?Erinnerungen an meine kranken H?nde��

Alexej von Jawlensky (1864-1941), one of the most important expressionist painters and a member the artist group "The Blue Four", suffered from severe rheumatoid arthritis. He was the first painter in the twentieth century to create extensive series of paintings especially of human faces. The medical history of Jawlensky as documented in his letters, is a harrowing document of a great artist who suffered from rheumatoid arthritis at a time when medical treatment was limited to physical therapy, pain medication and other relatively ineffective modalities, including the unnecessary extraction of teeth. Jawlensky's disease was characterized by a rapidly progressive course with severe pain, rapid onset of disability and ending up with complete immobilization and paralysis for several years until his death.The artistic processing and sublimation of his illness and suffering resulting in a series of over 1,000 small format meditations are the impressive and touching example of creative coping with rheumatoid arthritis. The meditations are unique in the history of art and often compared with icons. However, knowing the medical condition of Jawlensky these paintings can also be seen as metaphors of suffering and in each image the great physical and mental effort is reflected in the artistic details. Therefore, his art agent Galka E. Scheyer formulated in a letter to him: "You are the painter of the human soul. I know of no other modern painter of the human soul."     

Severe aortic insufficiency in juvenile chronic arthritis

Valvular heart disease is rare in patients with juvenile chronic arthritis. We describe a 27-year-old woman with the systemic-onset form of juvenile chronic arthritis in whom aortic insufficiency necessitated valve replacement. Nodules were seen on both the aortic and anterior mitral leaflets at surgery, and histopathologic evaluation of the excised aortic leaflets demonstrated nonspecific changes similar to those described in rheumatoid valve disease causing aortic insufficiency in adults with rheumatoid arthritis. We believe that this is the first reported case of aortic insufficiency in systemic-onset juvenile chronic arthritis in which the pathologic condition of the valve can be attributed to the underlying disease.     

Studies with the NBT test in various clinical forms of juvenile rheumatoid arthritis]

Fifty-six healthy neonates and children as well as 33 patients with juvenile rheumatoid arthritis and other diseases of the locomotor system were examined using a modification of the NBT test. In healthy newborn infants there was observed an increase in the number of NBT-positive cells between the first and tenth days. The test results obtained for the 33 patients with juvenile rheumatoid arthritis were positive in 73%, with positive results being obtained especially in those patients where the disease was in its second stage of activity. Highest values were obtained in patients with a predominantly articular manifestation of the disease. Stimulative effects produced by immune complexes are considered to be chiefly responsible for this. However, this method of examination is of small practical value from a differential diagnosis point of view.     

Lyme disease: musculoskeletal manifestations

A previously unrecognized musculoskeletal syndrome led to the recognition of this "new" infectious disease. Several distinct patterns of musculoskeletal involvement can be seen throughout the course of untreated Lyme disease. Diffuse, nonspecific muscle achiness and stiffness can be seen early to be followed by characteristically brief, recurrent episodes of LIMP and transient arthritis that help to differentiate LD from other arthropathies. Chronic oligoarticular arthritis is seen in a few. Lyme arthropathy most closely resembles the reactive arthropathies. In a few cases, LD has mimicked other connective tissue disorders such as juvenile rheumatoid arthritis, rheumatoid arthritis, myositis, and scleroderma. It would appear that the full spectrum of musculoskeletal LD is still being defined.     

Profilographic studies with 99mTc-pertechnetate in joint diseases in children

The inflammatory activity of joints can be stated after intravenous administration of 99mTc-pertechnetate by demonstration of an enrichment of isotope. In 85 out of 111 patients (77%) a good accordance between profilogram and clinical finding could be seen. "False positive" results could be found in juvenile rheumatoid arthritis, ulcerative colitis, reactive arthritis and juvenile gout. In some cases of juvenile rheumatoid arthritis the clinical finding is later obvious than the demonstration by nuclear medicine. "False negative" results mainly can be seen at the wrists of patients with juvenile rheumatoid arthritis and tendosynovitis as well as in already existing radiological changes of the bones. Differential diagnostical evidences of profilography cannot be expected with the investigation technique used here. It, however, may contribute to assure the diagnosis in non-inflammatory joint diseases ("psychogenic rheumatism", lymphatic oedema).     

