Kinder-Rheuma

Rheumatic disease in children and adolescents

Research Topic: Paediatric Rheumatology

Rheumatic diseases are generally considered to affect older people, but it is a little known fact that children and adolescents can also be affected. An estimated 20,000 children and adolescents are affected by chronic inflammatory rheumatic diseases nationwide. These diseases include chronic joint inflammation (such as juvenile idiopathic arthritis [JIA]), connective tissue diseases (collagenosis, such as systemic lupus erythematosus, juvenile dermatomyositis, scleroderma), primary chronic vascular inflammation (vasculitis, such as polyarteriitis nodosa, granulomatosis with polyangiitis) and autoinflammatory diseases (such as periodic fever syndromes FMF, TRAPS, CAPS). All these diseases are associated with a high risk of joint and organ damage, permanent disability and long-term impairment of quality of life and participation. To prevent this, they must be quickly identified and treated.

Juvenile Idiopathic Arthritis (JIA) is the most common of the paediatric rheumatic diseases. Approximately one in every 1,000 children and adolescents is affected, i.e. we expect about 15,000 patients nationwide. JIA has many faces: clinical presentation ranges from oligoarthritis (inflammation of a single or a few joints), to polyarthritis (inflammation of many small and large joints), to systemic form of rheumatoid arthritis (a highly acute febrile disease with skin rash and inflammation of internal organs). Due to the very different clinical symptoms, different forms of JIA are distinguished from each other.

Tabelle: Erscheinungsformen

Information on the individual forms of JIA, the consequences resulting from the disease, the treatment concepts and the care situation of the children and adolescents concerned is provided by the core documentation of children and adolescents suffering from rheumatic disease (Kinder-KD).

Abbildung Fragebögen: Arztbogen, Elternbogen, Jugendbogen

JIA is difficult to detect: there is no diagnostic parameter. The leading symptom of JIA is joint swelling, however, pain is often not indicated. Children typically show symptoms when they are 2 to 3, an age when pain is not expressed verbally, but through abnormalities in the posture of the joints or in the course of movement. Depending on which joints are affected, it is noticeable that the children are less active, more limp, grip differently or have difficulty dressing or other everyday movements. Children often tend to alter their movements and actions to minimize strain on the painful joints.

Abbildung: Erste Hinweise auf Gelenkrheuma

JIA requires consistent, long-term treatment that rests on several therapy pillars. Medicinal, physiotherapeutic, ergotherapeutic, orthopaedic and psychosocial measures are applied. The earlier the treatment starts, the better the results. The aim of the therapy is no longer only to alleviate the symptoms, but to completely suppress the rheumatic inflammation, i.e. to achieve an inactive disease and unrestricted everyday function. Affected persons should be able to lead a normal life with the highest possible quality of life in the long term.

For the drug treatment of JIA there are cortisone-free, pain- and inflammation-relieving drugs, cortisone as a strong anti-inflammatory and the so-called disease-modifying drugs (DMARDs, disease modifying antirheumatic drugs) available. Which substances are used, how the therapy changes over time and what effects this has on the prognosis of the patients are investigated with the core documentation for children and adolescents suffering from rheumatic inflammation.

Abbildung: Therapie

A better understanding of disease processes has enabled the development of so-called biologics (bDMARD). Biologics are drugs that are produced using biotechnology and are based on natural groups of substances. They intervene relatively specifically in the rheumatic inflammatory process by slowing down activated inflammatory cells or intercepting inflammatory messengers such as the tumour necrosis factor alpha or certain interleukins.

With the new therapy strategies, it is becoming increasingly possible to control inflammation and stop the progression of the disease. Therapies are very effective in suppressing the inflammatory reaction; if it is stopped, the inflammation often flares up again. Despite the progress made, rheumatic diseases cannot yet be cured.

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