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Query: UMLS:C0003864 (arthritis)
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Juvenile rheumatoid arthritis or, more correctly, juvenile chronic polyarthritis with its many clinical manifestations can be separated into the Still-syndrome with acute beginning, high fever and a high percentage of extra-articulalar, i.e. visceral symptoms, and the chronic polyarthritis in the more strict sense with non-visceral symptoms. The subsepsis allergica should be regarded as a subseptic first stage of the Still syndrome. The Still-syndrome implies a systemic disease mainly of the reticulo-endothelial system, with carditis, nephropathy, recurrent erythemas, and a progressing polyarthritis. Later symptoms are amyloidosis, chronic nephritis, myo- and pericarditis, and artheriitis necroticans. Predominanly the involvement of the kidneys is the reasons for the high mortality rate of 13%. Chronic polyarthritis in the strict sense is similar in children and adults, though in children rheumatic factors are rarely detected. The exsudative form of arthritis tends to cause early deterioration. Joint symptoms are distributed asymmetrically and show locally inflammed growth otherwise less common in Still-syndrome. Spondylitis cervicalis rapidly causes ankylosis. Atlanto-axial-arthritis with consequent atlanto-axial dislocation can be the reason for neurological disturbances. Juvenile mono- or oligo-arthritis often turns into polyarthritis; but for joints the prognosis is more favourable. In contrast, rheumatoid iridocyclitis as found in 22% of the cases causes unfavourable complications because symptoms are not noticed in time so that treatment is often too late. Juvenile spondylitis ankylosans begins with a peripheral arthritic stage which is not easily distinguished from chronic polyarthritis. The male sex, mono- or oligoarthritis of the outer extremities, pain in the heel, atlanto-axial-arthritis, iridocyclitis, and a positive HLA of 27 give a diagnostic clue. -- Characteristics of the therapy will be discussed.
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PMID:[Juvenile rheumatoid arthritis and related collagen diseases. Clinical aspects (author's transl)]. 1 66

Careful study of inflammatory and immunologic parameters in JRA and other connective tissue diseases of childhood has contributed to our current understanding of these diseases. Examination of serum for acute phase reactants, and antibodies to immunoglobulin antigens and nuclear antigens, combined with radiologic evaluation of symptomatic joints and, when indicated, examination of joint fluid or synovial membrane, may confirm the diagnosis of JRA. Changes in inflammatory indicators can be used as an index of adequacy of treatment. An ANA positivity in the child with pauciarticular arthritis should always prompt frequent slit-lamp examinations for asymptomatic iridocyclitis. The role played by auto-antibodies in the pathogenesis of JRA is unknown, as is the possible effect of proteins like CRP on the regulation of the inflammatory process in these children.
Arthritis Rheum 1977 Mar
PMID:Serologic studies in juvenile rheumatoid arthritis: a review. 9 62

We followed 210 cases of JRA closely for 14 years. Thirty-six patients (17.2%) developed iridocyclitis; it most frequently occurred in young females (0-4 years) with monarticular or pauciarticular form of the disease. In 30% of the patients iridocyclitis was first detected after 16 years of age. Forty-two percent had active iridocyclitis on entry. By combining quantitative antinuclear antibody titers with age, sex, and time on onset of arthritis, it has been possible to define the risk of developing iridocyclitis in individual patients. Our approach was effective in detecting iridocyclitis in new cases and exacerbations of the disease in established cases. Forty-four percent of patients with iridocyclitis had one or more identifiable early signs or symptoms. Iridocyclitis in 36% of patients did not respond to more than 6 months of intensive topical treatment with corticosteroids and mydriatics. Despite this statistic, patients had a better outcome than those experiencing the 50% incidence of blinding complications cited in earlier studies. Cataract and band keratopathy occurred in only 19% and 11% of our group, respectively. Only one case of chorioretinopathy was found in 173 patients who had received antimalarials; a significant number of posterior subcapsular cataracts were found in 75 patients who had received systemic corticosteroids. Keratoconjunctivitis sicca developed in 3 patients with iridocyclitis. Surgical treatment of cataracts, band keratopathy, and glaucoma achieved uniformly discouraging results.
Arthritis Rheum 1977 Mar
PMID:The ocular manifestations of juvenile rheumatoid arthritis. 26 99

