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Background: Antibodies against cyclic citrullinated peptide (anti-CCP) are considered to be specific for rheumatoid arthritis (RA). Objective: To assess the clinical significance of anti-CCP in a cohort of patients with [...]

Objective. Pharmacokinetic studies have shown that the biological effect of triamcinolone acetonide (TA) is equivalent to that of triamcinolone hexacetonide (TH), if used at double the dosage. In this study we compared the efficacy of intra-articular TA at a dose twice that of TH in symmetrically involved joints, in children with juvenile idiopathic arthritis (JIA).Method. Children with active arthritis and a similar degree of inflammation in two symmetrical joints were enrolled in the study. The symmetry was assessed by both clinical examination and synovial fluid analysis. The dose given was 1 mg/kg up to 40 mg of TH or 2.0 mg/kg up to 80 mg of TA. The identity of injected compound was blinded to the patient and to the physician.Results. Thirty-seven patients, 30 female, seven male, with JIA, entered the study. A total of 86 joints were injected. Twenty-one (53.8%) of the joints injected with TA relapsed first compared with only six (15.4%) of the joints injected with TH. In three (7.7%) relapse occurred simultaneously. Nine (23%) were still in remission after 24-month follow-up. The percentage of joints with lasting remission was higher with TH than with TA (80 vs 47.5% after 12 months and 63.6 vs 32.4% after 24 months, respectively; log rank test P = 0.003).Conclusion. Even when TA is given at higher doses, TH is more effective and should be considered the drug of choice for intra-articular treatment of JIA.

Objective. We analysed our experience with the use of iloprost for the treatment of critical ischaemic digits (ID) in children with connective tissue diseases (CTD) in order to assess its safety and efficacy.Methods. This was a retrospective analysis of paediatric patients with CTD who were treated with iloprost for critical ID resistant to conventional therapy. Information on demographics, clinical and laboratory features, the regimen of iloprost treatment and outcome were collected.Results. Fifteen patients (10 female, five male) treated one or more times with iloprost were included (total of 19 treatments). Six had juvenile systemic sclerosis, five had systemic lupus erythematosus, three had mixed connective tissue disease and one had cutaneous polyarteritis nodosa. Thirteen patients were already taking calcium channel blockers with no improvement; in two patients ID were the presenting signs of the disease without prior treatment. Eleven patients had more than two fingers involved; one child had involvement of all 10 fingers. Normal digital blood flow was achieved in 74% of treatments and significant improvement was noted in 26%. Fingertip necrosis was present in 11 patients (14 treatments). It healed completely in seven, improved in one and remained unchanged in six. Raynaud's phenomenon (RP) was present in 14 patients (17 treatments): in two no RP attack occurred during the follow-up period, eight improved both in the number of attacks per week and in the duration of each attack. Complete pain relief was observed in 10/17 treatments (59%) and there was a significant decrease in pain in the remaining seven. No major side-effects or withdrawal symptoms were reported. Minor side-effects reported include reversible headache (seven patients), hypotension or irritability (three), nausea/vomiting (two) and injection site reaction (one).Conclusions. Iloprost appears to be a safe and effective treatment for ischaemic digits and digital ulcers in children with CTD. In conjunction with immunosuppressive drugs, it has a potential role in preventing irreversible complications, such as digital gangrene and amputation.

Objective. To compare the efficacy and safety of intra-articular triamcinolone hexacetonide (TH) and triamcinolone acetonide (TA) in children with oligoarticular juvenile idiopathic arthritis (JIA).
Methods. One hundred and thirty joints of 85 patients undergoing intra-articular injections were randomly treated with either TH or TA depending on the availability of the drug. The efficacy of both treatments was evaluated prospectively in a blinded fashion. A good response was defined as a decrease in the articular score of ≥60% from baseline. Clinical, laboratory and immunological variables were noted in order to examine possible factors, other than treatment, predictive of the result.
Results. Seventy injections were performed using TH and 60 with TA. The two groups were comparable for clinical, immunological and laboratory characteristics. The rate of response was significantly higher with TH than with TA: 81.4% vs 53.3% (P = 0.001) at 6 months, 67.1 vs 43.3% (P = 0.006) at 12 months, and 60 vs 33.3% (P = 0.002) at 24 months.
Conclusion. At comparable doses TH appeared to be much more effective than TA for intra-articular use, in both short- and long-term follow-up. This result was not affected by disease duration or degree of local and systemic inflammation.

