1. What is adult Still’s disease?
Adult Still’s disease is a systemic autoimmune inflammatory disease, a special type of rheumatoid arthritis, once called “subacute allergic sepsis”. The disease occurs in 30-40 years old, more women than men, the course of the disease arthritis, fever, rash and other acute attacks and remission alternately.
2.What are the clinical features of adult Still’s disease?
(1) Fever Almost 100% of patients have fever, mostly flaccid fever, regressive fever or periodic intractable hyperthermia, as daily unimodal or bimodal fever.
(2) Skin rash
In the early stage of the disease, 90% of patients develop maculopapular rash or erythema, mainly on the trunk, upper arms and lower extremities and mechanically irritated areas. Very few rashes appear on the face, palms and toes, brownish-red or orange in color, sometimes appearing only for a few hours at the peak of fever, or continuing into the evening or night, becoming a residual rash that gradually disappears, and appearing and disappearing is the characteristic of this rash.
(3) Joint and muscle symptoms
Joint pain and arthritis is one of the main manifestations, the incidence of more than 80%, can be polyarthritis or oligoarthritis, increased when the fever, the fever subsides to reduce or alleviate. In synovitis, there may be joint effusion, but it is usually mild and transient, and rarely causes joint disability or destruction. Joint swelling and pain may be wandering, with the knee joint being the earliest and most susceptible, and the proximal interphalangeal and metacarpophalangeal joints are also commonly affected. The joint symptoms may be the first to appear, or they may occur weeks or years after the onset of fever. More than half of the patients have varying degrees of muscle pain, which may be generalized or predominantly gastrocnemius pain.
(4) Sore throat 50%-90% may have sore throat, but pharyngeal examination is only slightly congested, red and swollen, and bacterial culture is negative.
(5) Lymphadenopathy and splenomegaly
The cervical and submandibular lymph nodes are often involved, and there may be mild tenderness on movement. Lymph node pathology shows plasma cell and neutrophil infiltration, which is reactive hyperplasia. Splenomegaly is mild to moderate and disappears as the systemic manifestations resolve with the lymph nodes and splenomegaly.
(6) Hepatomegaly and liver function abnormalities
Half of the patients have mild hepatomegaly and abnormal liver function, and a few may have jaundice. Liver biopsies mostly show non-specific inflammatory cell infiltration or blast cell hyperplasia, interstitial inflammation and steatosis in the confluent area.
(7) Hematological manifestations
In the active stage of the disease, more than 90% of patients have increased neutrophils, about 80% of patients have blood leukocyte count ≥ 15×109/L; about 50% of patients have elevated platelet count; can be combined with orthocytic orthochromic anemia; almost 100% of patients have increased sedimentation.
(8) Serum ferritin Serum ferritin is increased and its level is positively correlated with disease activity, which is meaningful to determine whether the disease is active and evaluate the effect of treatment.
3.How to treat adult Still’s disease
(1) A few patients can control their symptoms by applying non-steroidal anti-inflammatory drugs alone.
(2) Glucocorticoid is the main drug for the treatment of this disease. The starting dose is 0.5-1 mm/kg body weight per day, and the dose is gradually reduced by 5-2.5 mg per week after 4-6 weeks, and the reduction rate is gradually slowed down and finally maintained at the minimum dose.
(3) For patients with obvious symptoms and recurrent disease in hormone reduction should be added early with chronic long-acting anti-rheumatic drugs, such as methotrexate 7.5-20 mg/week, azathioprine 50-150 mg once daily, cyclophosphamide 0.1-0.2 g once every other day, or rhodopsin 20 mg three times daily. Ixepro-led biologics have shown good efficacy in adults with Still’s disease.