Pulmonary Evaluation of Brief background of sarcoidosis - - PDF document

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Feb 15, 2024 395 likes •461 views

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

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Pulmonary Evaluation of Sarcoidosis

Laura L. Koth, MD Professor of Medicine, UCSF Director, Sarcoidosis Research Program Robert L. Kroc Chair in Rheumatic and Connective Tissue Diseases

Overview

❑ Brief background of sarcoidosis ❑ Demographics ❑ Etiologies ❑ Pulmonary manifestations and complications

Brief Background

◼ Inflammatory disease

❑ Granulomatous infiltrates in tissues ❑ Clinical manifestations ≈ organs involved

◼ No single diagnostic test ◼ 90% of patients have pulmonary involvement

Demographics

◼ Any race and gender ◼ Highest incidence reported in

❑ African Americans ❑ Northern Europeans ❑ Women > men

◼ Onset 30-50 yrs

❑

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

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Pulmonary Symptoms

◼ Dyspnea

❑ slowly progressive

◼ Dry cough ◼ Chest pain ◼ Other common symptoms

❑ Fatigue

Etiology: Antigen Trigger?

◼ 1938 debate: tuberculosis vs. lepra bacilli, leishmania

r spectrum of Hodgins lymphoma

◼ No organism ever cultured ◼ Nickerson-Kveim Reagent

❑ Developed in 1935 ❑ “suspension of sarcoid-involved spleen or lymph node” ❑ “injected into the skin” ❑ Dermal reactions at injection site weeks later = positive ❑ Used to diagnose patients suspected of sarcoidosis

Arm injection Arm biopsy: granulomas Siltzbach, L.E., 1954 Am J Med

Pathogenesis of Sarcoidosis: Paradigm

Granulomas GENE: susceptible host ENVIRONMENT: Antigenic or inflammatory trigger (e.g. 9/11 WTC) Resolution/ Repair Chronic inflammation +/- Fibrosis Other

rgans

Lung/LN Skin Heart Bone Liver, Spleen

Mycobacteria Tuberculosis as a Cause of Sarcoidosis?

◼ Possible

❑ Likely only accounts for

a fraction of cases

◼ Autoimmne disease

❑ acute form

◼ Lung involvement may

be a clue ≈ inhalational exposure?

Paraneoplastic Drug-induced Regional Lofgren’s Extrathoracic “Sarcoidoses”

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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

2016 2010

Chest CT findings: Mediastinal and Bilateral Hilar Lymphadenopathy (BHL)

Transverse section coronal section

CT Findings: BHL with Parenchymal Nodules

◼ Distribution: peri-lymphatic nodules, upper lobe

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CT Findings: Fibrosis

Mycetoma

Biopsy: Options

◼ Bronchoscopy with EndoBronchial UltraSound

(EBUS)

❑ Systematic review: symptomatic and asymptomatic

bilateral hilar lymphadenopathy

◼ sarcoidosis ≈72% (95% CI 61-81%) ◼ 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 PET CT scan

◼ #1

❑ May identify organs (e.g. lymph

nodes) accessible to biopsy

◼ #2

❑ cases of end stage fibrosis ❑ Assess if “active” granulomatous

inflammation present when considering immunosuppression

Example of #2

◼ Diagnosis of sarcoidosis in remote past ◼ Chest CT appears fibrotic ◼ Would patient benefit from 12 months of

immunosuppression?

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Monitoring: essential

◼ Still do not have biomarkers to predict who will

have progressive inflammation and/or fibrosis

◼ Radially expanding peri-bronchiolar fibrosis

At diagnosis 5 years later

Take Home Points

◼ When considering Sarcoidosis as a diagnosis

❑ Get a good chest CT ◼ Contrast helps delineate lymph nodes but experienced

radiologists do not need it

❑ Involve pulmonary specialists early ❑ Push for lung and/or lymph node biopsies ❑ Bronchoscopy with Endobronchial ultrasound biopsies

f lymph nodes by experienced proceduralist

❑ No single diagnostic test

◼ Make sure your sarcoidosis patients are

monitored

THANK YOU FOR YOUR ATTENTION!

Sarcoidosis Listserv

for Clinicians and Researchers

◼ To join, send an e-mail to:

◼ sarcoid-list@uiowa.edu

◼ OR ◼ AASOG Website: www.aasog.net

◼ http://aasog.net/physician-resources/sarcoidosis-

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