Temporomandibular joint (TMJ) involvement is quite frequent in juvenile idiopathic arthritis (JIA). We describe a 15-year-old girl with isolated TMJ arthritis presenting as a unique manifestation of JIA, and its successful treatment. She underwent arthroscopic synovectomy followed by intraarticular steroid injection. Early use of synovectomy and intraarticular steroids in TMJ arthritis may reduce pain, improve jaw function, and prevent irreversible deformities.

Objective. To increase the current knowledge of the outcome of juvenile systemic sclerosis (jSSc), which is currently limited.Methods. In order to investigate the patient outcome and prognostic factors, starting October 1994, we distributed questionnaires to 324 paediatric rheumatology centres.Results. Until 15 May 1998 responses from 46 centres were received, 34 of which returned completed questionnaires on a total of 135 patients. One hundred and twenty‐two of the 135 patients were Caucasian, 100 were female. The mean age at disease onset was 8.8 yr (s.d. ± 3.3 yr). The mean disease duration at the last follow‐up was 5 yr(s.d. ± 3.3 yr). At the last follow‐up the disease was still active and required medication in 82 patients, 36 had inactive disease on medication, and 16 were in remission. Ninety per cent of the living patients were fully active in daily life at the last follow‐up. Eight of the 135 patients had died. These patients had a median age at onset of the disease of 10.5 yr (range 6.7–15.8 yr). The median disease duration until death was 2 yr (range 1–8 yr). The causes of death were heart failure (five), renal failure (one), sepsis (one) and in one case the cause was not defined. The 1 yr survival rate was 99%, the 2 yr was 97% and the 4 yr was 95%.Conclusions. At a mean follow‐up of 5 yr, the current results show a favourable outcome in most patients with childhood onset jSSc and a significantly better survival than in the adult SSc patients.

Objective. To increase the current knowledge of the outcome of juvenile systemic sclerosis (jSSc), which is currently limited.
Methods. In order to investigate the patient outcome and prognostic factors, starting October 1994, we distributed questionnaires to 324 paediatric rheumatology centres.
Results. Until 15 May 1998 responses from 46 centres were received, 34 of which returned completed questionnaires on a total of 135 patients. One hundred and twenty-two of the 135 patients were Caucasian, 100 were female. The mean age at disease onset was 8.8 yr (s.d. ± 3.3 yr). The mean disease duration at the last follow-up was 5 yr(s.d. ± 3.3 yr). At the last follow-up the disease was still active and required medication in 82 patients, 36 had inactive disease on medication, and 16 were in remission. Ninety per cent of the living patients were fully active in daily life at the last follow-up. Eight of the 135 patients had died. These patients had a median age at onset of the disease of 10.5 yr (range 6.7-15.8 yr). The median disease duration until death was 2 yr (range 1-8 yr). The causes of death were heart failure (five), renal failure (one), sepsis (one) and in one case the cause was not defined. The 1 yr survival rate was 99%, the 2 yr was 97% and the 4 yr was 95%.
Conclusions. At a mean follow-up of 5 yr, the current results show a favourable outcome in most patients with childhood onset jSSc and a significantly better survival than in the adult SSc patients.

Photopheresis (ECP) is a new type of photochemotherapy, used for the treatment of oncological and autoimmune diseases. Lymphocytes are drawn from the patients by leukapheresis, treated with 8-methoxypsoralen (8-MOP) and ultraviolet light A (UVA) in an extracorporeal system and then reinfused. Skin exposure to 8-MOP and UVA (PUVA) has been shown to relieve cutaneous symptoms of graft-versus-host disease (GVHD) in bone marrow transplant (BMT) recipients. ECP, which is similar in some ways to PUVA, has been used in this study to treat four paediatric patients who developed chronic GVHD following BMT and in whom GVHD had failed to respond to conventional immunosuppressive therapy. Following ECP, skin lesions cleared almost completely and pulmonary function tests improved in two of three patients with cutaneous and lung involvement. Serum bilirubin and transaminases gradually normalized, and γGT decreased considerably in the remaining patient who had a severe cholestatic hepatopathy. The Karnofsky performance score increased to 90% in the three patients with positive responses to ECP and remained unchanged (40%) in the patient who did not respond. Immunosuppressive therapy was reduced in three patients and eventually discontinued in two. No significant side-effects were observed during the treatment. Our results suggest that ECP is a non-aggressive treatment that may benefit patients with chronic GVHD who do not respond to standard immunosuppressive therapy.

