, presented with severe upper interscapular back pain since 1 month. Radiological evaluation revealed a right upper lobe lung mass with T5 and T9 vertebral body metastasis. [omcr.oxfordjournals.org]

In the present cases, we indicate that serum ferritin level correlated with activity of A/SIP with DM. Two patients, a 65-year-old woman and a 30-year-old woman, were diagnosed with anti-MDA5 antibody-associated A/SIP with DM. [ncbi.nlm.nih.gov]

In addition, the presentation of a patient with AIP may overlap with the clinical presentation of patients with underlying fibrotic disease who present with an acute exacerbation. [emedicine.medscape.com]

Foci of hyaline membrane, associated with the presence of thrombi within capillaries and small pulmonary arteries are often present. Prominent alveolar cell hyperplasia and foci of squamous metaplasia may also be present. [histopathology-india.net]

Antibiotic therapy for all patients presenting with SIRS criteria (temperature >38.0°C or 90 bpm, respiratory rate > 20 or PCO2 < 32 Torr). [emdocs.net]

• Fever

  Acute interstitial pneumonitis typically progresses rapidly, with hospitalization and mechanical ventilation often required only days to weeks after initial symptoms of cough, fever, and difficulties breathing develop. [en.wikipedia.org]

  The disease is thought to be preceded clinically by a viral episode with myalgia, arthralgia, fever and malaise. The respiratory insufficiency develops relatively quickly. [pulmonary-fibrosis.net]

  Symptoms of acute interstitial pneumonia consist of the abrupt onset of fever, cough, and shortness of breath, which in most patients increase in severity over 7 to 14 days, progressing to respiratory failure. [merckmanuals.com]

  Symptoms In the beginning, these patients can have a dry, unproductive cough, shortness of breath, and/or a low-grade fever. Unfortunately, this form of interstitial lung disease is rapidly progressing and is often fatal. [child-foundation.org]

  Symptoms include dyspnea, cough, fever. CXR: Basilar reticular or fine nodular changes. [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]

  His physical examination showed hypoxemia (PulseO 2 79%), tachypnea (31/min), tachycardia (120/min) and diffuse crackles at the bilateral basal lung zones. [oatext.com]

  Oxygen therapy should be prescribed if hypoxemia is detected with exercise or persists at rest. [consultant360.com]

  […] dyspnea – cough – sputum production – chest pain – fever – hemoptysis – altered mental status: confusion/delirium, somnolence Signs: – localizing findings on physical examination to suggest infection, volume overload, pneumothorax, DVT – new or worsening hypoxemia [clinicaladvisor.com]

  Worsening hypoxemia in a patient diagnosed with IPF could be secondary to pneumonia, pulmonary embolism and heart failure [Table 1]. [thoracicmedicine.org]

• Pathologist

  Pathologists also share in the confusion. Terms that were commonly used by pathologists just a few years ago have also undergone an evolution. [thedoctorsdoctor.com]

  Our multidisciplinary care team of pediatric lung specialists, pathologists, radiologists, nutritionists, social workers and nurse specialists brings a wide range of experience and expertise. [childrenscolorado.org]

  Histologic classification Can be complex, [1] and the combined efforts of clinicians, radiologists, and pathologists can help in the generation of a more specific diagnosis. [2] [3] Idiopathic interstitial pneumonia can be subclassified based on histologic [librepathology.org]

  New, high-resolution CAT scans Minimally invasive diagnostic techniques — including bronchoscopy and bronchoalveolar lavage (BAL) Expert thoracic surgeons for diagnostic procedures requiring surgery Lab reports read by a pulmonologist and a pathologist [uchospitals.edu]

  […] of the following medical societies: American Association for the Advancement of Science, American College of Chest Physicians, American Medical Association, American Society for Investigative Pathology, American Thoracic Society, College of American Pathologists [emedicine.medscape.com]

• Lymphadenopathy

  Lymphadenopathy in left hilus, right hilus and paratracheal (1-2-3 sign). Often with calcifications. Uncommon findings: Conglomerate masses in a perihilar location. [radiologyassistant.nl]

