What you need to know about Scleroderma and Lung Disease - Dr. Selvan
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Interstitial lung disease (ILD) is a common complication of scleroderma that leads to inflammation and scarring of the lungs. In this session, we will review the prevalence of scleroderma-associated ILD (SSc-ILD), classic symptoms, and the approach to evaluating patients with suspected disease. In addition, we will cover various treatments available for patients with SSc-ILD. This talk was presented at the Scleroderma Patient Education Conference on May 4, 2024, hosted by the Scleroderma Foundation of Greater Chicago. For more info about scleroderma and the foundation, head to www.stopscleroderma.org
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What you need to know about Scleroderma and Lung Disease - Dr. Selvan
- 1. Scleroderma and Interstitial Lung Disease May 4, 2024 Kavitha Selvan, MD Clinical Instructor of Medicine Section of Pulmonary and Critical Care Medicine University of Chicago
- 2. Disclosures I have no financial disclosures. 2
- 3. Outline 1. What is interstitial lung disease (ILD)? 2. Who is at risk for developing ILD? 3. What are the signs and symptoms of ILD? 4. What type of testing is required to diagnose and monitor ILD? 5. How is scleroderma-associated ILD treated? 3
- 4. What is interstitial lung disease (ILD)? 4
- 5. Interstitial Lung Disease (ILD) Family of over 200 different diseases that lead to inflammation and/or scarring of the lungs Scarring referred to as “pulmonary fibrosis” Can affect the walls of the air sacs (alveoli), the tissue, and the space around the air sacs (interstitium) 5
- 6. Who is at risk for developing ILD? 6
- 7. Causes of ILD Interstitial Lung Disease (ILD) Etiology known Inhaled exposures Asbestos, Silica, Coal dust, Birds, Mold, Hay Drugs/Radiation Nitrofurantoin, Amiodorone, Methotrexate, Chemotherpy, Radiation Autoimmune disease Scleroderma, Rheumatoid arthritis, Sjogren’s syndrome, Myositis,Vasculitis, Lupus Genetic mutations Etiology unknown (Idiopathic) 7
- 8. Scleroderma and ILD (SSc-ILD) Between 35-53% of patients with scleroderma develop ILD Lung = 2nd most affected organ Risk factors for developing ILD: Male gender Older age at time of diagnosis African American race Diffuse cutaneous form of scleroderma (vs. limited) Positive anti-Scl-70 antibody Negative anti-centromere antibody Cottin V, et al. Respir Res 2019;20:13. Khanna D. et al. J Scleroderma Relat Disord 2022;7(3):168-78. 8
- 9. Scleroderma and ILD (SSc-ILD) Disease course can be variable Stable disease over time (~1/3 patients) Disease progression, followed by stability Progressive disease Leading cause of morbidity in scleroderma Outcomes worse with fibrotic patterns Outcomes better than idiopathic pulmonary fibrosis (IPF) Hoffmann-Vold AM, et al. Arthritis Rheumatol 2018; 70: A799. Early diagnosis and prompt initiation of treatment is KEY! 9
- 10. Scleroderma and ILD (SSc-ILD) Most likely to progress within the first 5 years following disease onset Risk factors for disease progression: Gastroesophageal reflux disease (GERD) Greater than 20% fibrosis on HRCT Decreased lung function at baseline Reduced SpO2 at baseline Positive anti-Scl-70 antibody Hoffmann-Vold AM, et al. Arthritis Rheumatol 2018; 70: A799. 10
- 11. What are the signs and symptoms of ILD? 11
- 12. Signs of ILD Low oxygen levels Digital clubbing Pulmonary crackles 12
- 13. Symptoms of ILD Most common = shortness of breath and dry cough May be mild or absent in early disease Often occurs with exertion Fatigue Weakness Chest discomfort Loss of appetite Weight loss Some patients are asymptomatic! 13
- 14. DO NOT IGNORE YOUR SYMPTOMS!! Diagnosis is delayed by 1-2 years in over half of patients Delays caused by: Symptoms being minimized Misdiagnosis Lack of reporting of early imaging findings Delay in referral Rahman KKM, et al. Eur Respir J 2016;48:PA861. Pritchard D, et al. Respir Res 2019;20:253. 14
- 15. Time Lost is Lung Lost 15
- 16. What type of testing is required to diagnose and monitor ILD? 16
- 17. Diagnostic Testing for ILD Pulmonary function tests High-resolution CT (HRCT) chest +/- Bronchoscopy and/or tissue biopsy 17
- 18. Diagnostic Testing for ILD Pulmonary function tests High-resolution CT (HRCT) chest +/- Bronchoscopy and/or tissue biopsy 18
- 20. Diagnostic Testing for ILD Pulmonary function tests High-resolution CT (HRCT) chest +/- Bronchoscopy and/or tissue biopsy 20
- 21. High-resolution CT Chest (HRCT) Most sensitive, non-invasive test for diagnosing SSc-ILD Obtain prone (left), supine (right), and expiratory images Considerations: Radiation exposure (0.2-0.98 mSv) 21