Objective.To examine the responsiveness of the disease activity measures more commonly used in juvenile chronic arthritis (JCA) clinical trials.Methods.Data were obtained from an open-label, non-controlled, multicentre trial designed to investigate the efficacy of methotrexate (MTX) in children with JCA. Outcome measures, including physician and parent global assessments, functional ability measures, articular variables, and laboratory indicators of systemic inflammation, were assessed at baseline and after 6 months of MTX treatment in 132 patients. Responsiveness of endpoint variables was evaluated by assessing the effect size (ES) and the standardized response median (SRM).Results.Physician and parent global assessments were the more responsive instruments, showing ES and SRM above 1.0. Erythrocyte sedimentation rate, C-reactive protein, functional status measures and articular variables showed intermediate responsiveness. Morning stiffness, haemoglobin and platelet count were the least responsive instruments.Conclusion.The results of our analysis indicate that subjective estimations of the disease activity, either by the physician or parents, are the most responsive instruments in the assessment of the therapeutic response in children with JCA. The responsiveness of outcome measures in JCA should be further investigated in prospective controlled studies.Keywords:Responsiveness, Assessment, Outcome measures, Juvenile chronic arthritis.

Photopheresis (ECP) is a new therapy for oncological and autoimmune diseases consisting in the reinfusion of 3-9x 109leukocytes, taken from the patient by leukapheresis, and treated in an extracorporeal system with 8-methoxypsoralen and ultraviolet light A. Nine patients affected by T cell immunomediated diseases (2 scleroderma, 1 chronic GVHD, 1 polyarteritis, 1 rheumatoid arthritis and 4 heart transplant patients with numerous episodes of acute rejection) were treated with ECP. Photopheresis was performed on 2 consecutive days every 3-4 weeks. All patients affected by autoimmune diseases experienced an improvement during treatment with ECP. In 2 of the 4 patients with heart transplant, rejection was reversed by photopheresis. No major side effects were observed during the treatment.In conclusion ECP is a safe and well tolerated therapy. Although the number of patients is small, ECP seems to be an effective modality in many diseases.

Systemic necrotizing vasculitis is uncommon in children and may be rarely associated with gangrene. We describe a 3-yr-old girl with parvovirus B19-induced necrotizing vasculitis whose digital gangrene was successfully treated with iloprost, a prostacyclin analogue.Key words: Parvovirus B19, Polyarteritis nodosa, Necrotizing Vasculitis, Iloprost.

Zulian F, Turra S, Bellettato M, Calore A, Gigante C. Monoarticular Juvenile Chronic Arthritis of the Shoulder. Report of a case and review of the literature. Scand J Rheumatol 1993; 22: 252-254.In 20% of the cases Juvenile Chronic Arthritis (JCA) has a monoarticular onset. Usually the inflammatory process spreads out to other joints with pauciarticular or polyarticular course. Very rarely the disease persists in one joint only and this is in about 70% of the cases, the knee. We describe a case of Monoarticular JCA with isolate and persistent involvement of the shoulder never reported in the literature. The clinical, pathologic features and different diagnoses arc discussed.