The temporomandibular joint from the rheumatologic viewpoint

Temporomandibular joint affections in rheumatic disease are described. They were seen in inflamed joint diseases, mostly in rheumatoid arthritis, and less so in juvenile rheumatoid arthritis, psoriatic arthritis, Reiter's syndrome, ankylosing spondylitis, and collagen vascular disease. In osteoarthrosis, the temporomandibular joint affection occurs frequently but pain seems to occur most infrequently. We must bear in mind that a painful temporomandibular joint could be a symptom of myalgia, especially fibrositis syndrome with its painful tendon.     

Acute hypoxic respiratory failure as the first manifestation of systemic-onset juvenile rheumatoid arthritis in a child

Systemic onset juvenile rheumatoid arthritis is the most common rheumatologic disorder of childhood. Pleuropulmonary manifestations are rare in children in this multiorgan disease, and are usually not severe. The diagnosis of systemic onset juvenile rheumatoid arthritis is made by exclusion, in the presence of clinical findings constellation. We present the case of an 8-year-old girl who developed acute hypoxic respiratory failure as the first manifestation of systemic onset juvenile rheumatoid arthritis, then severe respiratory relapse 16 months later. Clinical and radiological improvement were achieved at both times after high dose pulse methylprednisolone therapy.     

OPHTHALMOLOGICAL SCREENING IN JUVENILE ARTHRITIS: SHOULD THE FREQUENCY OF SCREENING BE BASED ON THE RISK OF DEVELOPING CHRONIC IRIDOCYCLITIS?

Chronic iridocyclitis (CI) may complicate juvenile chronic arthritis(JCA) and if left untreated may cause significant ocular impairment.It is usually not symptomatic and diagnosis relies on slit lampbiomicroscopy. It is unclear how often children with JCA shouldbe screened for this complication. From a review of the literature,the following recommendations could be made, although theserequire scientific validation. All children with JCA shouldhave at least one adequate slit lamp examination as soon aspossible after diagnosis of the arthritis. If CI is detectedthen appropriate treatment and follow up should be determinedby the ophthalmologist. If CI is not detected initially, allchildren with JCA should be screened by slit lamp examinationsevery 3–4 months for the first 5 years after arthritisonset. After 5 years, CI screening could be stopped. The onlyexceptions would be arthritic children at low risk for CI, includingsystemic onset JCA, juvenile spondyloarthropathy and juvenileonset rheumatoid arthritis, who do not need to be screened ifthe initial slit lamp examination is normal. KEY WORDS: Chronic iridocyclitis, Chronic anterior uveitis, Arthritis, Child, Juvenile chronic arthritis, Juvenile rheumatoid arthritis, Screening     

Validity of reported pain as a measure of clinical state in juvenile rheumatoid arthritis.

Reported pain is one valid indicator of clinical state which should be used in the assessment and management of children with juvenile rheumatoid arthritis. Parents' reports of children's pain for 101 children with juvenile rheumatoid arthritis differed significantly by type of juvenile rheumatoid arthritis. When multiple regression was used separately for pauciarticular and polyarticular classifications of juvenile rheumatoid arthritis a measure of clinical state, which reflected joint activity, morning stiffness, and overall disease activity as rated by the doctor, had a significant and independent effect upon pain reported by parents. The child's age was not significantly related to the pain reported by parents. Children's pain reports did not differ significantly between older and younger children.     

HLA studies in IgM rheumatoid-factor-positive arthritis of childhood.

The clinical course of children with IgM rheumatoid-factor-positive chronic arthritis closely resembles that of seropositive rheumatoid disease in adults. The frequency of HLA DR4 is known to be increased in adults with seropositive rheumatoid arthritis, but the first major report on childhood arthritis did not suggest a correlation, though only 8 seropositive cases were included. Fifty-two children with polyarthritis beginning before the age of 16 who persistently carried IgM rheumatoid factor were studied. HLA DR4 was present in 60% of these children but was found in only 29% of 93 patients with seronegative juvenile arthritis and 27% of a normal adult population. These results suggest immunogenetic similarities in patients with seropositive arthritis irrespective of age of onset.     

