Pulmonology

Diffuse Parenchymal Lung Disease

Questions discussed in this category

What criteria do you use to decide when to perform a BAL in hypersensitivity pneumonitis?

Do you perform it on every suspected case, or are there specific indications you look for before proceeding with BAL?

2 Answers available

How do you approach PJP prophylaxis in patients with rheumatic disease on corticosteroids?

Does the presence of ILD change your approach?

3 Answers available

Do you perform routine interval screening for pulmonary fibrosis in individuals who have been identified through genetic testing as being at risk for developing pulmonary fibrosis but don't exhibit any signs or symptoms?

Specifically considering individuals with known mutations in MUC5B, TERT/TERC, etc

2 Answers available

What is the duration of steroids that you prescribe for organizing pneumonia?

1 Answer available

What is your approach to evaluation and management of GERD in a patient with IPF?

1 Answer available

Would you offer lung SBRT in a patient with Pulmonary Langerhans Cell Histiocytosis (PLCH)?

1 Answer available

Are you using genomic classifiers to diagnose UIP when imaging is indeterminate or suggestive of an alternate diagnosis?

4 Answers available

Is it necessary to prescribe a steroid taper after two weeks of high-dose prednisone (60 mg daily)?

4 Answers available

Have you adopted the use of low dose morphine for treatment of cough in patients with IPF?

If so, what dose and formulation of morphine do you prefer?The PACIFY COUGH trial utilized controlled-release morphine 5 mg BID, however the lowest do...

1 Answer available

Do you administer immunosuppression to patients with idiopathic NSIP who have normal lung function and mild to moderate respiratory symptoms?

1 Answer available

How do you approach a patient with sarcoidosis who cannot tolerate steroids and who is developing ILD?

Do you use TNF-i or MMF?

1 Answer available

Do you monitor CBCs to assess for drug toxicity in patients on nintedanib?

1 Answer available

How do you approach management of ILD in the presence of weakly positive RF and ANA but no other objective systemic findings of connective tissue disease?

Is this managed as CTD-ILD with immunosuppressive +/- anti-fibrotic agents?

1 Answer available

In a patient with low titer +anti-SAE antibody and known ILD, but no other clinical features of dermatomyositis, how would you approach further testing or would you treat the patient as dermatomyositis associated ILD?

1 Answer available

Would you add abatacept to treat active inflammatory arthritis in a patient with history of RA-ILD who is already taking mycophenolate?

1 Answer available

What criteria do you use to determine when to start a steroid-sparing agent for hypersensitivity pneumonitis?

1 Answer available

Would you consider adding or switching to pirfenidone for a patient with progressing UIP (based on imaging and PFTs) who is currently on nintedanib?

3 Answers available

How do you approach management of new onset ILD in a patient with RA who is otherwise well controlled on methotrexate or leflunomide?

1 Answer available

Would you initiate antifibrotic therapy in a patient with CTD-ILD experiencing worsening symptoms and declining lung function, despite no clear evidence of fibrosis on CT scans?

CT changes are subsolid and ground glass nodules.

1 Answer available

How do you approach the treatment of indeterminate UIP on HRCT with grossly positive MPO antibody and no other features consistent with AAV?

1 Answer available

Do you consider administration of nintedanib or pirfenidone via enteral tube in patients unable to take PO due to recurrent aspiration?

1 Answer available

How does the presence of interstitial lung disease affect your decision to offer SBRT for early stage NSCLC?

Specifically in O2-dependent patients? Have any dose/fractionation regimens been shown to reduce the risk of pneumonitis in this population?

6 Answers available

What dose and duration of steroid therapy do you employ while starting mycophenolate in an outpatient with relatively stable CTD-ILD?

Do you ever start mycophenolate without steroids?

1 Answer available

Do you routinely perform genetic testing in patients presenting with pulmonary fibrosis who endorse a family history of fibrotic lung disease?

1 Answer available

Do you ever consider close clinical monitoring over antifibrotic therapy in patients ascribed an MDD diagnosis of IPF who have normal lung function and are asymptomatic?

2 Answers available

How do you manage diarrhea in a patient with CTD-ILD on MMF who was recently started on full dose nintedanib?

5 Answers available

Is there a role for nintedanib in the management of patients with radiation-induced pulmonary fibrosis?

1 Answer available

How do you approach patients with RA and severe bronchiectasis with associated findings of UIP?

1 Answer available

Would you restart sirolimus for a decline in lung function or pneumothoraces in a pregnant patient with LAM?

