Patients with COVID-19 who have underlying chronic lung disease are at increased risk for developing severe illness related to the disease. However, to date, few studies have been published about patients with pre-existing interstitial lung disease (ILD) or pulmonary sarcoidosis.
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Brian Southern, MD, a pulmonologist in Cleveland Clinic’s Respiratory Institute, recently authored a review article in Cleveland Clinic Journal of Medicine’s COVID-19 Curbside Consults series. In this article, he takes a closer look at what is known about ILD and sarcoidosis in the context of the pandemic and offers insights for managing care.
Bigger picture insights: ILD and COVID-19
The pathologic picture. The relationship between ILD and COVID-19 has not been well-established; however, an early pathology report of a patient who died of respiratory complications related to COVID-19 showed lung injury that was characteristic of diffuse alveolar damage in acute respiratory distress syndrome.1 These traits — desquamation of pneumocytes, hyaline membrane formation and pulmonary edema — are also present in acute ILD, suggesting a similar disease pathology.
A trigger of acute ILD exacerbations? Although the exact mechanism of acute exacerbation in ILD has not been elucidated, infectious and idiopathic pathologies may be involved.2 There is also speculation that respiratory infection with COVID-19 could trigger an exacerbation of existing ILD, potentially resulting in worse outcomes in these patients. There is a similar concern for patients with sarcoidosis, particularly those with fibrotic manifestations. 3
Utilizing virtual care in the COVID-19 era. A timely diagnosis of ILD and well-managed care are essential to improved outcomes in these patients. The pandemic may have shifted plans for care, making in-office appointments unfeasible. Thus, Dr. Southern encourages providers to utilize virtual appointments and telephone visits and rely on patient-reported outcomes to adjust therapies as needed during the pandemic.
Considerations for managing your patient’s care
Dr. Southern offers the following considerations when developing a care pathway for patients with pre-existing ILD and pulmonary sarcoidosis who have contracted the virus.
Consider a low-threshold for hospital admission and a potential drug regimen. Patients with underlying ILD or pulmonary sarcoidosis are at higher risk for developing severe COVID-19-related complications that require supportive care, similar to that of ARDS. Additionally, a drug regimen may be appropriate. The NIH now recommends the investigational antiviral agent remdesivir for patients hospitalized with the disease through the FDA’s emergency use authorization. Hospital admission and treatment plan should be developed in partnership with the infectious disease team.
Use clinical judgment when it comes to managing steroid use. Generally, steroid use in COVID-19 patients is not recommended and has not been supported by the WHO, except in special cases such as exacerbation of asthma, COPD, or septic shock. 4 However, Dr. Southern notes, steroids should be considered in cases of ILD related to exacerbation of underlying connective tissue disease, which may involve more organizing pneumonia or alveolar hemorrhage.
Continue use of immunosuppressive therapy. Patients with ILD or pulmonary sarcoidosis are managed with chronic immunosuppressive therapy and should continue according to the current standard of care. Although the CDC lists it as a risk factor for progression to more severe COVID-19-related illness, transplant literature suggests that unlike other community-acquired viruses (i.e. influenza, adenovirus, or rhinovirus), coronaviruses are not linked to worse outcomes in patients receiving immunosuppressive treatments. 5
No rationale to discontinue anti-fibrotic drugs. Pirfenidone and nintedanib are used to treat idiopathic pulmonary fibrosis. Findings from recent SENCSIS and INBUILD trials showed that nintedanib can be extended to patients with pulmonary fibrosis secondary to systemic sclerosis and in all forms of progressive fibrosing ILD.6-7 Unless complications linked to liver or kidney failure arise, discontinued use of these drugs in COVID-19 patients is unwarranted.
Avoid mechanical ventilation if possible. Dr. Southern recommends that providers consider avoiding mechanical ventilation and opt for palliative measures in patients who are positive for COVID-19 and who are experiencing an acute exacerbation of ILD. Recent evidence suggests that the mortality rate for ventilated COVID-19 patients may be between 16%-25%. 8-9 This coupled with the already high risk of mortality in ILD patients who are on ventilation should be considered when developing a plan for care.
Long-term implications of COVID-19
At this point, the long-term implications of COVID-19 on lung disease are not well-described. There is evidence from survivors of SARS and MERS patients that suggests lung damage and pulmonary fibrosis may lead to a reduced quality of life and disability. Given the mechanistic and pathological similarities among these diseases, it’s reasonable to suspect some survivors of COVID-19 may also experience these longer-term outcomes.
Read the complete article from Dr. Southern and a full list of references by visiting Cleveland Clinic’s Journal of Medicine’s Curbside Consult series.
- Xu Z, Wang Y, et al. Pathological findings of COVID-19 associated with acute respiratory distress syndrome. Lancet Respir Med. 2020; 8(4):420–422.
- Kreuter M, Polke M, Walsh SLF, et al. Acute exacerbation of idiopathic pulmonary fibrosis: international survey and call for harmonisation. Eur Respir J. 2020; 55(4):1901760
- Panselinas E, Judson MA. Acute pulmonary exacerbations of sarcoidosis. Chest. 2012; 142(4):827–836.
- World Health Organization. Clinical management of COVID-19. Updated May 27, 2020. Accessed June 12, 2020.
- D’Antiga L. Coronaviruses and immunosuppressed patients: the facts during the third epidemic. Liver Transpl. 2020; 26(6):832–834.
- Distler O, Highland KB, Gahlemann M, et al. Nintedanib for systemic sclerosis-associated interstitial lung disease. N Engl J Med. 2019; 380(26):2518–2528.
- Flaherty KR, Wells AU, Cottin V, et al. Nintedanib in progressive fibrosing interstitial lung diseases. N Engl J Med. 2019; 381(18):1718–1727
- Ziehr DR, Alladina J, Petri CR, et al. Respiratory pathophysiology of mechanically ventilated patients with COVID-19: a cohort study [published online ahead of print April 29, 2020]. Am J Respir Crit Care Med. 2020; 10.1164/rccm.202004-1163LE.
- Richardson S, Hirsch JS, Narasimhan M, et al. Presenting characteristics, comorbidities, and outcomes among 5700 patients hospitalized with COVID-19 in the New York City area. JAMA. 2020; 323(20):2052–2059.