Knee pain as a reason for referral to a paediatric rheumatologist: A retrospective study.

AIM: Knee pain is the most frequently reported type of lower extremity pain among children and adolescents. The objective of this study was to establish the features distinguishing inflammatory knee pain from non-inflammatory knee pain and to determine the specific variables to consider in suspected juvenile idiopathic arthritis (JIA). METHODS: A retrospective chart review was performed among children with knee pain evaluated through paediatric rheumatology consultations at a single centre between 2012 and 2019. RESULTS: Among the 262 children, 32 patients (12.2%) were diagnosed with JIA, 46 patients (17.6%) presented with inflammatory knee pain (IP) of an origin different than JIA, and 57 patients (21.7%) with non-inflammatory knee pain (NIP). In 127 cases (48.5%), no musculoskeletal disorder was diagnosed (NDD). The presence of limping, joint swelling, decreased passive range of motion and decreased active range of motion of the knee joint were registered more frequently in the JIA group compared to the other three groups. Multivariate analysis showed that a family history of autoimmune diseases and pain in other joints were associated with inflammatory pain. Increased pain after physical activity, pain only in the knee joint and absence of limping were predictors of NIP. The risk factors of JIA were limping and an erythrocyte sedimentation range of ≥10 mm after an hour. In the NDD group, the risk factors included no increase in pain after physical activity, serum C-reactive protein level < 5 mg/L and normal musculoskeletal ultrasound image. CONCLUSIONS: The majority of children with knee pain referred to a paediatric rheumatologist do not have arthritis. Knee pain alone, as a chief complaint, rarely leads to a final JIA diagnosis. Further studies are necessary in order to design the appropriate diagnostic and therapeutic algorithms.

Exceptional manifestation of polyautoimmunity in a very young girl - a case report.

Polyautoimmunity is defined as the presence of more than one autoimmune disease in a single patient. The exact pathogenic mechanisms responsible for the coexistence of distinct autoimmune diseases within an individual have not been clearly explained. We report a case of a very young girl with the extremely rare co-existence of four distinct autoimmune diseases i.e. juvenile idiopathic arthritis, type 1 diabetes mellitus, coeliac disease and autoimmune hepatitis, recognized based on validated international classification criteria. The best to our knowledge there has been no case reporting coexistence of these particular four disorders in an individual. Moreover, all these diseases occurred during first three years of life, which also cause that case unique. Molecular studies of human leukocyte antigen (HLA) class II in our patient showed the presence of the HLA DRB1*01, HLA DRB1*03, HLA DQB1*02, HLA DQB1*05 molecules, which may suggest immunogenetic links between those autoimmune diseases. The presented case highlights the importance of active screening for other autoimmune diseases, if a patient with one autoimmune disease manifests with new or nonspecific symptoms.

The profile of polyunsaturated fatty acids in juvenile idiopathic arthritis and association with disease activity.

We investigated the association between dietary intake of n-3 and n-6 polyunsaturated fatty acids (PUFAs), serum profiles, and immune and inflammatory markers in juvenile idiopathic arthritis (JIA) in relation to onset, activity, and duration. A total of 66 JIA patients and 42 controls were included. Serum PUFA levels were assessed by gas-liquid chromatography-mass spectrometry, a dietary intake by 7-day dietary record method, and IL-6, IL-10, and IL-17A levels using ELISA. Dietary PUFA intake did not differ between the JIA group and controls. Intakes of n-6 and n-3 PUFA and serum levels were not associated. Levels of total n-6 PUFA and linoleic acid (LA) were higher in inactive JIA than in active JIA. Patients with active and short-lasting disease (less than 3 months from diagnosis) had significantly lower levels of arachidonic acid (AA) and docosahexaenoic acid (DHA) than the control. Serum α-linolenic acid (ALA) levels were significantly higher in poly-JIA than in oligo-JIA and in controls. We found significantly higher serum IL-10 levels in JIA than in controls. Serum n-6 and n-3 levels were significantly negatively correlated with active joint count, erythrocyte sedimentation rate, and C-reactive protein and positively with platelet count. Our study presents the low levels of AA and DHA in the active phase of short-lasting JIA, particularly poly-JIA, and the relationship between n-6 and n-3 PUFA and classic markers of inflammation. PUFAs may contribute to the pathogenesis of JIA and support a necessity to identify new targets suitable for successful interventional studies in JIA patients.

