Löfgren’s

By Peter Galvin, MD

Today’s case is from the Alexandria University Faculty of Medicine in Alexandria, Egypt. A 26-year-old man who had emigrated from Nepal seven years earlier was referred to the pulmonary clinic with a six-month history of remitting and relapsing ankle swelling, a two-month history of cough and unintentional weight loss, and a three-day history of leg rash. A chest X ray obtained six weeks after the onset of cough had shown hilar adenopathy (Panel A, circled area). This is enlargement of the lymph nodes at the root of the lungs in the center of the chest, an area known as the hilum. At the current presentation, physical examination was notable for erythematous (red), tender nodules and plaques on the anterior shins (Panel B), and swelling and tenderness of the ankles. CT scan of the chest showed enlarged mediastinal and hilar lymph nodes (Panel C, asterisks) and otherwise normal lungs. Because of the concern about possible tuberculosis, a transbronchial biopsy of the right paratracheal and hilar lymph nodes was performed. Pathology analysis showed noncaseating granulomas (solid clumps of inflammatory cells). Microbiologic studies for fungal and mycobacterial infections, including a test for Mycobacterium tuberculosis, were negative.

A diagnosis of Löfgren’s syndrome, an acute form of sarcoidosis characterized by erythema nodosum (the leg rash), migratory polyarthritis (the waxing and waning ankle pain and swelling), and hilar adenopathy, or lymph node enlargement, was made. Sarcoidosis is a non-infectious granulomatous disease that can affect the skin, lungs, heart, and joints. It is characterized by abnormal clumps of inflammatory cells that form lumps known as granulomas. The cause of sarcoidosis is unknown, but it is thought to be an immune system reaction to a trigger such as an infection or exposure to chemicals in those who are genetically predisposed. It does run in families. Sarcoidosis is characterized by large lymph nodes at the lung roots (hila), high calcium levels with normal parathyroid hormone levels, and high levels of angiotensin-converting enzyme (ACE).

Often, sarcoidosis may resolve on its own over a period of a few years. When symptoms warrant treatment, it will usually respond to anti-inflammatory medications such as NSAIDs like ibuprofen or oral glucocorticoids like prednisone. The patient in this case was placed on a 6-week tapering course of oral glucocorticoids and his condition rapidly improved. Lastly, by coincidence, today is the 24th anniversary of the 9/11 disaster. Many cases of sarcoidosis are thought to have been caused by exposure to the toxins present that day and for many months afterward. Thankfully, as a 9/11 first responder, I didn’t develop sarcoidosis, but many fellow first responders did.

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