  No finger‐clubbing, peripheral cyanosis, heart murmur or lymphadenopathy were noted, but fine crackles were audible over both sides of the chest. [academic.oup.com]

  A younger patient with nodular changes along bronchovascular structures and bilateral hilar lymphadenopathy on HRCT imaging is highly likely to have a diagnosis of sarcoidosis, and the presence of a significant lymphocytosis on a BAL cell count determination [link.springer.com]

• Chills

  Common initial symptoms include myalgia, arthralgia, pyrexia, chills, and malaise. Severe exertional dyspnea develops over a matter of days to weeks 13. AIP is characterized histologically by diffuse alveolar damage (DAD) 2. [radiopaedia.org]

  Brit Long, MD (@long_brit, EM Attending Physician at SAUSHEC) A 55 year-old female with a history of idiopathic pulmonary fibrosis and pulmonary hypertension (prednisone, sildenafil, and bosentan therapy) presents with a three day history of cough, chills [emdocs.net]

  Fever, chills, dyspnea, leukocytosis may occur 4-6 hours after exposure and eventually resolve; symptoms and signs may recur on re-exposure. CXR: Acute - normal to reticulonodular pattern; Chronic - progressive fibrosis, honeycombing. [lumen.luc.edu]

• Cough

  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]

  Acute interstitial pneumonitis typically progresses rapidly, with hospitalization and mechanical ventilation often required only days to weeks after initial symptoms of cough, fever, and difficulties breathing develop. [en.wikipedia.org]

  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]

  More common symptoms of chILD include Abnormal shadows on chest X-rays or CT scans Abnormal lung function tests Rapid breathing Trouble breathing Wheezing Shortness of breath when exercising Chronic or intermittent coughing Coughing up blood Recurring [childrens.com]

  The most common symptoms of interstitial lung disease are a dry cough and shortness of breath. [medicinenet.com]

• Dyspnea

  On the 215th day after initiation of alectinib administration, she was admitted to our hospital with the symptom of progressive dyspnea. [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]

  Clinically, it is characterized by an insidious onset of breathlessness with exertion and a nonproductive cough, leading to progressive DYSPNEA. [fpnotebook.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]

• Dry Cough

  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]

  Many interstitial lung disease sufferers also experience a dry cough. Weight loss can also be a common symptom of interstitial lung disease, especially in patients who have pneumonia. [callung.com]

  A person may have these symptoms: A dry cough Shortness of breath, which may occur after effort or become worse over time Difficult or labored breathing Fatigue Clubbing, or enlargement of the fingertips at the base of the nails. [my.clevelandclinic.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 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 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]

  Severe exertional dyspnea develops over a matter of days to weeks 13. AIP is characterized histologically by diffuse alveolar damage (DAD) 2. [radiopaedia.org]

• Dyspnea at Rest

  […] that progresses to dyspnea at rest Persistent nonproductive cough Fatigue Other symptoms related to underlying primary disorder (eg, joint pain in connective tissue disorder ) Later stages of disease: digital clubbing due to chronic hypoxia IPF : The [amboss.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]

• Sweating

  Fever occurs in virtually all individuals and drenching sweats are frequent. The chest x-ray often shows a distinctive pattern. This consists of fluffy or patchy infiltrates in the periphery of the lungs. [eplerhealth.com]

  Systemic symptoms such as fatigue, night sweats and weight loss are common. Loefgren's syndrome, an acute presentation of sarcoidosis, consists of arthritis, erythema nodosum, bilateral hilar adenopathy and occurs in 9-34% of patients. [radiologyassistant.nl]

• Myalgia

  The disease is thought to be preceded clinically by a viral episode with myalgia, arthralgia, fever and malaise. The respiratory insufficiency develops relatively quickly. [pulmonary-fibrosis.net]

  Common initial symptoms include myalgia, arthralgia, pyrexia, chills, and malaise. Severe exertional dyspnea develops over a matter of days to weeks 13. AIP is characterized histologically by diffuse alveolar damage (DAD) 2. [radiopaedia.org]

