Adult onset Still's disease(AOSD): Symptoms, Diagnosis & Treatments

Adult still's disease is an inflammatory disorder with Fever, Arthritis, and Evanescent rash. Still's disease is an inherited disease in children, but it is called Adult still's disease when a similar symptom appears in patients in their 30s and 40s. It is an uncommon disease that can take up to 0.16 out of 100,000. The prevalence of men and women is globally same, but in the Orient it is known to be slightly higher in females. It affects 15 to 25 years old and develops once again at 36 and 46 years old. It has a recurrence characteristic after the onset. There is no definite explanation for the cause, but it has been pointed out that EBV and CMV are involved now.

Symptoms
It is characterized by a fever of over 39 degrees and shows the appearance of spiking fever. In addition, arthralgia appears in major joints such as hands, elbows, knees, and feet. Rashes are often accompanied by fever and show typical rheumatoid rash. In some cases, pericarditis and chest wallitis may occur. Laboratory findings of ASD include leukocytosis, elevation of serum ferritin, ESR and CRP.


Diagnosis
It is important to know the criteria for AOSD diagnosis, using Yamaghuchi criteria and the contents are as follows. If you meet more than 5 in the criteria below and two of the major criteria are met, you will be able to diagnose it when you exclude other diseases.

Major criteria
- Fever of at least 39C lasting at least one week
- Arthralgia or arthritis lasting two weeks or longer
- A nonpruritic macular or maculopapular skin rash that is salmon-colored in appearance and usually found over the trunk or extremities during febrile episodes
- Leukocytosis with at least 80 percent granulocytes

Minor criteria
- Sore throat
- Lymphadenopathy
- Hepatomegaly or splenomegaly
- Abnormal liver function studies, especially elevations in aspartate and alanine aminotransferase and lactate dehydrogenase concentrations
- Negative tests for antinuclear antibody (ANA) and rheumatoid factor (RF)

Treatments
Depending on the patient's condition, NSAIDS, steroids and DMARD can be used as a treatment. The decision to determine disease activity based on whether organ system involvement is significant or not is determined by the use of NSAIDS, which is 10 to 20%. In patients who are thought to be mainly moderate-severe, Steroid administration does not stop with NSAIDS but begins with systemic symptoms and severe inflammation. In cases of difficulty, steroids may be administered as a pulse, or immunosuppressants or globulin may be administered.

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