Evaluation of the ILAR criteria for juvenile idiopathic arthritis.

OBJECTIVE: A pediatric rheumatology committee of ILAR has proposed new classification criteria for chronic childhood arthritis. The umbrella term "juvenile idiopathic arthritis (JIA)" was chosen and the disease was subdivided into 7 categories. Evaluation for these criteria is under way. METHODS: We analyzed data of 200 consecutive children with rheumatic diseases. RESULTS: In total 172 patients fulfilled criteria for JIA. Twenty-seven of these (15.7%) had to be grouped into the category "other arthritis": 16 met criteria for 2 categories; the other 11 did not fit into any category. CONCLUSION: We suggest minor changes in the classification in order to classify 24 of these 27 patients into one of the specific categories without losing the claim for homogeneity in the different patient groups. Among the 44 patients with rheumatoid factor negative polyarthritis, 26 resembled oligoarthritis, with an extended oligoarticular joint pattern of 5 to 8 involved joints within the first 6 months. 18 had positive antinuclear antibodies, and 7 chronic uveitis. For these patients the introduction of a separate category "extended oligoarthritis at onset" should be considered to establish comparable patient groups.     

Elderly onset of adult Still's disease: Report of a case

Summary Adult Still's disease is a variant of juvenile rheumatoid arthritis occurring most frequently in women 16–35 years of age, but rarely in elderly people. We describe a 75-year-old woman who was considered as having adult Still's disease.     

Predictive value of synovial fluid analysis in juvenile chronic arthritis.

To investigate the value of synovial fluid analysis in predicting the articular evolution of juvenile chronic arthritis, synovial fluid from 29 patients with oligoarticular onset juvenile chronic arthritis were examined prospectively. The patients were subsequently classified after a three year period of observation as having polyarticular (10 patients) or pauciarticular (19 patients) disease. The synovial fluid samples were analysed for total and differential white blood cell count, total protein, beta 2 microglobulin, and total complement activity. For comparison, synovial fluid samples from 95 patients with adult onset rheumatoid arthritis were also analysed. In patients with polyarticular disease polymorphonuclear cells and beta 2 microglobulin concentrations were higher than in the patients with pauciarticular disease (80 (29.2) v 58.1 (25.3), and 3.6 (1.2) v 2.2 (0.5) mg/l, respectively), but there was no significant difference from the patients with rheumatoid arthritis. Synovial fluid analysis may be useful in predicting the evolution of juvenile chronic arthritis and improve definition of its subtypes.     

Recurrent new-onset uveitis in a patient with rheumatoid arthritis during anti-TNFalpha treatment

Inflammation involving the uveal tract of the eye, termed uveitis, is frequently associated with various rheumatic disease, including seronegative spondylarthropathies, juvenile rheumatoid arthritis, Crohn's disease and Beh?et's disease. Scleritis and keratitis may be associated with rheumatoid arthritis and systemic vasculitides such as Wegener's granulomatosis. Immune-mediated uveitis can have a chronic relapsing course and produce numerous possible complications, many of which can result in permanent vision loss. Treatment typically includes topical or systemic corticosteroids with cycloplegic-mydriatic drugs and/or noncorticosteroid immunosuppressants, but often there is an insufficient clinical effectiveness. Anti-TNFalpha therapy is promising in the treatment of sight threatening uveitis, particularly in patients with Beh?et's disease. However, there have been also reports of new-onset uveitis during treatment of joint disease with TNFalpha inhibitors. We describe a case of new-onset uveitis in a patient with rheumatoid arthritis during therapy with etanercept at first and infliximab at last. Although we cannot exclude uveitis as linked to rheumatoid arthritis, it is unlike that the uveitis arises when the joint disease is well controlled. The hypothetical paradoxical effect of anti-TNF is here discussed.     