Complex Regional Pain Syndrome type I with atypical scintigraphic pattern--diagnosis and evaluation of the entity with three phase bone scintigraphy. A case report.

Complex Regional Pain Syndrome (CRPS) is a neurological disorder of unknown etiology which may lead to severe disability. Its diagnosis is very difficult and based on diagnostic criteria which have been changing over last years. Still, there is no golden standard in diagnosis of this entity. Three-phase bone scan is a widely used diagnostic modality which has been proved useful in CRPS evaluation. The syndrome may present various scintigraphic patterns. Different diagnostic modalities can also be helpful when CRPS is suspected including plain film radiography, magnetic resonance imaging and ultrasonography. Multidisciplinary approach is necessary for proper and quick diagnosis. We present a case of CRPS in 12-year-old girl in whom the diagnosis was based on the bone scan.

The clinical profile of Kawasaki disease of children from three Polish centers: a retrospective study.

Kawasaki disease (KD) is one of the most common vasculitides of childhood. The aim of this retrospective study is to determine the incidence of KD and to evaluate its presenting symptoms, clinical course, laboratory tests, and treatment in patients with complete KD and incomplete KD at three pediatric rheumatology centers in Poland from January 2011 to December 2012. A total of 27 Caucasian children (12 boys and 15 girls) with median age of 3 years (range 4 months-12 years) were included in this study. The incidence of complete versus incomplete KD was 17 (63 %) versus 10 (37 %) children, respectively. Patients with incomplete KD significantly less presented cervical lymphadenopathy (20 vs. 88.2 %; p = 0.00075), changes in extremities (30 vs. 76.5 %; p = 0.04), and bilateral nonpurulent conjunctivitis (60 vs. 100 %; p = 0.01). Cardiac assessments show that the majority of patients with KD have not got coronary artery aneurysms (CAA). The median time from the onset of symptoms to intravenous immunoglobulin (IVIG) infusion was 7 days for complete KD and 11 days for incomplete KD. IVIG delay in the incomplete KD had no effect on the incidence of CAA. In conclusion, there were no differences in demographic features, age of onset, and laboratory tests of patients with complete and incomplete KD. Patients with incomplete KD significantly rarely presented cervical lymphadenopathy, changes in extremities, and conjunctival injection. Electrocardiography is a sensitive test to recognize cardiac involvement in the acute phase of KD. Despite the fact that incomplete forms of presentation often delay diagnosis, in most patients treatment with IVIG can avoid complication of CAA.

Clinical remission in juvenile idiopathic arthritis after termination of etanercept.

Biologicals are very effective for inhibiting disease progression in active juvenile idiopathic arthritis (JIA). To date, there have been no recommendations on how and when to stop therapy with TNF inhibitors. Our objective was to analyze characteristics and the disease course of JIA patients who discontinued etanercept due to achievement of inactive disease. Data of 39 patients with JIA from two clinical pediatric rheumatology centers in Bydgoszcz and Lublin (Poland) were analyzed retrospectively. All patients discontinued etanercept due to a remission on treatment. Etanercept was started after a mean 33.7 ± 36 (range 3-137) months of disease. The mean duration of therapy with etanercept was 34.7 ± 16.7 (range 6-72) months, with a mean duration of remission on medication 21.3 ± 9.6 (range 4-42) months before withdrawal of etanercept. The mean duration of remission after etanercept discontinuation was 14.2 ± 12.1 (range of 1-60) months. Only 12/39 (30.8 %) patients did not develop a disease exacerbation until the end of the study. Early flares, that is less than 6 months after termination of etanercept, were observed in 15/39 (38.5 %) patients. Twelve (30.8 %) patients restarted etanercept after exacerbation-all patients responded satisfactorily. Our data show that etanercept discontinuation in a substantial proportion of JIA patients results in early disease exacerbation. In many cases, reintroduction of etanercept is needed. Patients, in whom etanercept was restarted, responded satisfactorily.

Etanercept treatment in juvenile idiopathic arthritis: the Polish registry.