- 23. Scleroderma- ILD (SSc-ILD) INFLAMMATION “Ground-glass opacities” or “GGOs” Image source: Chung JH, et al. J Vis Exp 2020. 23
- 24. Scleroderma- ILD (SSc-ILD) FIBROSIS “Traction bronchiectasis” and “honeycombing” or “honeycomb cysts” Airway Blood vessel Image source: Chung JH, et al. J Vis Exp 2020. 24
- 25. Scleroderma- ILD (SSc-ILD) INFLAMMATION & FIBROSIS “Reticulation” or “reticular opacities” Image source: Chung JH, et al. J Vis Exp 2020. 25
- 26. Diagnostic Testing for ILD Pulmonary function tests High-resolution CT (HRCT) chest +/- Bronchoscopy and/or tissue biopsy 26
- 27. Bronchoscopy +/- Tissue Biopsy Image source: drugs.com 27
- 28. How is scleroderma- associated ILD treated? 28
- 29. Treatment Immunosuppression Anti-fibrotic medications Supplemental oxygen Pulmonary rehabilitation 29
- 30. Treatment Immunosuppression Anti-fibrotic medications Supplemental oxygen Pulmonary rehabilitation 30
- 31. Immuno- suppression Raghu G, et al. Am J Respir Crit Care Med 2024;209(2):137-152. When should treatment be initiated? 31
- 32. Mycophenolate Mofetil (CellCept) Dosing: 500mg twice daily, increase up to 1500mg twice daily Side effects: Diarrhea, infection, skin cancer Monitoring: Blood counts, liver function tests (every 3 months) Tashkin DP, et al. Lancet Respir Med 2016; 4:708-719. • Improved lung function at 2-years • Better tolerated and improved survival vs. cyclophosphamide (SLS-II, 2016) 32
- 33. Rituximab Meta-analysis of 3 available trials found: Reduced decline in FVC by 3.3% vs. placebo at 24-48 weeks No significant difference in survival Dosing: 1000 mg injections on days 1 and 15, every 6 months Side effects: Infection (reactivation of Hep B), infusion reaction, progressive multifocal encephalopathy (PML) Monitoring: Screen for Hep B, Blood counts prior to infusions and monthly Raghu G, et al. Am J Respir Crit Care Med 2024;209(2):137-152. 33
- 34. Tocilizumab Improved lung function at 2-years (focuSSced Trial, 2020) Dosing: 162 mg injection weekly Side effects: Diverticulitis/GI perforation, infection, hepatitis, anaphylaxis Monitoring: Screen for TB, Blood counts and liver function tests every 1-2 months x6 months, followed by every 3 months Khanna D, et al. Lancet Respir Med 2020;8:963-74 34
- 35. Treatment Immunosuppression Anti-fibrotic medications Supplemental oxygen Pulmonary rehabilitation 35
- 36. Anti-fibrotic Medications Nintedanib – SENSCIS Trial (2019) Reduced annual rate of FVC decline vs. placebo Pirfenidone – currently not approved, requires further investigation Distler O, et al. NEJM 2019;380(6):2518-2528. Raghu G, et al. Am J Respir Crit Care Med 2024;209(2):137-152. 36
- 37. Treatment Immunosuppression Anti-fibrotic medications Supplemental oxygen Pulmonary rehabilitation 37
- 38. Supplemental Oxygen Monitor resting and exertional oxygen levels with 6-minute walk test (6MWT) Initiate supplemental oxygen when resting SpO2 < 90% - may improve survival! Consider if ambulatory SpO2 < 88% Improves quality of life and breathlessness (AmbOx Trial, 2018) Visca D, et al. Lancet Respir Med 2018; 6: 759–770. Jacobs SS, et al. Am J Respir Crit Care Med 2020; 202: e121–e141. 38
- 39. Treatment Immunosuppression Anti-fibrotic medications Supplemental oxygen Pulmonary rehabilitation 39
- 40. Pulmonary Rehabilitation Supervised program that includes: Breathing techniques Exercise training: High intensity exercises >> low intensity Health education Psychological counseling Nutritional counseling 2-3 sessions per week for 1-3 months https://www.nhlbi.nih.gov/health-topics/pulmonary-rehabilitation 40
- 41. Benefits of Pulmonary Rehabilitation Increased walk distance (40 meters in 6 minutes) Decreased shortness of breath Improved quality of life Benefits often persist 6-12 months after completion of rehab! Dowman L et al. Cochrane Database of Systematic Reviews 2021, Issue 2. Art. No.: CD006322. 41
- 42. Additional Therapies Management of co-existing medical conditions Tobacco use disorder Obesity Congestive heart failure Immunizations Pneumococcal vaccine Seasonal influenza COVID-19 42
- 43. Questions? Thank you to the Scleroderma Foundation of Greater Chicago! Patient resources: Pulmonary Fibrosis Foundation (PFF): pulmonaryfibrosis.org Contact information: Kavitha Selvan, MD Clinical Instructor of Medicine Section of Pulmonary and Critical Care Medicine University of Chicago Email: Kavitha.Selvan@uchicagomedicine.org 43
Editor's Notes
- Think of inflammation as two ”flavors” of ILD – we’ll talk more about these flavors later.
