Systemic-onset juvenile idiopathic arthritis

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Systemic juvenile idiopathic arthritis
Other namesSystemic-onset juvenile rheumatoid arthritis, Still's Disease
Specialty Pediatrics/rheumatology

Systemic juvenile idiopathic arthritis (or the juvenile onset form of Still's disease [1] ) is a type of juvenile idiopathic arthritis (JIA) with extra-articular manifestations like fever and rash apart from arthritis. It was originally called systemic-onset juvenile rheumatoid arthritis or Still's disease.

Contents

Predominantly extra-articular manifestations like high fevers, rheumatic rash, enlargement of the liver and spleen, enlargement of the lymph nodes, and anemia. Other manifestations include inflammation of the pleura, inflammation of the pericardium, inflammation of the heart's muscular tissue, and inflammation of the peritoneum are also seen.[ citation needed ] It is sometimes called "juvenile-onset Still's disease" to distinguish it from adult-onset Still's disease. However, there is some evidence that the main difference between two conditions is the age of onset. [2]

Presentation

Systemic JIA is characterized by arthritis, fever, which typically is higher than the low-grade fever associated with polyarticular and a salmon pink rash. It accounts for 10-20% of JIA and affects males and females equally, unlike the other two subtypes of JIA, and affects adolescents. It generally involves both large and small joints. Systemic JIA can be challenging to diagnose because the fever and rash come and go. Fever can occur at the same time every day or twice a day (often in late afternoon or evening) with a spontaneous rapid return to baseline (vs. continuous fever of septic arthritis). The rash often occurs with fever. It is a discrete, salmon-pink macules of different sizes. It migrates to different locations on skin, rarely persisting in one location more than one hour. The rash is commonly seen on trunk and proximal extremities or over pressure areas.[ citation needed ]

Arthritis is often absent in the first weeks or even 6–8 months into the illness. Systemic JIA may have internal organ involvement such as hepatosplenomegaly, lymphadenopathy, serositis, hepatitis, or tenosynovitis.[ citation needed ]

Cause

The cause is unknown but it's thought to be related to environmental, genetic, and hormonal factors.[ citation needed ]

A polymorphism in macrophage migration inhibitory factor has been associated with this condition. [3]

Diagnosis

Rheumatoid factor and ANA tests are generally negative in systemic JIA. Lab findings: anemia of chronic disease (can also appear in non-systemic types [4] ), neutrophilia, thrombocytosis, elevated acute phase reactants (ESR, CRP, ferritin).[ citation needed ]

Treatment

Treatment with either glucocorticoids, methotrexate, anakinra, or tocilizumab has been examined. [5] Anakinra has been shown to resolve the clinical features of the disease in 87% of patients. [6] It also induces remission in half of corticosteroid-resistant patients. [7] The results of another study were similar, with half of the patients responding to treatment with Anakinra. [8] Canakinumab, an antibody to interleukin-1 beta, is indicated for treatment in patients who respond poorly to other treatments. [9]

Prognosis

25% of cases progress to severe destructive arthritis. [10] In the United States, mortality is estimated at 4% [11] and in Europe, mortality is estimated at 21.7%. [12]

History

Still's disease is named after English physician Sir George Frederic Still (1861–1941). [13] [14] It was characterized by EG Bywaters in 1971. [15] [16]

Related Research Articles

<span class="mw-page-title-main">Arthritis</span> Type of joint disorder

Arthritis is a term often used to mean any disorder that affects joints. Symptoms generally include joint pain and stiffness. Other symptoms may include redness, warmth, swelling, and decreased range of motion of the affected joints. In some types of arthritis, other organs are also affected. Onset can be gradual or sudden.

<span class="mw-page-title-main">Rheumatoid arthritis</span> Type of autoimmune arthritis

Rheumatoid arthritis (RA) is a long-term autoimmune disorder that primarily affects joints. It typically results in warm, swollen, and painful joints. Pain and stiffness often worsen following rest. Most commonly, the wrist and hands are involved, with the same joints typically involved on both sides of the body. The disease may also affect other parts of the body, including skin, eyes, lungs, heart, nerves and blood. This may result in a low red blood cell count, inflammation around the lungs, and inflammation around the heart. Fever and low energy may also be present. Often, symptoms come on gradually over weeks to months.

