Lofgren syndrome is a clinical phenotype of acute sarcoidosis. Sarcoidosis is an inflammatory disorder that is recognized by swelling of lymph nodes in the chest, fever, arthritis, and red knobs on the shins. It is more commonly found in women than in men especially in the areas of Irish, Scandinavian, Puerto Rican, and African heritage.
Background
Lofgren syndrome was first described by a Swedish pulmonologist, Sven Lofgren in 1946 as a milder form of sarcoidosis. He stated that Lofgren syndrome was a distinct type of acute sarcoidosis. Sarcoidosis is an inflammatory disease in which a cluster of inflammatory cells is formed in different organs of the human body that causes organ inflammation.
Cutaneous indications of sarcoidosis are seen in up to 33% of patients and might be the main clinical indication of the sickness. Sarcoidosis has a slow disease progression, however, unlike sarcoidosis, Lofgren syndrome presents acutely.
Affected Areas in Lofgren syndrome
When a person has Lofgren syndrome, the following areas of his body are affected:
- Eyes
- Lungs
- Skin
- Lymph nodes
- Liver
- Heart
- Spleen
- Brain
What are the Causes?
Lofgren syndrome rarely occurs in infants and children. People aged somewhere between 20 to 40 are mostly affected by Lofgren syndrome. However, the main contributor to Lofgren syndrome could be a person’s gender, race, and genetics.
Although the main cause of Lofgren syndrome or even sarcoidosis is not known yet, upon experience and assumptions following can put one at risk of getting Lofgren syndrome:
- Gender Role
Lofgren is more common in women than in men.
- Geographical Role
People residing of African-American descent are at high risk of developing Lofgren syndrome.
- Genetic Role
People having a history of Lofgren syndrome or sarcoidosis are more likely to develop Lofgren syndrome.
What are the Symptoms And Signs?
Unlike sarcoidosis which is recognized by patches of inflammatory cells in organs and tissues in a human body, Lofgren syndrome does not have any such long-term chronic effect on the human body along with little to no damage to human organs. However, the symptoms of Lofgren syndrome are somewhat similar to the symptoms of sarcoidosis.
Here is a list of Lofgren syndrome symptoms:
- Fatigue
- Fever
- Malaise / low energy
- Arthritis
- Joint pain
- Abdominal swelling
- Inflammation of the eyes
- Swollen and inflamed salivary glands
- Swollen lymph nodes in the chest
- Tender red, painful bumps on the skin (usually on leg front)
- Dry mouth
- Nose bleeding
- Skin rashes
- Dry eyes
- Itchy eyes
- A discharge from your eyes
Diagnosis
As mentioned earlier, a combination of fatigue, arthritis, fever, swollen lymph nodes, and skin rash symptoms can be related to Lofgren syndrome. However to be more sure it is advisable to get your blood tests done for better diagnosis since these symptoms are similar to those of arthritis and cancer.
These medical tests can help gain a better insight into the diagnosis. For instance, getting an MRI(Magnetic resonance imaging) scan of the chest can help in finding out any granulomas or inflammatory cells in the organs such as the lung or any swelling in the lymph nodes in the chest.
Moreover, high blood levels of an enzyme(Angiotensin-converting enzyme) can also be one of the causes of Lofgren syndrome. They cause the blood vessels to shrink, which in return increases blood pressure and inflammation.
For a better diagnosis, your doctor might check you physically for any:
- Skin bump or rash
- Swollen lymph nodes
- Swollen liver or spleen
- Diagnose heart and lungs
Once done, based on the results you will be prescribed either of the following diagnostic tests:
- A chest x-ray for granulomas or swollen lymph nodes
- A chest CT scan to take cross-sectional pictures of your chest for a better clearer picture.
- A lung function test to check if your lung has been affected or not
- A biopsy by examining your tissues for any granulomas
Treatment
Lofgren syndrome gets treated and vanishes away within a few months or a year, all on its own without any treatment. However, doctors do prescribe a few drugs depending on your body, such as non-steroidal anti-inflammatory drugs that can help ease the pain. (anti-inflammatory drugs for example aspirin and acetaminophen. Steroidal drugs can also be used to reduce inflammation and pain).
In case if the following are affected, you might then follow a treatment plan:
- Eyes
- Nervous system
- Heart
- Lungs
Depending upon your progress (that varies from person to person), you will be then prescribed certain medications to suppress your immune system.
Prognosis
Lofgren syndrome can be seen with a good prognosis since almost more than 90% experiencing the condition tend to get better within two years.
Complications in Lofgren Syndrome
As per the prognosis, almost 90% of people showed positive recovery from Lofgren syndrome. Likewise, most people with Lofgren syndrome do not experience complications. However, some complications may occur such as:
- Irregular, unusual heartbeat
- Kidney failure
- Lung infection
- Infertility
Differential Diagnosis
The response example of fever, arthralgia, and injuries of erythema nodosum, however suggestive are not limited to sarcoidosis. Responses might be brought about by a few different triggers including:
- Prescription actuated reasons for penicillin, sulfa drugs, oral contraceptives, vaccinations
- Other fundamental provocative inflammatory illnesses like fiery entrail illness, lupus, Behcet infection
- Pregnancy
- Contaminations like Streptococcus, enteric microbes, Mycobacterium tuberculosis, coccidioidomycosis, histoplasmosis, blastomycosis
Bottom Line
Lofgren syndrome is a clinical phenotype of acute sarcoidosis. It is more commonly found in women than in men. Sarcoidosis has a slow disease progression, however, unlike sarcoidosis, Lofgren syndrome presents acutely.
The causes of Lofgren syndrome can vary from person to person but the roles played by gender, geography, and genetics can put one at high risk of contracting this condition. Lofgren syndrome does not have any such long-term chronic effect on the human body along with little to no damage to human organs.