- Other main pulmonary manifestation of scleroderma is pulmonary hypertension, or elevated pressures of the pulmonary arteries, which affects 8-12% of patients
- Data from largest scleroderma database (European Scleroderma Trials and Research (EUSTAR) Group) Subsequent study found that of those with initial progression in first year, half had further progression over 5 years Median survival reported between 5-8 years (vs. >10 years for overall scleroderma population)
- Data from largest scleroderma database (European Scleroderma Trials and Research (EUSTAR) Group) Subsequent study found that of those with initial progression in first year, half had further progression over 5 years
- Clubbing is a non-specific sign that can occur in other diseases of the lungs, heart, and liver
- Non-specific nature of these symptoms make diagnosis a challenge Can be mistaken for other manifestations of scleroderma, such as cardiac disease or musculoskeletal involvement – important to have a high index of suspicion!
- Typically performed in a lab or office Takes up to 1 hr to perform and can be quite difficulty, physically! Requires being able to blow out for > 6 seconds and a 10-second breath hold Results vary based on patient effort Monitored every 3-6 months in patients with ILD
- Radiation associated with HRCT = .2-.9 mSv 6.5 times less than standard CT scan, 12x more than CXR
- Monitor every 1-2 years, or sooner if clinical symptoms or lung function declines, other abnormalities (like pulmonary nodules) that need to be monitored more closely, or other reasons for closer scans (such as yearly lung cancer screening in those that are current smokers)
- Performed in a procedure suite by a pulmonologist – outpatient procedure, typically go home the same day Requires either IV sedation or general anesthesia, depending on what will be done during the procedure Once scope is inserted into the lungs, you can take washings of the lungs to analyze, or small pieces of tissue, or biopsies Previously was the gold standard diagnostic test for diagnosing and determining the cause of ILD Now, advanced in CT allow us to make the diagnosis with imaging alone in many cases – preventing the need for invasive procedures in many patients (particularly those with scleroderma ILD, where there is typically high certainty on the cause of the ILD) Still utilized in cases where other diagnoses may need to be ruled-out, such as co-existing infection on top of existing ILD
- Clinical Practice Guideline from the ATS – reviewed all the available evidence on different treatment modalities used in Ssc-ILD and provided evidence-based recommendations for clinicians Initiating therapy depends on disease severity (mild/non-progressive vs. moderate/severe or progressive): Minimize risk factors and monitor PFTs/6MWT q3-6 months vs. initiate treatment immediately with medications, O2, pulmonary rehab, GERD treatment
- Teratogen – avoid in pregnancy
- 2/3 trials were not specific to scleroderma-ILD PML: Neuro-infection due to JC virus
- Teratogen – avoid in pregnancy
- Nintedanib – small molecule inhibitor that binds to tyrosine kinase receptors and inhibits the production of growth factors, such as VEGF - 50% of patients in SENSCIS trial (in both arms) were receiving MMF - Nintedanib also approved for PPF (NEJM 2019) – slows annual rate of FVC decline Pirfenidone – MOA not fully understood, reduces production of mediators of fibrosis, such as TGF-beta
- Guidelines on long-term O2 therapy (>15 hours/day) extrapolated from COPD trials which have shown improved survival in patients randomized to therapy Despite limited evidence in ILD, all major guidelines, including ATS, recommend therapy for severe hypoxemia or moderate with evidence of end-organ hypoxia Myths: O2 causes fires (no – doesn’t cause, can just support a flame) Common side effects: nose bleed, nasal dryness, headaches – can used water based moisturizers to help with dryness and order humidifier for home O2 (but must use distilled water)