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 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

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

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

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

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