pulmonary evaluation of
play

Pulmonary Evaluation of Brief background of sarcoidosis - PDF document

Overview Pulmonary Evaluation of Brief background of sarcoidosis Demographics Sarcoidosis Etiologies Pulmonary manifestations and complications Laura L. Koth, MD Professor of Medicine, UCSF Director, Sarcoidosis Research Program


  1. Overview Pulmonary Evaluation of ❑ Brief background of sarcoidosis ❑ Demographics Sarcoidosis ❑ Etiologies ❑ Pulmonary manifestations and complications Laura L. Koth, MD Professor of Medicine, UCSF Director, Sarcoidosis Research Program Robert L. Kroc Chair in Rheumatic and Connective Tissue Diseases Brief Background Demographics ◼ Any race and gender ◼ Inflammatory disease ◼ Highest incidence reported in ❑ Granulomatous infiltrates in tissues ❑ African Americans ❑ Clinical manifestations ≈ organs involved ❑ Northern Europeans ❑ Women > men ◼ No single diagnostic test ◼ Onset 30-50 yrs ◼ 90% of patients have pulmonary involvement 30% over 50 years of age (ACCESS study: Baughman, AJRCCM, 2001) ❑ ◼ Prevalence: U.S. Optum health care database* ❑ U.S. whites (~50 per 100,000) ❑ U.S. blacks (~140 per 100,000) *Baughman, R. P., et al Ann.Am.Thorac.Soc. 2016; 13:1244-1252 1

  2. Pulmonary Symptoms Etiology: Antigen Trigger? Arm injection ◼ 1938 debate: tuberculosis vs. lepra bacilli, leishmania ◼ Dyspnea or spectrum of Hodgins lymphoma ❑ slowly progressive ◼ No organism ever cultured ◼ Dry cough ◼ Chest pain ◼ Nickerson-Kveim Reagent Arm biopsy: granulomas ❑ Developed in 1935 ❑ “suspension of sarcoid - involved spleen or lymph node” ◼ Other common symptoms ❑ “injected into the skin” ❑ Fatigue ❑ Dermal reactions at injection site weeks later = positive ❑ Used to diagnose patients suspected of sarcoidosis Siltzbach, L.E., 1954 Am J Med Pathogenesis of Sarcoidosis: Paradigm Mycobacteria Tuberculosis as a Cause of Sarcoidosis? ENVIRONMENT: Antigenic or GENE: “ Sarcoidoses ” inflammatory susceptible Granulomas trigger (e.g. 9/11 ◼ Possible host WTC) ❑ Likely only accounts for Chronic Resolution/ a fraction of cases inflammation +/- Lung/LN Paraneoplastic Drug-induced Repair Fibrosis ◼ Autoimmne disease Other organs ❑ acute form Skin Bone ◼ Lung involvement may Liver, Spleen Regional Extrathoracic Lofgren’s be a clue ≈ inhalational Heart exposure? 2

  3. Criteria for Sarcoidosis Diagnosis ◼ Compatible clinical picture ❑ ~90% pulmonary disease ◼ Non-necrotizing granulomas ◼ Exclusion of other diseases ❑ Lymphoma, tuberculosis, histoplasmosis, berylliosis, amyloidosis, metastatic cancer, silicosis ❑ Rarely lymphomatoid granulomatosis ATS/ERS/WASOG. Am J Respir Crit Care Med. 1999 Chest CT findings: Mediastinal and Bilateral CT Findings: Hilar Lymphadenopathy (BHL) BHL with Parenchymal Nodules Transverse section ◼ Distribution: peri-lymphatic nodules, upper lobe coronal section 2010 2016 3

  4. CT Findings: Fibrosis Biopsy: Options ◼ Bronchoscopy with E ndo B ronchial U ltra S ound (EBUS) ❑ Systematic review: symptomatic and asymptomatic bilateral hilar lymphadenopathy ◼ sarcoidosis ≈72% (95% CI 61 -81%) Mycetoma ◼ lymphoma ≈ 10% (95% CI 5.3 -19%) ◼ other diagnoses (silicosis, fibrosis, and amyloidosis) ≈ 7.7% (95% CI 3.6-15.8%). ◼ Lymph node diameter > 0.5 cm ◼ Transbronchial biopsy ◼ Mediastinoscopy with lymph node dissection Role of body 18-fluorodeoxyglucose Example of #2 PET CT scan ◼ Diagnosis of sarcoidosis in remote past ◼ Chest CT appears fibrotic ◼ #1 ◼ Would patient benefit from 12 months of ❑ May identify organs (e.g. lymph immunosuppression? nodes) accessible to biopsy ◼ #2 ❑ cases of end stage fibrosis ❑ Assess if “active” granulomatous inflammation present when considering immunosuppression 4

  5. Monitoring: essential Take Home Points ◼ When considering Sarcoidosis as a diagnosis ◼ Still do not have biomarkers to predict who will have progressive inflammation and/or fibrosis ❑ Get a good chest CT ◼ Contrast helps delineate lymph nodes but experienced ◼ Radially expanding peri-bronchiolar fibrosis radiologists do not need it ❑ Involve pulmonary specialists early At diagnosis 5 years later ❑ Push for lung and/or lymph node biopsies ❑ Bronchoscopy with Endobronchial ultrasound biopsies of lymph nodes by experienced proceduralist ❑ No single diagnostic test ◼ Make sure your sarcoidosis patients are monitored Sarcoidosis Listserv for Clinicians and Researchers THANK YOU FOR YOUR ◼ To join, send an e-mail to: ATTENTION! ◼ sarcoid-list@uiowa.edu ◼ OR ◼ AASOG Website: www.aasog.net ◼ http://aasog.net/physician-resources/sarcoidosis- listserv/ 5

Download Presentation
Download Policy: The content available on the website is offered to you 'AS IS' for your personal information and use only. It cannot be commercialized, licensed, or distributed on other websites without prior consent from the author. To download a presentation, simply click this link. If you encounter any difficulties during the download process, it's possible that the publisher has removed the file from their server.

Recommend


More recommend