Juvenile rheumatoid arthritis is a diverse group of diseases that includes systemic-onset, polyarticular, and pauciarticular types. Appreciation of the different types and their hallmarks is particularly important to accurate diagnosis, which is determined by exclusion of other known disease entities in children with chronic arthritis (more than three months' duration). Therapy should be directed at the arthritis per se (synovitis), at the extra-articular manifestations, and at the whole child. Salicylates provide the most satisfactory control of the arthritis per se and of the systemic manifestations in most cases. Iridocyclitis should be managed in consultation with an ophthalmologist. Patients should not be regarded as invalids or restricted needlessly. The prognosis for children with juvenile rheumatoid arthritis is good. In most patients, the disease remits without causing permanent joint damage.
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PMID:Juvenile rheumatoid arthritis. 29 41

In the present study we have reconfirmed an earlier observation of the association of iridocyclitis with limited joint involvement in children with JRA. Children who develop iridocyclitis are predominantly girls whose sera are positive for antinuclear antibodies. Iridocyclitis was found to be asymptomatic at onset. Only periodic slit lamp examinations will detect the majority of cases. Iridocyclitis began a median of 2 years after onset of arthritis. Although most episodes occurred within 7 years after onset of arthritis, the risk of this development may never be absent in these children, even after they enter adulthood. In the present study, 16% of the afflicted children experienced either unilateral or bilateral blindness.
Arthritis Rheum 1977 Mar
PMID:Clinical patterns of chronic iridocyclitis in children with juvenile rheumatoid arthritis. 31 21

A study was made, in co-operation with several gastroenterology and rheumatology centres, of the clinical and genetic characteristics (HLA B27) of 50 patients suffering from both inflammatory bowel disease (38 Crohn's disease (CD), 12 ulcerated colitis (UC)) and ankylosing spondylitis (AS), the latter diagnosis being established according to the New York criteria. 20 CD (52.6%) and 8 UC (66.7%) patients were HLA B27 positive. The presence of HLA B27 was studied in relation to clinical parameters, such as first occurrence of symptoms of AS or inflammatory bowel disease (IBD), a history of peripheral arthritis, iridocyclitis, and a positive history of AS or IBD. Our patients were found to have heterogeneous clinical features: on one side of the spectrum a group of cases was distingiushed with the typical characteristics of idiopathic AS, often being HLA B27 positive. On the other side a smaller group of HLA B27 negative patients was observed, with severe intestinal inflammatory pathology, lacking most of the typical clinical features of idiopathic AS ('secondary' form of AS). Finally, between these two extremes a group of patients was found with less pronounced clinical or genetic characteristics. These different clinical and histocompatibility patterns suggest a mixed aetiopathogenesis of AS in IBD patients. Such a 'syndrome' of AS might harbour both idiopathic AS and forms of AS 'secondary' to the intestinal inflammatory pathology.
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PMID:Ankylosing spondylitis and inflammatory bowel disease. III. Clinical characteristics and results of histocompatibility typing (HLA B27) in 50 patients with both ankylosing spondylitis and inflammatory bowel disease. 62 2

An epidemic form of arthritis has been occurring in eastern Connecticut at least since 1972, with the peak incidence of new cases in the summer and early fall. Its identification has been possible because of tight geographic clustering in some areas, and because of a characteristic preceding skin lesion in some patients. The authors studied 51 residents of three contiguous Connecticut communities -- 39 children and 12 adults -- who developed an illness characterized by recurrent attacks of asymmetric swelling and pain in a few large joints, especially the knee. Attacks were usually short (median: 1 week) with much longer intervening periods of complete remission (median: 2.5 months), but some attacks lasted for months. To date the typical patient has had three recurrences, but 16 patients have had none. A median of 4 weeks (range: 1-24) before the onset of arthritis, 13 patients (25%) noted an erythematous papule that developed into an expanding, red, annular lesion, as much as 50 cm in diameter. Only 2 of 159 family members of patients had such a lesion and did not develop arthritis (P less than 0.000001). The overalll prevalence of the arthritis was 4.3 cases per 1,000 residents, but the prevalence among children living on four roads was 1 in 10. Six families had more than 1 affected member. Nine of 20 symptomatic patients had low serum C3 levels, compared to none of 31 asymptomatic patients (P less than 0.005); no patient had iridocyclitis or a positive test for antinuclear antibodies. Neither cultures of synovium and synovial fluid nor serologic tests were positive for agents known to cause arthritis. "Lynne arthritis" is thought to be a previously unrecognized clinical entity, the epidemiology of which suggests transmission by an arthropod vector.
Arthritis Rheum
PMID:Lyme arthritis: an epidemic of oligoarticular arthritis in children and adults in three connecticut communities. 83 38