  Worsening shortness of breath, particularly worse on exertion (Obstructive sleep apneoa) Other features include finger clubbing fine late inspiratory crackles cyanosis rarely, other constitutional symptoms e.g. flu-like illness, fatigue, weight loss; myalgia [dundeemedstudentnotes.wordpress.com]

  Pleuritic chest pain and myalgia may be present in over half of all patients. The average age at presentation is 30 years-old and there is no gender preference. A smoking history is present in 40% of patients ( 6, 59 ). [shortnessofbreath.it]

  A patient with a significant exposure history to potential organic antigens (e.g. bird fancier or farmer) with a HRCT findings of upper lung field dominant centrilobular ground glass nodules, acute or subacute onset of symptoms (e.g. dyspnea, myalgias [link.springer.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]

[…] often needed to make a diagnosis, with pertinent information and expertise contributed by the pulmonologist, radiologist and pathologist. 4 High-resolution CT should be obtained in virtually all patients with a suspected ILD. 4 Additional diagnostic workup [appliedradiology.com]

BAL cell count and differential may be useful in the diagnostic workup of the hospitalized patient with ILD. [bmcmedicine.biomedcentral.com]

• Pulmonary Infiltrate

  infiltrates, PaO 2 /FiO 2 ≤ 300, and PAWP ≤ 18) in the absence of heart failure, pulmonary infection, pulmonary embolism (PE), aspiration, or drug reaction. 4,10-13 Acute exacerbations have been documented in patients with the subgroup classifications [emdocs.net]

  Radiology A chest x-ray is performed at first instance, showing bilateral pulmonary infiltrates. This aspect is not specific for any etiology; however, it can be useful to rule out pneumothorax. [shortnessofbreath.it]

  Chronic Eosinophilic Pneumonia Cough, dyspnea, wheezing, fever, malaise, blood eosinophilia, and dense focal alveolar infiltrates. Cause unknown; course in subacute or chronic. CXR: "photographic negative of pulmonary edema." [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]

• Lymphocytosis

  Computed tomography (CT) revealed diffuse ground glass opacities and consolidations in both lungs, and analysis of bronchoalveolar lavage fluid revealed pronounced lymphocytosis. [ncbi.nlm.nih.gov]

  BAL :- lymphocytosis CD4 : CD8 > 3.5 is most specific PFT :- Restrictive pattern But Obstructive component present in many Biopsy :- non caseating granulomas lymphocytosis Sr. ACE levels:- Hyper calciuria or Hypercalcemia 31. RARE ILD 32. [slideshare.net]

  Investigations Blood tests: FBC (may be raised neutrophilia, lymphocytosis, eosinophilia), raised ESR and CRP. Blood gases: hypoxaemia. Sputum or culture of lung secretions with bronchoscopy. [patient.info]

  An increase in BALF neutrophils has also been suggested, however, lymphocytosis can be seen ( 39 ). [shortnessofbreath.it]

  […] lung disease, especially idiopathic pulmonary fibrosis (IPF) : history of IPF, background of dense fibrosis and honeycombing Acute hypersensitivity pneumonitis : history of exposure to causative antigens, remission of symptoms after antigen removal, lymphocytosis [pathologyoutlines.com]

• Neutrophilia

  Investigations Blood tests: FBC (may be raised neutrophilia, lymphocytosis, eosinophilia), raised ESR and CRP. Blood gases: hypoxaemia. Sputum or culture of lung secretions with bronchoscopy. [patient.info]

  BAL findings were characteristic, with a huge neutrophilia associated with scattered atypical type II pneumocytes collected in clusters with extracellular amorphous material (fragments of hyaline membranes) observed in two out of three cases. [erj.ersjournals.com]

  [49] Bronchoscopic assessment The role of BAL is pivotal in excluding infection as the etiology of AE-IPF; however, negative BAL culture still does not exclude the possibility of viral infections. [33] AE-IPF is morphologically characterized by BAL neutrophilia [thoracicmedicine.org]