1 Answer available

Is there an age at which you consider not starting antifibrotic therapy in a patient with IPF?

1 Answer available

Do you consider immunosuppressive agents in progressive interstitial pneumonias or pneumonitis without a clear driving etiology?

1 Answer available

What is your approach to steroid sparing therapy in patients with suspected CTD-ILD?

2 Answers available

What is your approach in differentiating IPAF (interstitial pneumonia with autoimmune features) versus "CTD ILD" in patients with serologic abnormalities but do not fulfill criteria for autoimmune disease?

1 Answer available

Do you ever initiate pirfenidone in patients with CTD-ILD?

1 Answer available

What is your approach to the management of a patient with idiopathic pneumonia syndrome following stem cell transplant who does not demonstrate clinical improvement with steroid therapy?

1 Answer available

When do you screen for pulmonary hypertension in ILD patients?

1 Answer available

What is your preferred steroid sparing agent in the treatment of Sjögren associated LIP?

2 Answers available

Would you consider the use of dual antifibrotic therapy (nintedanib and pirfenidone together) for progressive fibrosing ILD?

4 Answers available

Does your approach to treating latent tuberculosis differ in a patient on anti-fibrotic therapy?

1 Answer available

When do you begin antifibrotic therapy for a patient with newly diagnosed ILD that is not IPF?

Do you base the decision on FEV1, 6MWD or symptoms at initial evaluation, or progression over time?

6 Answers available

Do you screen for interstitial lung disease in patients with newly diagnosed polymyositis or dermatomyositis in the absence of respiratory symptoms?

3 Answers available

What dose and duration of steroid therapy do you use in steroid responsive ILD?

1 Answer available

Do you offer immunosuppression to patients with CTD-ILD with concomitant well controlled HIV?

2 Answers available

Does your approach to the management of a patient with an acute exacerbation of CPFE where the ILD is attributed to IPF differ from the management of a patient with an exacerbation of IPF alone?

1 Answer available

Is there a role of everolimus or capecitabine and temozolomide in diffuse idiopathic pulmonary neuroendocrine cell hyperplasia in patients already on octreotide?

1 Answer available

Do you prescribe antifibrotics to patients with combined pulmonary fibrosis and emphysema (CPFE)?

1 Answer available

Is there any PFT criteria that you use in guiding decision making to offer transbronchial lung cryobiopsy to patients with interstitial lung disease?

1 Answer available

What is your approach to the work up and management of interstitial lung abnormalities?

1 Answer available

What steroid regimen do you employ in the treatment of patients with post COVID-19 pulmonary fibrosis?

2 Answers available

Which images do you routinely request when ordering a HRCT chest?

1 Answer available

How do you counsel patients regarding prognosis following a diagnosis of IPF?

1 Answer available

Do you consider the use of antifibrotics at presentation in patients with a radiographic UIP pattern of pulmonary fibrosis in the presence of positive serologies without any symptoms of CTD who have not yet demonstrated evidence of a progressive phenotype?

1 Answer available

Is there a role for antifibrotic therapy in post COVID-19 pulmonary fibrosis?

1 Answer available

Papers discussed in this category

Clin Lung Cancer, 2017 Jul 10

Impact of Pretreatment Interstitial Lung Disease on Radiation Pneumonitis and Survival in Patients Treated With Lung Stereotactic Body Radiation Therapy (SBRT).

J Thorac Oncol,

Impact of pretreatment interstitial lung disease on radiation pneumonitis and survival after stereotactic body radiation therapy for lung cancer.

Clinical lung cancer, 2018-07

Fatal Radiation Pneumonitis in Patients With Subclinical Interstitial Lung Disease.

Int. J. Radiat. Oncol. Biol. Phys., 2017 Mar 15

Treatment-Related Toxicity in Patients With Early-Stage Non-Small Cell Lung Cancer and Coexisting Interstitial Lung Disease: A Systematic Review.

Radiotherapy and oncology : journal of the European Society for Therapeutic Radiology and Oncology, 2016-06

Pre-treatment non-target lung FDG-PET uptake predicts symptomatic radiation pneumonitis following Stereotactic Ablative Radiotherapy (SABR).

Journal of vascular and interventional radiology : JVIR, 2021 Mar 31

Effectiveness of Thermal Ablation and Stereotactic Radiotherapy Based on Stage I Lung Cancer Histology.

Annals of the rheumatic diseases, 2022 Nov 03

2022 EULAR recommendations for screening and prophylaxis of chronic and opportunistic infections in adults with autoimmune inflammatory rheumatic diseases.