We followed 210 cases of juvenile rheumatoid arthritis closely for eleven years. Thirty-six of the 210 patients (17.2%) developed iridocyclitis. Iridocyclitis was seen most frequently in young female patients (0 to 4 years) with the monoarticular or pauciatricular form of the arthritis. However, 30% of the patients developed uveitis after 16 years of age. Although 61% of patients had a noncontributory ocular history on entry, 42% had active uveitis on entry. Our approach was effective in detecting uveitis in new cases and exacerbations of uveitis in established cases. Forty-four percent of patients with uveitis had one or more identifiable signs or symptoms, such as red eye, ocular pain, decreased visual acuity, or photophobia, in order of decreasing frequency. Even after early detection and prompt treatment, 41% of cases of uveitis did not respond to more than six months of intensive topical treatment with corticosteroids and mydriatics. Despite this, there was a dramatic decrease in the 50% incidence of blinding complications of uveitis cited in earlier studies. Cataract and band keratopathy occurred in only 22 and 13% of our group, respectively. We used chloroquine or hydroxychloroquine in 173 of 210 cases and found only one case of chorioretinopathy attributable to these drugs. Systemically administered corticosteroids were used in 75 of 210 cases; a significant number of posterior subcapsular cataracts was found. Typical keratoconjunctivitis sicca developed in three of the uveitis cases. This association with uveitis and JRA was not noted previously. Surgical treatment of cataracts, band keratopathy, and glaucoma achieved uniformly discouraging results.
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PMID:Ocular manifestations of juvenile rheumatoid arthritis. 107 93

One hundred and twelve well-studied patients with a prior diagnosis of juvenile rheumatoid arthritis were differentiated into seven clinically distinct subgroups, including a group in whom recognizable ankylosing spondylitis had developed by time of follow-up. An apparent increased prevalence of HLA-B27 in the entire series (26%) was clearly related to its increased prevalence in only two subgroups: patients whose disease had progressed to overt ankylosing spondylitis (five of five patients) and boys with pauciarticular arthritis whose disease would be consistent with early ankylosing spondylitis (11 of 18 patients). There were no significant associations of B27 with systemic onset JRA, polyarticular JRA, pauciarticular JRA in girls, or JRA with chronic iridocyclitis. The only other significant alterations found were increased prevalences of HLA-A2 and HLA-BW15 in patients with polyarticular disease without identifiable rheumatoid factor. This study emphasizes that the clinical disorders included under the category of juvenile rheumatoid arthritis represent more than a single disease and that this heterogeneity must be considered in interpreting studies such as those of histocompatibility typing.
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PMID:Histocompatibility antigens in childhood-onset arthritis. 127 Nov 90

The human herpes virus 6 (HHV 6) may induce not only the wellknown condition of exanthem subitum, but also a number of more common (cf. Part 1) or rare, even previously unknown, clinical manifestations. Part 2 of this paper deals with the more rarely observed manifestations. These include complications of ARD (sinusitis, otitis media, bronchial pneumonia) hepatitis, encephalitis or Pfeiffer's disease (mononucleosis-like syndrome). In individuals with a relevant disposition (genetic HLA/DR type?) initiation or (re-)activation of rheumatoid arthritis (JCA = juvenile chronic arthritis) or chronic iridocyclitis may occur. Although, on account of the high prevalence of vaccination in our population (approximately 95%), prenatal infections are extremely rare, they may manifest in a severe "septic" form (fatalities have occurred) or may lead to neurological deficits (comparable with cytomegalovirus infection). To date, no specific therapy (e.g. gammaglobulin, virostatics) or reliable preventive measures (e.g. vaccination) are available.
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PMID:[Infections with herpesvirus 6--really only "exanthema subitum"? Part 2: Rare or unknown disease pictures]. 133 53


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