Rheumatology is a branch of medicine devoted to the diagnosis and management of disorders whose common feature is inflammation in the bones, muscles, joints, and internal organs. Rheumatology covers more than 100 different complex diseases, collectively known as rheumatic diseases, which includes many forms of arthritis as well as lupus and Sjögren's syndrome. Doctors who have undergone formal training in rheumatology are called rheumatologists.

<span class="mw-page-title-main">Ankylosing spondylitis</span> Type of arthritis in which there is long-term inflammation of the joints of the spine

Ankylosing spondylitis (AS) is a type of arthritis characterized by long-term inflammation of the joints of the spine, typically where the spine joins the pelvis. Occasionally, areas affected may include other joints such as the shoulders or hips. Eye and bowel problems may occur as well as back pain. Joint mobility in the affected areas generally worsens over time.

<span class="mw-page-title-main">Juvenile idiopathic arthritis</span> Childhood rheumatic disease

Juvenile idiopathic arthritis (JIA), formerly known as juvenile rheumatoid arthritis (JRA), is the most common chronic rheumatic disease of childhood, affecting approximately 3.8 to 400 out of 100,000 children. Juvenile, in this context, refers to disease onset before 16 years of age, while idiopathic refers to a condition with no defined cause, and arthritis is inflammation within the joint.

<span class="mw-page-title-main">Anakinra</span> Pharmaceutical drug

Anakinra, sold under the brand name Kineret, is a biopharmaceutical medication used to treat rheumatoid arthritis, cryopyrin-associated periodic syndromes, familial Mediterranean fever, and Still's disease. It is a recombinant and slightly modified version of the human interleukin 1 receptor antagonist protein. It is marketed by Swedish Orphan Biovitrum. Anakinra is administered by subcutaneous injection.

Macrophage activation syndrome is a severe, potentially life-threatening, complication of several chronic rheumatic diseases of childhood. It occurs most commonly with systemic-onset juvenile idiopathic arthritis (SoJIA). In addition, MAS has been described in association with systemic lupus erythematosus (SLE), Kawasaki disease, and adult-onset Still's disease. It is thought to be closely related and pathophysiologically very similar to reactive (secondary) hemophagocytic lymphohistiocytosis (HLH). The incidence of MAS is unknown as there is a wide spectrum of clinical manifestations, and episodes may remain unrecognized.

Periodic fever syndromes are a set of disorders characterized by recurrent episodes of systemic and organ-specific inflammation. Unlike autoimmune disorders such as systemic lupus erythematosus, in which the disease is caused by abnormalities of the adaptive immune system, people with autoinflammatory diseases do not produce autoantibodies or antigen-specific T or B cells. Instead, the autoinflammatory diseases are characterized by errors in the innate immune system.

<span class="mw-page-title-main">Hemophagocytic lymphohistiocytosis</span> Immune disorder in the blood leading to hyperinflammation

In hematology, hemophagocytic lymphohistiocytosis (HLH), also known as haemophagocytic lymphohistiocytosis, and hemophagocytic or haemophagocytic syndrome, is an uncommon hematologic disorder seen more often in children than in adults. It is a life-threatening disease of severe hyperinflammation caused by uncontrolled proliferation of benign lymphocytes and macrophages that secrete high amounts of inflammatory cytokines. It is classified as one of the cytokine storm syndromes. There are inherited and non-inherited (acquired) causes of HLH.

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Childhood arthritis is an umbrella term used to describe any rheumatic disease or chronic arthritis-related condition which affects individuals under the age of 16. Most types are autoimmune disorders.

Adult-onset Still's disease (AOSD) is a form of Still's disease, a rare systemic autoinflammatory disease characterized by the classic triad of fevers, joint pain, and a distinctive salmon-colored bumpy rash. The disease is considered a diagnosis of exclusion. Levels of the iron-binding protein ferritin may be extremely elevated with this disorder. AOSD may present in a similar manner to other inflammatory diseases and to autoimmune diseases, which must be ruled out before making the diagnosis.

