Written by: Cindy Wu, Phoebe Wen, Shams Esam, Blessy Thomson, Emma Wang, Amarthya Pandian, Kimora Sellers, Gabriella Sentosa, and Mai Lam
Introduction:
Schnitzler syndrome is a rare autoinflammatory condition. Schnitzler syndrome is also characterized by chronic, nonpruitic urticaria in association with monoclonal gammopathy. Schnitzler syndrom is quite mystery in healthcare industry because the cause of Schnitzler syndrome is unknown. Some associated symptoms of Schnitzler syndrome included recurrent fever, bone pain, and arthritis.
Cause:
The exact cause of Schnitzler syndrome is not yet fully understood. It is believed to be a complex and rare autoinflammatory disorder with an underlying genetic predisposition. It is thought to involve abnormal activation of the immune system, leading to excessive production of certain proteins and inflammation. A few patients have been described with a mutation in the gene NLRP3.However, the specific genetic and molecular mechanisms involved in Schnitzler syndrome are still being investigated.
Effects:
Schnitzler syndrome causes chronic hives, fever, joint and bone pain, fatigue, and swelling of the lymph nodes, liver, and spleen. The symptoms can have a significant impact on a person's quality of life, with many individuals experiencing chronic pain, difficulty sleeping, depression and increase the risk of other health problems like amyloidosis.
Risk Factor:
The risk factor of Schnitzler syndrome remains unknown, but medical experts have identified a correlation between the syndrome's symptoms and heightened levels of the inflammation factor interleukin 1. This factor contributes to the development of fever and other symptoms associated with Schnitzler syndrome. However, the reasons for the increased presence of IL-1 in individuals with Schnitzler syndrome are unclear at this time.
When to see doctor:
Schnitzler syndrome is a rare autoinflammatory disease that may start to appear during adulthood, and it should be considered during the differential diagnosis process of urticarial rash, monoclonal gammopathy, and fever of unknown origin. However, the diagnosis of Schnitzler syndrome is often delayed since it is infrequent (An estimated fewer than 1,000 people in the U.S. have this condition). One criterion for diagnosing is the Strasbourg criteria. If any symptoms occur, seeking health professionals is advised to get a correct diagnosis.
Life Expectancy:
Due to the rarity of the condition, there is no specific information on life expectancy for individuals with Schnitzler syndrome. However, it is generally believed that Schnitzler syndrome has a relatively favorable prognosis, with most individuals having a normal life expectancy. Such as 91% percent for a solid 15 years(patients usually range from 13-17 years in age), 15% risk for 10 years of developing a lymphoproliferative disorder
Who's more at risk:
There are currently no specific risk factors for Schnitzler syndrome, however the disease itself is suspected to be in some way hereditary. Most patients will have anemia at the time of diagnosis. Leukocytosis and thrombocytosis (both reactive) are also very common, however do not work as an indicator of the disease.
Treatment:
Patients infected with the Schnitzler syndrome benefit greatly from Anakinra treatment. Anakinra medicine is an interleukin-1 receptor antagonist. A drug that aims to inhibit the activity of interleukin-1 (a type of cytokine that sends signals from the immune system to the rest of the body). Furthermore, it is a protein produced by the body that aids in the regulation of inflammation. Nonsteroidal anti-inflammatory drugs (NSAIDs), also known as corticosteroids, are another medication used to treat Schnitzler syndrome. It is a class of medication aid in the reduction of inflammation and pain associated with the disorder. Where it is also function to improve the skin rash and fever. Ibuprofen is an NSAID medication that is 200 to 400 milligrams (mg) every four to six hours for adults. However, the dose varies depending on the age of the child. For infants and children aged 6 months to 11 years old, the dose is typically 10-15 mg per pound of body weight. However, not all NSAIDs medications are safe for children and can harm them. That being said, it is critical to consult a doctor before using any specific medication.
Source:
“Schnitzler Syndrome - Symptoms, Causes, Treatment: Nord.” National Organization for Rare
Disorders, 12 Jan. 2023, https://rarediseases.org/rare-diseases/schnitzler-syndrome/.Accessed 7 May. 2023.
Prevention:
There is no specific prevention for Schnitzler Syndrome, as the risk factors and triggers are not well understood. However, some general measures that may help reduce the severity or frequency of symptoms include:
Avoiding known allergens or irritants that may worsen the skin rash
Taking antihistamines or other medications as prescribed by your doctor to control the inflammation and itching
Seeking medical attention promptly if you develop signs of infection, such as fever, chills, or swollen lymph nodes
Following a healthy lifestyle that includes regular exercise, adequate hydration, and a balanced diet
Monitoring the blood tests and organ function regularly as advised by your doctor.
Citation:
(Cover Image Source: Reumatología Clínica)
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Jarrett, Pual. "Schnitzler syndrome." DermNet, dermnetnz.org/topics/schnitzler-syndrome. Accessed 8 May 2023.
Mamadgi, Jyothika et al. “Schnitzler's Syndrome: A Diagnostic Consideration in Evaluating the Constellation of Monoclonal Gammopathy and Chronic Urticaria.” Journal of hematology vol. 10,3 (2021): 143-146. doi:10.14740/jh800
Murota, Hiroyuki. "Schnitzler's Syndrome." Dermatology Advisor, 13 Mar. 2019, www.dermatologyadvisor.com/home/decision-support-in-medicine/dermatology/schnitzlers-syndrome/#:~:text=Prognosis%3A%20Schnitzler%27s%20syndrome%20has%20a,patients%20live%20for%2015%20years.
"SCHNITZLER SYNDROME." Belgian Journal of Hematology, www.bjh.be/conditions/schnitzler-syndrome/. Accessed 8 May 2023.
"Schnitzler syndrome." National Center for Advancing Translation Sciences, Feb. 2023, rarediseases.info.nih.gov/diseases/12390/schnitzler-syndrome.
"Schnitzler Syndrome." NORD National Organization for Rare
Disorders, 25 June 2018, rarediseases.org/rare-diseases/schnitzler-syndrome/.
“Schnitzler Syndrome - Symptoms, Causes, Treatment: Nord.” National Organization for Rare
Disorders, 12 Jan. 2023, https://rarediseases.org/rare-diseases/schnitzler-syndrome/.Accessed 7 May. 2023.
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