Löfgren Syndrome and Its Connection to Sarcoidosis

Löfgren syndrome resists easy explanation. It's an inflammatory condition that has a telltale triad, or set of three symptoms. It's related to sarcoidosis, but it isn't exactly the same thing. To add to the complexity, not all people with Löfgren syndrome go on to have sarcoidosis, and not all people with sarcoidosis start with Löfgren syndrome.

So first things first. Sarcoidosis is an inflammatory disease where clumps of immune cells (granulomas) nestle themselves in different parts of the body and cause bothersome symptoms. Sarcoidosis is a chronic (long-term) disease that can last for decades and cause serious complications, even death.

About 5% to 10% of people with sarcoidosis start off with Löfgren syndrome — and they tend to be younger adults. The good news is that their symptoms tend to resolve quickly. Let’s find out more about this condition and how it relates to the complex disease of sarcoidosis.

What are the symptoms of Löfgren syndrome?

Three main features make up Löfgren syndrome:

1. Joint pain and stiffness

Joint inflammation, or arthritis, causes joint pain and stiffness. Any joints in the body can be affected, although most people have symptoms that start in the ankles, followed by other large joints in the legs. Joint symptoms can affect several joints at the same time (“polyarthritis”) and can move from joint to joint (“migratory polyarthritis”). Joints may feel painful, stiff, swollen, and even red. Some people can also get inflamed tendons, the thick cords that attach muscles to bones. When tendons get inflamed, it can be uncomfortable to move joints and limbs in new and different directions. 

2. Erythema nodosum

Sarcoidosis and Löfgren syndrome cause a unique pattern of skin lumps. The lumps appear in the fatty layer of the skin, below the surface. These lumps tend to be warm, red, firm, and painful. Erythema nodosum usually appears on the shins and the forearms. 

3. Enlarged lymph nodes in the chest

In Löfgren syndrome and sarcoidosis, clusters of lymph nodes (glands) in the lungs get larger than normal. These often show up on a chest X-ray or a CT scan. The medical term for this is “bilateral hilar lymphadenopathy.”

What’s the difference between Löfgren syndrome and sarcoidosis?

Löfgren syndrome is a rare type of sarcoidosis. Unlike sarcoidosis, the symptoms of Löfgren syndrome come on suddenly and quickly go away. It’s important for healthcare providers and those affected to distinguish Löfgren syndrome and sarcoidosis because the pattern and outcome of both diseases is quite different.    

Löfgren syndrome is easier to diagnose than sarcoidosis

Because Löfgren syndrome has such a typical pattern of symptoms, any person who has joint symptoms, enlarged lymph nodes on a chest X-ray, and erythema nodosum is diagnosed with Löfgren syndrome. Studies have shown you’re 95% likely to have Löfgren syndrome if you have these three sets of symptoms. Contrast this with sarcoidosis, for which no single test exists. People with sarcoidosis often endure months to years of tests before they get a diagnosis. These tests can range from heart and lung scans to biopsies and regular blood draws.

There are cases of Löfgren syndrome that aren’t “typical,” meaning that one or more of the classic symptoms is missing. Your sex may even play a role in the pattern of Löfgren symptoms you have (more on this below).

Löfgren syndrome is easier to treat and usually goes away on its own

Treatment for Löfgren syndrome is different from treatment for sarcoidosis. That’s because people with Löfgren syndrome typically recover within 1 to 2 years without treatment. Long-term complications are also rare. Treatment for Löfgren syndrome focuses on your symptoms, meaning you’ll get medications to ease unpleasant symptoms such as pain. These medications don’t actually change the course of the disease because it’ll resolve on its own. But treatment for sarcoidosis relies on medications such as steroids or other immunosuppressants in order to combat the disease from progressing. 

Medications that healthcare providers commonly prescribe for Löfgren syndrome include NSAIDs and colchicine. Steroids are only needed in rare cases. 

Who can get Löfgren syndrome?

Löfgren syndrome primarily affects young adults. And anyone who gets it can go on to develop sarcoidosis. Although both females and males can get Löfgren syndrome, symptoms vary by sex. When men get Löfgren syndrome, they are less likely to have erythema nodosum than women. Researchers aren’t really sure why this happens. Women may also be less likely to describe the classic ankle and leg joint pains when they have Löfgren syndrome.

Is Löfgren syndrome an autoimmune disease?

Löfgren syndrome and sarcoidosis are examples of inflammatory disease. Although they share some similarities to autoimmune diseases — technically they aren’t classified as such. If that seems complicated, that’s because it is. Scientific researchers still aren’t sure what causes Löfgren syndrome or sarcoidosis. Research is ongoing about the role of genetics and environmental triggers (like seasonality).

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Written by:

Meg Burke, MD

Meg Burke, MD, is a practicing primary care geriatrician in the Denver, Colorado area. She is board-certified in internal medicine and geriatric medicine, and received her undergraduate and medical degrees from Georgetown University in Washington, D.C. After graduating from medical school, Dr. Burke completed her internal medicine residency at Johns Hopkins Bayview Medical Center and her two years of subspecialty training in geriatric medicine at the Johns Hopkins Hospital in Baltimore, Maryland.

Edited by:

Sophie Vergnaud, MD

Sophie Vergnaud, MD, is the Senior Medical Director for GoodRx Health. An experienced and dedicated pulmonologist and hospitalist, she spent a decade practicing and teaching clinical medicine at academic hospitals throughout London before transitioning to a career in health education and health technology.

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