Schnitzler syndrome or Schnitzler's syndrome is a rare disease characterised by onset around middle age of chronic hives (urticaria) and periodic fever, bone pain and joint pain, weight loss, malaise, fatigue, swollen lymph glands and enlarged spleen and liver.

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<span class="mw-page-title-main">Lupus</span> Human autoimmune disease

Lupus, technically known as systemic lupus erythematosus (SLE), is an autoimmune disease in which the body's immune system mistakenly attacks healthy tissue in many parts of the body. Symptoms vary among people and may be mild to severe. Common symptoms include painful and swollen joints, fever, chest pain, hair loss, mouth ulcers, swollen lymph nodes, feeling tired, and a red rash which is most commonly on the face. Often there are periods of illness, called flares, and periods of remission during which there are few symptoms.

<span class="mw-page-title-main">Cryopyrin-associated periodic syndrome</span> Medical condition

Cryopyrin-associated periodic syndrome (CAPS) is a group of rare, heterogeneous autoinflammatory disease characterized by interleukin 1β-mediated systemic inflammation and clinical symptoms involving skin, joints, central nervous system, and eyes. It encompasses a spectrum of three clinically overlapping autoinflammatory syndromes including familial cold autoinflammatory syndrome, the Muckle–Wells syndrome (MWS), and neonatal-onset multisystem inflammatory disease that were originally thought to be distinct entities, but in fact share a single genetic mutation and pathogenic pathway, and keratoendotheliitis fugax hereditaria in which the autoinflammatory symptoms affect only the anterior segment of the eye.

<span class="mw-page-title-main">Intermittent hydrarthrosis</span> Medical condition

Intermittent hydrarthrosis (IH), also known as periodic synoviosis, periodic benign synovitis, or periodic hydrarthritis, is a chronic condition of unknown cause characterized by recurring, temporary episodes of fluid accumulation (effusion) in the knee. While the knee is mainly involved, occasionally other joints such as the elbow or ankle can additionally be affected. Fluid accumulation in the joint can be extensive causing discomfort and impairing movement, although affected joints are not usually very painful. While the condition is chronic, it does not appear to progress to more destructive damage of the joint. It seems to affect slightly more women than men.

Amita Aggarwal is an Indian clinical immunologist, rheumatologist and a Professor and Head at the Department of Clinical Immunology and Rheumatology of the Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow. Known for her studies in autoimmune rheumatic diseases, Aggarwal is a recipient of the Shakuntala Amir Chand Award of the Indian Council of Medical Research and an elected fellow of the National Academy of Sciences, India, National Academy of Medical Sciences and the National Academy of Medical Sciences. The Department of Biotechnology of the Government of India awarded her the National Bioscience Award for Career Development, one of the highest Indian science awards, for her contributions to biosciences in 2004.

<span class="mw-page-title-main">Adenosine deaminase 2 deficiency</span> Medical condition

Deficiency of Adenosine deaminase 2 (DADA2) is a monogenic disease associated with systemic inflammation and vasculopathy that affects a wide variety of organs in different patients. As a result, it is hard to characterize a patient with this disorder. Manifestations of the disease include but are not limited to recurrent fever, livedoid rash, various cytopenias, stroke, immunodeficiency, and bone marrow failure. Symptoms often onset during early childhood, but some cases have been discovered as late as 65 years old.

Maria Virginia Pascual is a Spanish-American pediatric rheumatologist.