Background: Preclinical diastolic dysfunction is highly prevalent in the aging population, but mechanisms for progression into heart failure with preserved ejection fraction (HFpEF) are still obscure. Recently, microvascular endothelial inflammation and endothelial dysfunction (ED) were advocated as primum movens in the development of HFpEF. Purpose: We aimed to evaluate whether ED and arterial stiffness relate to diastolic and other structural and functional cardiac parameters. This was studied in patients with chronic kidney disease (CKD), known to be prone to diastolic dysfunction and left ventricular hypertrophy. Methods: Consecutive CKD patients, without concomitant cardiovascular disease, were included. Diastolic parameters were assessed by cardiac ultrasound using E/e´ ratio and left atrial volume index (LAVi). Also, left ventricular mass index (LVMi) and interventricular septum thickness (IVSd) were included. Endothelial function was evaluated by flow-mediated dilation (FMD) of the brachial artery induced by hyperaemia. Arterial stiffness was assessed by measuring carotid-femoral pulsed wave velocity (PWV). Results: After exclusion of patients with normal diastolic function (n=11), 52 patients (age 53.9 ± 12.8 years, 53.2% male) were assessed, of whom 36 underwent a second analysis after 3 months (total measurements 88). Mean creatinin clearance (eGFR) was 42.9 ± 23.2 ml/min/1.73m2. Comorbidities included arterial hypertension (90.4%) and diabetes mellitus (9.6%). Mean Framingham Heart score was 18.9% ± 18.7. Endothelial function was impaired (FMD 4.64% ± 2.61), and patients showed increased arterial stiffness (PWV 8.96 m/s ± 2.18). Ratio of E/e´ was elevated (>12) in 36.4% of measurements. LVMi was raised in 28.4%, and LAVi was elevated in 45.1%. Patients with E/e´>12 had impaired FMD (p=0.005) and elevated PWV (p=0.047). In bivariate correlation analysis, FMD correlated with E/e´ (r=-0.289, p=0.010) and with IVSd (r=-0.315, p=0.005). PWV did not show a relation with any of the diastolic indices (all p>0.05). In a multiple linear regression model, accounting for age, sex, smoking, eGFR, and PWV, FMD remained independently associated to E/e´ (ß=-0.279, p=0.011) and IVSd (ß=-0.232, p=0.026). Conclusions: In CKD patients with preclinical diastolic dysfunction, impaired endothelial function correlates with higher filling pressures and structural cardiac changes. This observation supports the paradigm that ED plays a role in the pathophysiology of diastolic dysfunction, even in an asymptomatic stage.

<it>Objective</it>. Juvenile localized scleroderma (JLS) includes a number of conditions often grouped together. With the long-term goal of developing uniform classification criteria, we studied the epidemiological, clinical and immunological features of children with JLS followed by paediatric rheumatology and dermatology centres. <it>Methods</it>. A large, multicentre, multinational study was conducted by collecting information on the demographics, family history, triggering environmental factors, clinical and laboratory features, and treatment of patients with JLS. <it>Results</it>. Seven hundred and fifty patients with JLS from 70 centres were enrolled into the study. The disease duration at diagnosis was 18 months. Linear scleroderma (LS) was the most frequent subtype (65%), followed by plaque morphea (PM) (26%), generalized morphea (GM) (7%) and deep morphea (DM) (2%). As many as 15% of patients had a mixed subtype. Ninety-one patients (12%) had a positive family history for rheumatic or autoimmune diseases; 100 (13.3%) reported environmental events as possible trigger. ANA was positive in 42.3% of the patients, with a higher prevalence in the LS-DM subtype than in the PM-GM subtype. Scl70 was detected in the sera of 3% of the patients, anticentromere antibody in 2%, anti-double-stranded DNA in 4%, anti-cardiolipin antibody in 13% and rheumatoid factor in 16%. Methotrexate was the drug most frequently used, especially during the last 5 yr. <it>Conclusion</it>. This study represents the largest collection of patients with JLS ever reported. The insidious onset of the disease, the delay in diagnosis, the recognition of mixed subtype and the better definition of the other subtypes should influence our efforts in educating trainees and practitioners and help in developing a comprehensive classification system for this syndrome.

<it>Objective</it>. The aim of this study was to define the clinical utility of infrared thermography in disease activity detection in localized scleroderma (LS). <it>Methods</it>. We retrospectively reviewed 130 thermal images of 40 children with LS and calculated the sensitivity and specificity of thermography, comparing clinical descriptions of the lesions and contemporary thermographs. The reproducibility of thermography was calculated by using the weighted kappa coefficient to determine the level of agreement between two clinicians who reviewed the thermographs independently. <it>Results</it>. The sensitivity of thermography was 92% and specificity was 68%. Full concordance between the two clinicians was observed in 91% of lesions, with a kappa score of 0.82, implying very high reproducibility of this technique. <it>Conclusion</it>. Our results demonstrate that thermography is a promising diagnostic tool when associated with clinical examination in discriminating disease activity, as long as it is applied to lesions without severe atrophy of the skin and subcutaneous fat. Further evaluation is needed to determine whether thermography can predict the future progression of lesions.