Still Illness in Adults, Adult Stills 4.44/5 (18)

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Volatile rash in Adult Stills disease. Gopalarathinam R, Case Rep Rheumatol, 2014. CC BY 2.0

Still's disease in adulthood (adult Stills disease) (ICD-10: M06.1)

Juvenile Stills Disease at children (systemic JIA) is described here

Definition

Adult Stills Disease (AOSD) differs from Stills Disease in Children, which is identical to juvenile arthritis (pediatric joints), systemic type, although the conditions have several common characteristics. Still's disease is characterized by daily high-temperature episodes, periodic rashes and joint swelling in both children and adults.Arthritis). Some have a less dramatic, chronic and more Arthritis-like disease course. Adult Still's disease typically attacks adolescents and young adults at the age of 1525. Cases in the age group between 36-46 years have also been reported (reference: Owlia MB, 2009). The disease is often classified as a "poly-genetic auto-inflammatory disease".

Disease Cause

The cause of the disease is unknown, but when Stills disease has started, the signaling molecules (interleukins) IL-1, IL-6 and IL18 elevated in the blood. There is probably a genetic predisposition to develop the disease (poly-genetic auto-inflammatory disease), so that it infects other auto-inflammatory diseases (most often breaks out in childhood)

Symptoms

High fever and arthritis without infection (60-100%)

  • The fever may occur in prolonged cycles of 8 weeks. Fever tips of more than 8 degrees C are common, combined with normal values ​​every day ("spiking fever")

Joint pain and swollen joints (Arthritis) are common (70-100%)

  • Arthritis does not necessarily occur at the onset of illness
  • The disease can attack few or many joints, often on both sides of the body. Stiffness in attaced joints in the morning
    • Wrists, knees, elbows

Et Light-red ("salmon-colored") eczema which comes and goes within minutes - hours, most often with the fever peaks are characteristic, but it can be difficult to register

  • The rash occurs in 60-80% of cases, mostly on the body, rarely in the face, soles of the feet or palms. Rarely itching

Other symptoms

  • Sore throat (70%)
  • Swollen lymph nodes (often on the neck) (50%)
  • Muscle Pain (45%)
  • Pain in the pleura (pleurisy) (21%)
  • Cardiovascular inflammation (Pericarditis) (16%)
  • Abdominal pain (18%)
  • Weight Loss 27%)
  • Enlarged spleen (40%)
  • Magnified liver (30%)
  • The process can be divided into three types that appear about as common:

Mono-phasic disease course

  • Duration over weeks to months, but without relapse. Fever, rash, and fiery inflammation of the heart and lungs (pericarditis, pleurisy) characterizes this group

Intermittent disease course

  • One or more relapses with or without joint inflammation. Symptom-free periods over weeks to two years occur in this group. The recurrences are often milder than on the onset

Chronic course with persistent disease activity

  • The inflammation of joints may be as in arthritis (Rheumatoid arthritis) in adults and cause permanent joint damage. Women are attacked more often than men, and ferritin the blood is often normal or just slightly elevated

Diagnosis

Typical symptoms and findings in medical examinations in the absence of other causes (see differential diagnoses below). Classification criteria can be used, but is not a requirement for the diagnosis. No antibody in blood samples usually turns out, though ferritin in the blood is expected to be clearly elevated by active disease and fever. Fast treatment response (hours-days) using Kineret (anakinra) also supports the diagnosis.

Classification criteria (Yamaguchi)

Complications

Blood tests

Blood tests show signs of inflammation

  • High CRP
  • High Sedimentation rate (ESR)
  • Very high ferritin
  • Increased white blood cell count
  • Increased platelet count (platelet counts)
  • Low blood percentage (anemia)
  • Liver irritation with rash in liver samples (ALT, AST)

Incorrect diagnosis? (Similar diseases / differential diagnoses) are described here

Other diseases with Arthritis or other rheumatic inflammation

Infections

Cancer

Autoinflammatory fever syndromes

Treatment

Treatment duration

Adult Stills' disease clearly has different pathways, so treatment and duration of treatment must be adapted to the individual.

  • Biological treatment can be attempted to be terminated after 6-12 months, provided there has been uncomplicated course of disease and absence of disease activity (clinical and blood tests) and without cortisone / Prednisolone last 6-12 months
  • If previous internal organ or blood complications (see "Complications" above), treatment should last longer, often many years
  • Biological treatment can be terminated gradually, for example, Kineret can be given every other day instead of daily for a few months before the treatment is attempted to end
  • Reference: Vercruysse F, 2019

Adult Stills Disease Pregnancy

Many pregnancies at Still's disease have been completed, usually with successful results. However, some complications have been observed

  • Recurrence of the disease occurs, most often in the latter part of pregnancy (second and third trimester)
  • Prednisone can be used, but high doses (over 15 mg / day) should only be used if necessary
  • Anakinra (Kineret) is used without any adverse effects being observed
  • Immunoglobulin intravenously is a possibility in some cases
  • If the disease is active or immunosuppressive drugs such as Prednisolone or anakinra are used, follow-up of a multidisciplinary team is recommended.
    • A rheumatologist
    • obstetrician
    • Midwife
    • General Practitioner
  • Reference: Gerfaud-Valentin M, 2014
  • More about pregnancy and rheumatic disease can be read here

In Norway the Rheumatology Department, OUS Rikshospitalet are among the departments with the greatest experience with the condition.

Medical prognosis

The severe inflammation reaction with fever can cause serious symptoms without treatment. During treatment, the majority respond well and can live virtually normal. After a few years the disease usually falls to rest and treatment can be terminated. The arthritis-like shape has a more elongated course.

Literature


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