Frontiers in immunology, 2021 Apr 12

Using Routine Laboratory Markers and Immunological Indicators for Predicting Pneumonia in Immunocompromised Patients.

Eur Respir Rev, 2021 Dec 15

Recovering from a pandemic: pulmonary fibrosis after SARS-CoV-2 infection.

Ann Am Thorac Soc,

Persistent Post-COVID-19 Interstitial Lung Disease. An Observational Study of Corticosteroid Treatment.

The European respiratory journal, 2015-10

An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features.

N Engl J Med, 2019 Sep 29

Nintedanib in Progressive Fibrosing Interstitial Lung Diseases.

American journal of respiratory and critical care medicine, 2011-02-15

Clinical course and prediction of survival in idiopathic pulmonary fibrosis.

Chest, 2011-07

Long-term course and prognosis of idiopathic pulmonary fibrosis in the new millennium.

The Lancet. Respiratory medicine, 2017-12

Palliative care in interstitial lung disease: living well.

J Heart Lung Transplant, 2021 Nov

Consensus document for the selection of lung transplant candidates: An update from the International Society for Heart and Lung Transplantation.

ERJ Open Res, 2019 Jul 08

Demographics and survival of patients with idiopathic pulmonary fibrosis in the FinnishIPF registry.

BMC Pulm Med, 2018 Jan 17

Barriers to timely diagnosis of interstitial lung disease in the real world: the INTENSITY survey.

Am J Respir Crit Care Med,

Nintedanib with Add-on Pirfenidone in Idiopathic Pulmonary Fibrosis. Results of the INJOURNEY Trial.

The European respiratory journal, 2018-08

Safety of nintedanib added to pirfenidone treatment for idiopathic pulmonary fibrosis.

The Lancet. Respiratory medicine, 2018-08

Analysis of protein-altering variants in telomerase genes and their association with MUC5B common variant status in patients with idiopathic pulmonary fibrosis: a candidate gene sequencing study.

Respir Med, 2011 Sep 13

Familial pulmonary fibrosis is the strongest risk factor for idiopathic pulmonary fibrosis.

Mayo Clin Proc, 2018 May 24

Short Telomere Syndromes in Clinical Practice: Bridging Bench and Bedside.

Respirology, 2021 Oct 06

Pulmonary fibrosis associated with telomere-related gene mutations: A complex inheritance.

Arthritis & rheumatology (Hoboken, N.J.), 2017-03

Progressive Decline of Lung Function in Rheumatoid Arthritis-Associated Interstitial Lung Disease.

N. Engl. J. Med., 2019 May 20

Nintedanib for Systemic Sclerosis-Associated Interstitial Lung Disease.

Lancet Respir Med, 2021 Jan

Efficacy and safety of nintedanib in patients with systemic sclerosis-associated interstitial lung disease treated with mycophenolate: a subgroup analysis of the SENSCIS trial.

Lancet Respir Med,

Interstitial lung abnormalities detected incidentally on CT: a Position Paper from the Fleischner Society.

Eur Respir Rev, 2022 Apr 13

Incidental discovery of interstitial lung disease: diagnostic approach, surveillance and perspectives.

N Engl J Med, 2012 May 20

Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis.

Chest, 2020 Aug 14

Clinical Characteristics of Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: A Retrospective Analysis.

Chest, 2020 Jul

Somatostatin Analogs Improve Respiratory Symptoms in Patients With Diffuse Idiopathic Neuroendocrine Cell Hyperplasia.

ERJ Open Res, 2020 Oct

Constrictive bronchiolitis in diffuse idiopathic pulmonary neuroendocrine cell hyperplasia.

Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases, 2022 Jun 11

Identification and Prognosis of Patients With Interstitial Pneumonia With Autoimmune Features.

European respiratory review : an official journal of the European Respiratory Society, 2021 Dec 22

Interstitial pneumonia with autoimmune features: challenges and controversies.

Eur Respir J, 2019 Apr 18

Therapeutic effects of nintedanib are not influenced by emphysema in the INPULSIS trials.

The Lancet. Respiratory medicine, 2016-09

Mycophenolate mofetil versus oral cyclophosphamide in scleroderma-related interstitial lung disease (SLS II): a randomised controlled, double-blind, parallel group trial.

Respirology, 2013 Nov 29

Rituximab in severe, treatment-refractory interstitial lung disease.