  Morphologically, this leads to neutrophilia in BAL and histopathological DAD ( 8, 17, 24 ). [frontiersin.org]

• Lymphocytes Increased

  BAL: Predominance of lymphocytes; increased IgG, IgM. Serum precipitins to offending antigen present. [lumen.luc.edu]

This makes high dose steroid as an attractive treatment option. [oatext.com]

After four courses treatment, the patient was judged to have progressive disease (PD). We had selected monotherapy using docetaxel (60 mg/m 2 ) for the 2 nd line treatment. [cancerjournal.net]

Still, treatments can slow lung damage and help you breathe more easily. Lung transplant remains an option for those who don’t respond to other medical treatment. [healthline.com]

The lack of reports describing the use of CyA for the treatment of RA‐associated AIP warrants caution in its use; nevertheless, in our opinion, the poor prognosis of this complication of RA justifies treatment with this agent. F ig. 1. [academic.oup.com]

These cases generally occurred within 2 months after the initiation of treatment [ 6 ]. [omcr.oxfordjournals.org]

We assessed the correlation between the serum PGRN levels and the activity indexes of ILD or prognosis in DM patients with ILD. [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]

Prognosis good but depends on the extent of fibrosis at diagnosis greater than 10 years. But Surgical Biopsy is needed to confirm. [slideshare.net]

Even despite mechanical ventilation, it often carries a grave prognosis with > 70% mortality at ~ 3 months 1. [radiopaedia.org]

Prognosis Prognosis is good with early diagnosis and management. Late-diagnosed chronic pneumonitis may lead to progressive, irreversible lung disease. [patient.info]

There is a wide variety of well-known etiologies, including infection/sepsis, shock, trauma, aspiration, oxygen toxicity and many others. [eprints.bice.rm.cnr.it]

The first is supportive; the second is aimed at treating identifiable underlying etiologies. [clinicaladvisor.com]

[…] specific etiologic agent or inciting event. [emedicine.medscape.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]

Katzenstein reserves the term AIP for those cases where there is no identifiable underlying etiology (such as sepsis). [histopathology-india.net]

The epidemiology of interstitial lung diseases. Am J Respir Crit Care Med. 1994; 150:967-972. Coultas D, Hughes M. Accuracy of mortality dat for interstitial lung diseases in New Mexico, USA. Thorax. 1996; 51:717-720. [emdocs.net]

Extremely rare (no conclusive epidemiological data available) Mean age 50 years but can occur at any age (7 - 83 years) ( Eur Respir J 2000;15:412 ) No sex predilection Sites Bilateral lung, usually in all five lobes of the lung Pathophysiology Both [pathologyoutlines.com]

Epidemiology [ edit ] Acute interstitial pneumonitis occurs most frequently among people older than forty years old. It affects men and women equally. There are no known risk factors; in particular, smoking is not associated with increased risk. [en.wikipedia.org]

Epidemiology It is common, if all causes of pneumonitis are considered. [patient.info]

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]

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]

However, pathophysiological mechanism and predictive patient and tumor related risk factors for crizotinib-induced ILD are not well understood. [oatext.com]

Eosinophilic pneumonia Eosinophilic pneumonia is similar to organizing pneumonia in pathophysiology, but is mediated instead by an inappropriate increase in the number of eosinophils and macrophages in alveoli. [appliedradiology.com]

[…] damage Epidemiology Extremely rare (no conclusive epidemiological data available) Mean age 50 years but can occur at any age (7 - 83 years) ( Eur Respir J 2000;15:412 ) No sex predilection Sites Bilateral lung, usually in all five lobes of the lung Pathophysiology [pathologyoutlines.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]

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]

The goal of treatment for people with ILD is to prevent more scarring and manage symptoms. [choc.org]

[…] 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]

Prevents the immune system from attacking cells in the body that cause fibrosis. Azathioprine – Another drug that may be used to reduce the amount of steroids required. Cyclophosphamide – Used if steroid treatment is ineffective or not possible. [inogen.com]