Stem cell transplantation for autoimmune disease: progress and problems

The current status of stem cell transplantation in rheumatoid arthritis, juvenile chronic arthritis, systemic lupus erythematosus, and systemic sclerosis are reviewed. From a large European bone marrow transplant registry, a birds' eye view of stem cell transplantation for autoimmune disease can be obtained. Among 43 rheumatoid arthritis patients, 35 juvenile chronic arthritis patients, 34 systemic lupus erythematosus patients, and 58 systemic sclerosis patients who underwent stem cell transplantation, initial responses in most patients were good to excellent. Although initial transplant related mortality was low for rheumatoid arthritis, somewhat higher rates for juvenile chronic arthritis, systemic lupus erythematosus, and systemic sclerosis may be falling with modifications in the stem cell transplantation regimens. In rheumatoid arthritis and systemic lupus erythematosus treatment, the criteria for patient selection are still not clear and the therapeutic regimens for stem cell transplantation (and whether follow-up treatment is necessary) are not fully defined. In juvenile chronic arthritis, responses are encouraging although little fully published data beyond that from the European Bone Marrow Transplant Registry exist. In systemic sclerosis, criteria for patient selection and a limited number of stem cell transplantation regimens have been agreed on and controlled trials are underway.     

Chronic myeloid leukemia in a woman with Still's disease treated with 198Au synoviorthesis

A 32-year-old woman presented with chronic myeloid leukemia 20 years after 2 198Au synoviortheses to her knees for severe juvenile rheumatoid arthritis. She progressed to blast phase of her disease and died from complications of therapy. The literature on rheumatoid arthritis, its therapy, radioisotopes and leukemia is reviewed.     

Lymphocyte nucleoli in the peripheral blood and knee joint effusions in patients with rheumatoid arthritis

Since the activation of nucleoli in lymphocytes reflected by the presence of nucleoli with more or less distinct nucleolonemata (compact nucleoli in the light microscope) in these cells is considered as a marker of the autoimmune disease development, nucleoli of lymphocytes were investigated in the peripheral blood (22 investigations) and knee joint fluid (52 investigations) of juvenile and adult persons with rheumatoid arthritis. The results clearly indicate that the investigated joint exudates possessed a higher number of "active" lymphocytes (lymphocytes with compact nucleoli) in comparison with the peripheral blood. This observation can be interpreted as an accumulation of stimulated immunocompetent cells in the target organ.     

A specific HLA-DP beta allele is associated with pauciarticular juvenile rheumatoid arthritis but not adult rheumatoid arthritis.

Nonradioactive sequence-specific oligonucleotide probes specific for the HLA-DP beta locus have been used in a simple dot-blot format to type samples amplified by the polymerase chain reaction from 44 patients with pauciarticular juvenile rheumatoid arthritis, 32 patients with adult rheumatoid arthritis, and 50 random controls. The sequences of four new DP beta alleles derived from these patients and controls are reported, bringing the total number of alleles identified thus far to 19. The DPB2.1 allele is significantly increased in juvenile rheumatoid arthritis patients over controls; this allele is not increased in patients with adult rheumatoid arthritis. The association of juvenile rheumatoid arthritis with the DPB2.1 allele is independent of linkage with previously defined HLA-D region markers of disease. Analysis of the DPB2.1 sequence shows that it differs from the nonsusceptible DPB4.2 allele by only 1 amino acid at position 69 in the beta 1 domain.     

Bronchiolitis obliterans in a case of juvenile rheumatoid arthritis presented with pneumomediastinum

We present a case of bronchiolitis obliterans associated with juvenile rheumatoid arthritis in whom pneumomediastinum was the presenting manifestation. Diagnosis of bronchiolitis obliterans was made on the basis of the clinical history, pulmonary function tests and high-resolution computerized tomography scan findings. Pneumomediastinum resolved in a few days following high-dose nasal oxygen. This case shows that bronchiolitis obliterans can occur in patients with juvenile rheumatoid arthritis. Complicating pneumomediastinum in such cases should be considered as an indication of underlying bronchiolitis obliterans.