Respir Res, 2022 Jun 21

Effect size of rituximab on pulmonary function in the treatment of connective-tissue disease-related interstitial lung disease: a systematic review and meta-analysis.

European journal of internal medicine, 2023 Sep 04

Abatacept in usual and in non-specific interstitial pneumonia associated with rheumatoid arthritis.

Autoimmunity reviews, 2021 Apr 19

Efficacy and safety of abatacept in interstitial lung disease of rheumatoid arthritis: A systematic literature review.

J Infect Dis, 2020 Jun 11

Interstitial Lung Abnormalities in People With HIV Infection and Uninfected Controls.

Clin Chest Med, 2013 Apr 08

Interstitial lung disease in HIV.

Clin Med Insights Circ Respir Pulm Med, 2021 Mar 30

Delphi Consensus Recommendations on Management of Dosing, Adverse Events, and Comorbidities in the Treatment of Idiopathic Pulmonary Fibrosis with Nintedanib.

Cureus, 2022 Sep 06

Mycophenolate Mofetil-Induced Cytomegalovirus Colitis in a Patient With Polymyositis.

Ann Am Thorac Soc, 2017 May

Minimizing Tuberculosis Risk in Patients Receiving Anti-TNF Therapy.

Respiratory medicine, 2016-12

Azathioprine response in patients with fibrotic connective tissue disease-associated interstitial lung disease.

Chest, 2021 Feb

Consensus Guidelines for Evaluation and Management of Pulmonary Disease in Sjögren's.

N Engl J Med, 2021 Jan 13

Inhaled Treprostinil in Pulmonary Hypertension Due to Interstitial Lung Disease.

Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation, 2014-06

Randomized, double-blind, placebo-controlled trial of soluble tumor necrosis factor receptor: enbrel (etanercept) for the treatment of idiopathic pneumonia syndrome after allogeneic stem cell transplantation: blood and marrow transplant clinical trials network protocol.

Blood, 2015 Apr 27

Idiopathic pneumonia syndrome after hematopoietic cell transplantation: evidence of occult infectious etiologies.

American journal of respiratory and critical care medicine, 2020 May 15

Interstitial Lung Disease in Relatives of Patients with Pulmonary Fibrosis.

BMJ open respiratory research, 2021 Jul

Fine crackles on chest auscultation in the early diagnosis of idiopathic pulmonary fibrosis: a prospective cohort study.

American journal of respiratory and critical care medicine, 2019 Jul 15

Clinical Effectiveness of Antifibrotic Medications for Idiopathic Pulmonary Fibrosis.

PloS one, 2022 Feb 03

Tolerability of nintedanib in the elderly with idiopathic pulmonary fibrosis: A single-center retrospective study.

Respiration; international review of thoracic diseases, 2022 Nov 10

Observational, Multicenter Study on the Efficacy, Tolerability, and Safety of Nintedanib in Patients with Idiopathic Pulmonary Fibrosis Older than 80 Years.

European respiratory review : an official journal of the European Respiratory Society, 2021 Nov 08

Antineutrophil cytoplasmic antibody-associated interstitial lung disease: a review.

Orphanet journal of rare diseases, 2021 Mar 17

Pregnancy after the diagnosis of lymphangioleiomyomatosis (LAM).

International journal of radiation oncology, biology, physics, 2023 Mar 07

Randomized Phase 2 Placebo-Controlled Trial of Nintedanib for the Treatment of Radiation Pneumonitis.

Lancet (London, England), 2011-05-21

Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials.

The New England journal of medicine, 2018 Aug 26

Effects of Aspirin for Primary Prevention in Persons with Diabetes Mellitus.

Respiratory research, 2021 Apr 26

Efficacy and safety of nintedanib in patients with idiopathic pulmonary fibrosis who are elderly or have comorbidities.

The New England journal of medicine, 2014 May 18

Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis.

Respiratory investigation, 2020 Sep 03

Tolerability and safety of nintedanib in elderly patients with idiopathic pulmonary fibrosis.

Advances in therapy, 2023 Mar 16

A Narrative Review of Real-World Data on the Safety of Nintedanib in Patients with Idiopathic Pulmonary Fibrosis.

American journal of respiratory and critical care medicine, 2011-10-01

Delayed access and survival in idiopathic pulmonary fibrosis: a cohort study.

American journal of respiratory and critical care medicine, 2023 Jan 01

Antifibrotics and Reduced Mortality in Idiopathic Pulmonary Fibrosis: Immortal Time Bias.

