Q&A: Early diagnosis, optimal treatment key for patients with pulmonary sarcoidosis
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A new review published in JAMA highlights the current diagnosis and treatment of pulmonary sarcoidosis.
The review authors performed online searches of PubMed and OVID from 2015 to 2021 and identified 87 randomized clinical trials, meta-analyses, guidelines, international society clinical statements, observational studies, cohort studies, case-control studies and cross-sectional studies of pulmonary sarcoidosis. The aim was to summarize the epidemiology, histopathology, diagnosis and treatment of pulmonary sarcoidosis.
Healio spoke with John A. Belperio, MD, interim chief in the division of pulmonary, critical care and sleep medicine, Clinical Immunology and Allergy at the David Geffen School of Medicine at the University of California, Los Angeles, and one of the authors of the review, about the prevalence of pulmonary sarcoidosis, challenges in management, diagnosis, current treatment options and areas for future research.
Healio: How common is pulmonary sarcoidosis?
Belperio: In the U.S., there are 100,000 to 300,000 active cases of sarcoidosis. It’s not an uncommon disease. The lowest incidence of sarcoidosis in the U.S. is among Asian individuals, at three per 100,000 people per year, and Hispanic individuals, at four per 100,000 people per year. In white individuals, the incidence is about double, at eight per 100,000 people per year. In Black individuals, the incidence is almost two to three times that of white individuals, at almost 20 per 100,000 people per year. In all races/ethnicities, the incidence is higher in women as compared to men.
Healio: What are the challenges of the various adverse outcomes associated with pulmonary sarcoidosis?
Belperio: Prompt diagnosis is important. Typically, pulmonary sarcoidosis presents with shortness of breath and dyspnea on exertion, cough and chest pain. Some patients with sarcoidosis do not have pulmonary symptoms.
There are four stages of sarcoidosis. Stage 1 sarcoidosis is identified by bilateral hilar lymphadenopathy without pulmonary infiltrates. Usually, many patients with stage 1 sarcoidosis will be asymptomatic. Stage 2 sarcoidosis is identified by bilateral hilar lymphadenopathy with pulmonary filtrates. Stage 3 sarcoidosis is identified by pulmonary infiltrates without bilateral hilar lymphadenopathy. Stage 4 is identified by extensive fibrosis with distortion or bullae.
Healio: Why is there a need to bring greater attention to the diagnosis and treatment of pulmonary sarcoidosis?
Belperio: Pulmonary sarcoidosis can progress to a higher stage over time. In many cases, early-stage sarcoidosis may progress in 10% to 30% of cases. A person can progress to the point that the lung no longer functions very well and a lung transplant is needed. That only happens in 3% to 5% of cases. It is important to diagnose sarcoidosis before the patient loses significant lung reserves and you can’t reverse the changes. Once someone progresses to stage 4 sarcoidosis, that’s where the changes are irreversible. More than 60% of sarcoidosis deaths are due to advanced pulmonary sarcoidosis.
Another complication is precapillary pulmonary hypertension (pulmonary artery hypertension) in sarcoidosis, which can occur in the early stages (I/II) but is much more common in stage III/IV disease. For instance, it’s relatively rare in stage I/II disease (< 5% of cases) then can increase to more than 70% of cases in stage III/IV disease. With precapillary pulmonary hypertension, 5-year mortality increases up to 40%. Precapillary pulmonary hypertension associated with sarcoidosis is treatable. When diagnosed early it is very reversible. If you catch it late, it’s difficult to reverse. That’s why it’s important to make the diagnosis early.
Healio: What are the challenges of diagnosing pulmonary sarcoidosis? How has the diagnosis of pulmonary sarcoidosis changed over time?
Belperio: Flexible fiberoptic bronchoscopy should be performed with transbronchial lung biopsies or endobronchial ultrasound-guided transbronchial needle aspiration in patients with pulmonary infiltrates or hilar or mediastinal lymphadenopathy on chest CT. An endobronchial biopsy is recommended if abnormal airways are present during bronchoscopy. Other causes of granulomas must be ruled out before a diagnosis of sarcoidosis is made.
Healio: What are the challenges of treating pulmonary sarcoidosis? How has the treatment of pulmonary sarcoidosis changed over time?
Belperio: Treatment is recommended for patients with sarcoidosis and pulmonary parenchymal abnormalities with significant symptoms or progression of disease. The cornerstone of treatment is oral glucocorticoids; an oral glucocorticoid-sparing agent may also be used. For pulmonary sarcoidosis, prednisone 20 mg to 40 mg per day is commonly used, tapered to 0 mg to 10 mg per day over 6 to 18 months. A longer course of oral glucocorticoids may be required for patients with symptoms, abnormalities and radiographs that do not improve or resolve in a year. Importantly, if a patient is asymptomatic with preserved lung function there is no need to treat. However, if you’re not going to treat, then you have to follow the patient closely, watching for an early decline in lung function when an intervention would be needed.
Prednisone is an oral glucocorticoid and it can have many side effects; it can lead to diabetes, high blood pressure, weight gain, osteoporosis or osteopenia/brittle bones. In the past, we used to treat with higher doses of steroids, and now we know you don’t have to use such higher doses of steroids. Most patients require 6 months to a year of steroids for pulmonary sarcoidosis.
Healio: What is the take-home message for physicians who encounter and care for patients with pulmonary sarcoidosis?
Belperio: No. 1 is to make sure you have the diagnosis for sarcoidosis and rule out other causes of granulomatous disease. No. 2 is, once you have that diagnosis of sarcoidosis, those patients typically do well. If you’re not going to treat them, you should still monitor them and see them at least twice a year. The easiest way to follow them is with a chest X-ray and a pulmonary function test. If you’re seeing changes in the chest X-ray and pulmonary function tests, especially if the patient is symptomatic, then treatment is necessary.
Sarcoidosis is a disease that, while not the rarest of diseases, does require some expertise. You don’t want to overtreat; you don’t want to treat everyone. Some patients will get better over time. You won’t have to treat many patients with pulmonary sarcoidosis. For instance, you might have a female patient with breast cancer and you diagnosed them with sarcoidosis, but they’re not symptomatic. So, you may say, “I don’t want to treat [this patient] with steroids, because that may interfere with their breast cancer treatment.” Instead watch them with chest X-ray and pulmonary function testing.
Really understanding this disease, how to treat it, who needs treatment and who doesn’t need treatment is important.
For more information:
John A. Belperio, MD, can be reached at jbelperio@mednet.ucla.edu.