Sarcoidosis often a chronic disease that affects multiple organs: Study
Patients at specialty centers have high rates of lung, heart problems
Sarcoidosis is a chronic, multi-organ disease for most patients receiving care in specialty centers, and it has the potential to become more severe with time, according to a large U.S. study.
Patients also had high rates of pulmonary fibrosis, or lung scarring, which is associated with worse lung function, and of cardiac sarcoidosis, when the disease affects the heart, data showed.
“Our data support the need for programs around prevention and treatment of pulmonary fibrosis and cardiac sarcoidosis, and the development of long-term, multi-disciplinary management strategies,” researchers wrote.
The study, “An inventory of clinical sarcoidosis status in the United States,” was published in CHEST.
‘The landscape of sarcoidosis in the US is unclear’
Sarcoidosis is marked by excessive immune system activation, which leads to the formation of small clumps of inflammatory cells called granulomas. It may affect different organs and cause permanent tissue damage.
“The landscape of sarcoidosis in the United States is unclear, which makes it difficult to optimize the allocation of [healthcare] resources, clinical care programs, and research activities for sites which specialize in sarcoidosis,” the researchers wrote.
With this in mind, a team of researchers in the U.S. evaluated the current clinical and socioeconomic status of people with sarcoidosis in the country.
The study, dubbed SARCoidosis Outcomes in all respiratory Viral Infectious Diseases, included 2,034 patients who lived in 39 states and who were recruited between 2020 and 2022. Their mean age was 56.6 years, and 53.8% were women. Most patients were white (70.8%), while 27.1% were Black.
About one-third of the participants had a history of smoking, but only 5.4% were current smokers. Most had at least one coexisting condition (68.1%), with obesity being the most common (49.4%), followed by high blood pressure (37.9%), diabetes (17.1%), and past or present cancer (16.4%).
In 90.3% of the cases, sarcoidosis diagnosis was determined through both clinical features and tissue biopsy. The lungs were the most commonly affected organ (76%), followed by lymph nodes, a type of immune structure, in the chest (68.9%).
Involvement of the skin, liver/spleen/bone marrow, heart, eyes, musculoskeletal system/joints, and central nervous system (CNS) were each reported in up to one-fifth of the participants at enrollment. The CNS is composed of the brain and spinal cord.
Among people with lung involvement, over one-third (35.9%) had signs of lung fibrosis, which was associated with worse lung function. This effect was more pronounced in those with bronchiectasis, a condition characterized by the irreversible enlargement and damage of the airways.
Pulmonary hypertension, when blood pressure in the arteries of the lungs is increased, was present in 11% of all participants.
New organ involvement more common in chronic sarcoidosis patients
At the time of enrollment, two-thirds of the participants had chronic disease, meaning it had been diagnosed for more than three years, and 79.8% had multi-organ disease affecting a median of two organs.
People with chronic sarcoidosis were significantly older than those with a more recent diagnosis, and the disease affected significantly more of their organs. They also had significantly higher rates of the more severe clinical profiles of pulmonary hypertension, pulmonary fibrosis, and cardiac sarcoidosis.
When comparing clinical features according to race, the team found that Black people had higher rates of chronic (73.6% vs. 63.6%) and treatment-requiring sarcoidosis (63.7% vs. 50%) than white individuals. Although the rate of lung involvement was comparable between groups, pulmonary fibrosis and bronchiectasis were more common in Black patients, and their lung function was worse.
During follow-up, 105 participants experienced new organ involvement, most commonly concerning the heart, CNS, and skin. This translated to a rate of 5.5 new organ disease cases per 100 patient-years, meaning that for every 100 individuals evaluated over one year, 5.5 showed new organ involvement.
Our data indicate that the narrative that sarcoidosis is often a fleeting and relatively benign disease needs to be updated to reflect the reality that, for many patients … sarcoidosis is complex and can become more severe over time.
New organ involvement was more common in people with chronic sarcoidosis than in those who were recently diagnosed (64.9% vs. 20.2%), emphasizing the progressive nature of the disease.
More than half of the participants (53.4%) required treatment at the time of study entry. A higher proportion were receiving a non-steroidal regimen (39.7%), while 35.5% were on a combination regimen of non-steroids and corticosteroids, and 24.8% were taking a corticosteroid alone. Corticosteroids are anti-inflammatory and immunosuppressive medications commonly used in sarcoidosis.
“Our data indicate that the narrative that sarcoidosis is often a fleeting and relatively benign disease needs to be updated to reflect the reality that, for many patients … sarcoidosis is complex and can become more severe over time,” the researchers wrote.
The median distance patients had to travel to the clinic was 29 miles, or 46.7 km. This finding “raises concern that those without the means to travel may not be receiving the care they need,” the researchers wrote. “Ongoing work around accessibility remains a critical need to improve the health and outcomes of patients with sarcoidosis.”
The team concluded that sarcoidosis patients should be monitored for heart and lung issues.
“As we await future studies to better define the risk factors and [underlying mechanisms] of high-risk [clinical profiles], our findings suggest that long-term surveillance for cardiac involvement and fibrotic transformation is prudent in many cases, and diligent screening for pulmonary hypertension may be an important consideration for all patients with unexplained cardiopulmonary impairments,” the researchers wrote.
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