Rheumatology (Oxford, England), 2021 Dec 24

Abatacept in monotherapy vs combined in interstitial lung disease of rheumatoid arthritis-multicentre study of 263 Caucasian patients.

PloS one, 2024 Jun 13

Tolerability and efficacy of switching anti-fibrotic treatment from nintedanib to pirfenidone for idiopathic pulmonary fibrosis.

Journal of the Endocrine Society, 2022 Dec 02

Perioperative Evaluation and Management of Patients on Glucocorticoids.

Diagnostics (Basel, Switzerland), 2021 Apr 20

Glucocorticoid Withdrawal-An Overview on When and How to Diagnose Adrenal Insufficiency in Clinical Practice.

Chest, 2017-03

Use of Mycophenolate Mofetil or Azathioprine for the Management of Chronic Hypersensitivity Pneumonitis.

ERJ open research, 2017-07

Outcomes of immunosuppressive therapy in chronic hypersensitivity pneumonitis.

Chest, 2023 Dec 19

Impact of Antigen Exposure on Outcomes and Treatment Response in Fibrotic Hypersensitivity Pneumonitis.

The European respiratory journal, 2023 Nov 29

Telomere length and immunosuppression in non-idiopathic pulmonary fibrosis interstitial lung disease.

Rheumatology (Oxford),

The reliability of immunoassays to detect autoantibodies in patients with myositis is dependent on autoantibody specificity.

Rheumatology (Oxford, England), 2023 Dec 01

Anti-SAE autoantibody in dermatomyositis: original comparative study and review of the literature.

Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG, 2004-03

Leflunomide for chronic sarcoidosis.

Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG, 2000-03

Methotrexate is steroid sparing in acute sarcoidosis: results of a double blind, randomized trial.

Chest, 2003-07

Etanercept for the treatment of stage II and III progressive pulmonary sarcoidosis.

American journal of respiratory and critical care medicine, 2006-10-01

Infliximab therapy in patients with chronic sarcoidosis and pulmonary involvement.

Diagnostics (Basel, Switzerland), 2024 Jan 23

A Retrospective Analysis of 2-Year Follow-Up of Patients with Incidental Findings of Sarcoidosis.

Chest, 1999 Aug

Oral prednisolone followed by inhaled budesonide in newly diagnosed pulmonary sarcoidosis: a double-blind, placebo-controlled multicenter study. Finnish Pulmonary Sarcoidosis Study Group.

Respiration; international review of thoracic diseases, 2012 Dec 29

Effect and safety of mycophenolate mofetil in chronic pulmonary sarcoidosis: a retrospective study.

The Lancet. Respiratory medicine, 2024 Jan 15

Morphine for treatment of cough in idiopathic pulmonary fibrosis (PACIFY COUGH): a prospective, multicentre, randomised, double-blind, placebo-controlled, two-way crossover trial.

NEJM evidence, 2023 May 22

Nalbuphine Tablets for Cough in Patients with Idiopathic Pulmonary Fibrosis.

The Lancet. Respiratory medicine, 2013-07

Anti-acid treatment and disease progression in idiopathic pulmonary fibrosis: an analysis of data from three randomised controlled trials.

The Lancet. Respiratory medicine, 2018-09

Laparoscopic anti-reflux surgery for the treatment of idiopathic pulmonary fibrosis (WRAP-IPF): a multicentre, randomised, controlled phase 2 trial.

The European respiratory journal, 2006 Jan

High prevalence of abnormal acid gastro-oesophageal reflux in idiopathic pulmonary fibrosis.

Chest, 2005-01

Analyses of efficacy end points in a controlled trial of interferon-gamma1b for idiopathic pulmonary fibrosis.

American journal of respiratory and critical care medicine, 2002 Jan 15

American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001.

Clinics in chest medicine, 2004 Dec

Cryptogenic organizing pneumonia.

The European respiratory journal, 2020 Aug 06

Bronchoalveolar lavage fluid lymphocytosis in chronic hypersensitivity pneumonitis: a systematic review and meta-analysis.

American journal of respiratory and critical care medicine, 2020 Aug 01

Diagnosis of Hypersensitivity Pneumonitis in Adults. An Official ATS/JRS/ALAT Clinical Practice Guideline.

American journal of respiratory and critical care medicine, 2019-11-01

Rare Protein-Altering Telomere-related Gene Variants in Patients with Chronic Hypersensitivity Pneumonitis.

The European respiratory journal, 2021 Mar 04

Leukocyte telomere length and mycophenolate therapy in chronic hypersensitivity pneumonitis.

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