References

  1. ↑ "Still's Disease". MedicineNet. Retrieved 8 June 2017.
  2. ↑ Vastert SJ, Jamilloux Y, Quartier P, Ohlman S, Osterling Koskinen L, Kullenberg T, Franck-Larsson K, Fautrel B, de Benedetti F (2019). "Anakinra in children and adults with Still's disease". Rheumatology. 58 (Suppl 6): vi9–vi22. doi:10.1093/rheumatology/kez350. PMC   6878842 . PMID   31769856.
  3. ↑ De Benedetti F, Meazza C, Vivarelli M, et al. (May 2003). "Functional and prognostic relevance of the -173 polymorphism of the macrophage migration inhibitory factor gene in systemic-onset juvenile idiopathic arthritis". Arthritis Rheum. 48 (5): 1398–407. doi:10.1002/art.10882. PMID   12746913.
  4. ↑ https://www.tm.mahidol.ac.th/seameo/2017-48-suppl-2/2017-48-supp2-141.pdf [ bare URL PDF ]
  5. ↑ DeWitt, Esi Morgan; Kimura, Yukiko; Beukelman, Timothy; Nigrovic, Peter A.; Onel, Karen; Prahalad, Sampath; Schneider, Rayfel; Stoll, Matthew L.; Angeles-Han, Sheila; Milojevic, Diana; Schikler, Kenneth N.; Vehe, Richard K.; Weiss, Jennifer E.; Weiss, Pamela; Ilowite, Norman T.; Wallace, Carol A. (1 January 2012). "Consensus treatment plans for new-onset systemic juvenile idiopathic arthritis". Arthritis Care & Research. 64 (7): 1001–10. doi:10.1002/acr.21625. PMC   3368104 . PMID   22290637.
  6. ↑ Vastert, Sebastiaan J; De Jager, Wilco; Noordman, Bo; Prakken, Berent J; Wulffraat, Nico M (1 January 2012). "IL-1 receptor antagonist restores IL-18 NK cell axis in systemic JIA". Journal of Translational Medicine. 10 (Suppl 3): P45. doi: 10.1186/1479-5876-10-S3-P45 . PMC   3508836 .
  7. ↑ Wulffraat, NM; de Jager, W; Prakken, B; Kuis, W (1 January 2008). "Early effects of Anakinra in corticosteroid naïve SOJIA patients". Pediatric Rheumatology. 6 (Suppl 1): P29. doi: 10.1186/1546-0096-6-S1-P29 . PMC   3334087 .
  8. ↑ Gattorno, Marco; Piccini, Alessandra; Lasigliè, Denise; Tassi, Sara; Brisca, Giacomo; Carta, Sonia; Delfino, Laura; Ferlito, Francesca; Pelagatti, Maria Antonietta; Caroli, Francesco; Buoncompagni, Antonella; Viola, Stefania; Loy, Anna; Sironi, Marina; Vecchi, Annunciata; Ravelli, Angelo; Martini, Alberto; Rubartelli, Anna (1 May 2008). "The pattern of response to anti–interleukin-1 treatment distinguishes two subsets of patients with systemic-onset juvenile idiopathic arthritis". Arthritis & Rheumatism. 58 (5): 1505–1515. doi:10.1002/art.23437. PMID   18438814.
  9. ↑ http://www.ema.europa.eu/docs/en_GB/document_library/EPAR_-_Product_Information/human/001109/WC500031680.pdf [ bare URL PDF ]
  10. ↑ Singh-Grewal, D.; Schneider, R.; Bayer, N.; Feldman, B. M. (1 May 2006). "Predictors of disease course and remission in systemic juvenile idiopathic arthritis: Significance of early clinical and laboratory features". Arthritis & Rheumatism. 54 (5): 1595–1601. doi:10.1002/art.21774. PMID   16645998.
  11. ↑ Hoffman, F. "Background Information". Roche Group Media Relations. http://www.roche.com/med-ra-sjia.pdf.pdf Archived 2016-03-04 at the Wayback Machine
  12. ↑ Davies, Rebecca; Southwood, T.; Kearsley-Fleet, L.; Lunt, M.; Hyrich, K. (2015). "Standardized Mortality Rates are Increased in Patients with Severe Juvenile Idiopathic Arthritis". Oxford Journal of Rheumatology. 54 (1): i153.
  13. ↑ synd/1773 at Who Named It?
  14. ↑ G. F. Still. A special form of joint disease met with in children. Doctoral dissertation, Cambridge, 1896.
  15. ↑ Bywaters EG (March 1971). "Still's disease in the adult". Ann. Rheum. Dis. 30 (2): 121–33. doi:10.1136/ard.30.2.121. PMC   1005739 . PMID   5315135.
  16. ↑ Cimaz, R; Von, Scheven; Hofer, M (9 May 2012). "Systemic-onset juvenile idiopathic arthritis: the changing life of a rare disease" (PDF). Swiss Medical Weekly. 142: w13582. doi: 10.4414/smw.2012.13582 . PMID   22573189.
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