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 (in Danish)
Contents
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 in both children and adults is characterized by daily episodes of high fever, intermittent rash and joint swelling (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 autoinflammatory 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 can occur in prolonged cycles of 8 weeks - 8 months. Fever peaks of more than 39 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 Bright-red ("salmon-colored") eczema which comes and goes in minutes - hours, most often together 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
- Disseminated intravascular coagulopathy (DIC)
- Enlarged spleen (Splenomegaly)
- Enlarged Liver (Hepatomegaly)
- Macrophage Activation Syndrome (MAS / Reactive HLH)
- Myocarditis
- Often younger men, the first year after the onset of the disease
- Pulmonary haemorrhage (Diffuse alveolar hemorrhage)
- Pericarditis (Inflammation of the heart bag)
- Peritonitis (Inflammation of the abdomen / abdomen)
- Pleurisy (Inflammation of the pleura)
- Thrombotic Thrombocytopenic Purple (TTP)
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 (in Danish)
Other diseases with Arthritis or other rheumatic inflammation
- Vasculitis diseases
- Systemic connective tissue diseases
Infections
- Not unexpectedly, the disease is often misunderstood, but antibiotics like penicillin and correspondingly have no effect
- Sepsis (blood poisoning)
- Endocarditis
- Tuberkulosis (Tbc), Yersinosis
- Virus hepatitis
Cancer
Autoinflammatory fever syndromes
Treatment
- NSAIDs such as Diclofenac, Ibux and the like usually do not have good effect (reference: Gerfaud-Valentin M, 2014)
- Cortisone (prednisolone), but a high dose must be used and the side effects will be significant
- In recent years, biological medication has been used in the form of Kineret (Anakinra) which inhibits interleukin -1 (IL-1 inhibitor) has rapid and lasting effect as long as the syringes are set daily (reference: Nordstöm D, 2012, Cavalli G, 2015). This drug has taken over from cortisone as the first choice of treatment
- Ilaris (canakinumab) is an alternative to Kineret
- A lesser used alternative is RoActemra (tocilizumab) which inhibits interleukin 6 (IL-6 inhibitor) (Reference: Cavalli G, 2015)
- Some have effect from supplement with Methotrexatewhile Enbrel has shown less effect (reference: Cavalli G, 2015).
- In chronic arthritis-like form, treatment as in arthritis (Rheumatoid arteritis) is tried
- Immunoglobulin intravenously (2g / kg body weight distributed over 3-5 days) is not usually used except for some cases of concomitant Macrophage activation syndrome (MAS / HLH), by other life-threatening complications or by high disease activity in pregnancy
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 tried after 6-12 months, provided that there has been an uncomplicated course of the disease and absence of disease activity (clinical and blood tests) and without cortisone / Prednisolone last 6-12 months
- If previous complications from internal organs or the blood (see «Complications» above) the treatment should last longer, often many years
- Biological treatment can be stopped gradually, for example Kineret can be given every other day instead of daily for a couple of months before the treatment is stopped.
- 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 Danish)
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.