BACKGROUND: To evaluate the long-term safety and efficacy of etanercept treatment in Polish patients with juvenile idiopathic arthritis (JIA). MATERIAL/METHODS: The study involved patients, fulfilling the JIA criteria of the International League of Associations of Rheumatology (ILAR), who were started on etanercept therapy after methotrexate and other synthetic disease-modifying antirheumatic drugs (DMARDs) had proven ineffective. Patient data were collected in an electronic registry. Disease improvement was assessed based on Giannini's criteria. RESULTS: The statistical analysis involved 188 patients. Significant improvement was observed in all clinical and laboratory parameters after the first month of therapy and was maintained in the following months. ACR Pediatric 30, 50, 70, 90, and 100 improvement was observed in 81.4%, 65.9%, 27.5%, 16.2%, and 15%, respectively, of patients after 3 months and in 94.7%, 88.4%, 62.1%, 34.7%, and 26.3%, respectively, after 24 months of treatment. Throughout the 72-month safety observation period, 1162 adverse events were reported; the exposure-adjusted AE rate was 2.96 per patient per year. CONCLUSIONS: In patients with various subtypes of JIA resistant to conventional DMARD treatment, etanercept resulted in significant and long-lasting improvements in disease activity. Combination treatment with etanercept and a DMARD was well tolerated.

Identification of dendritic cells in the blood and synovial fluid of children with Juvenile Idiopathic Arthritis.

Childhood chronic arthritis of unknown etiology is known collectively as juvenile idiopathic arthritis (JIA) and consists of heterogeneous subtypes with unique clinical patterns of disease. JIA is the commonest rheumatic disease in children and may still result in significant disability, with joint deformity, growth impairment, and persistence of active arthritis into adulthood. Basic research is rather focused on rheumatoid arthritis, and this lead to small number of publications considering JIA. In this study we examine, by flow cytometry, the expression of dendritic cells (DCs) in the peripheral blood and synovial fluid of children with active JIA in a group of 220 patients. We reveal a significant decrease in the percentage of immature DCs in the blood of patients compared to control children. Surprisingly, we found higher percentages of mature circulating dendritic cells. Both populations of DCs, immature and mature, were accumulated in patients' synovial fluid. We also confirmed the presence of CD206+/CD209+ in JIA samples, which can represent a population of macrophages with dendritic cells morphology. Our results support the thesis that dendritic cells are crucial in the induction and maintenance of autoimmune response and local inflammation during juvenile idiopathic arthritis.

[Diagnostics of joint pain in children--controversies]. / Diagnostyka bólów stawów u dzieci--kontrowersje.

Musculoskeletal symptoms can be a manifestation of varied conditions and ailments which are not connected with rheumatic diseases. The aim of the article is to present some controversial elements of differential diagnosis of joint pain in children. Insidious course of the disease can be a cause of diagnostic errors and delays in introducing effective treatment. The article recalls differentiation between rheumatic diseases and neoplastic diseases, osteonecrosis, fibromyalgia and growing pains.

[Rheumatological symptoms masking neoplasm in children]. / Objawy reumatologiczne jako przepowiadajace schorzenia nowotworowe--obserwacje wlasne.

UNLABELLED: Fevers, arthritis, myalgia and skin changes are the most typical features of rheumatic diseases, although one should remember that the same symptoms could mask neoplasm. This study shows that some patients with rheumatological symptoms treated in the Department of Lung Diseases and Children Rheumatology, Medical University of Lublin were finally diagnosed with neoplasm. In focus is the initial phase of the patients' illnesses. MATERIAL AND METHODS: We analyzed retrospectively the case histories of all patients admitted to the department between 1997 and 2005 (1560 hospitalizations). An oncological disease was diagnosed in 9 cases: leukemia in 4 children (acute lymphoblastic leukemia-ALL- in 3 cases, acute non-lymphoblastic leukemia-ANLL- in 1 case), Hodgkin's disease in 1 child, bone tumours in 2 children, liver tumour in 1 child and a tumour of the central nervous system in 1 child. CONCLUSIONS: The cases described should draw the physicians' attention to the fact that in the initial phase an oncological disease may be masked by rheumatological symptoms.

Prevalence of asthma and some respiratory symptoms in the years 1995 and 2001 in schoolchildren from rural regions of Poland.

The aim of our study was to estimate the prevalence of asthma and some respiratory symptoms and diseases in schoolchildren from rural regions of Poland in 2001 and to compare these data with previous estimations in 1995. Repeated cross-sectional epidemiological studies were performed among 594 primary schoolchildren in 1995 and 541 in 2001 using the same standardized questionnaire. Lifetime prevalence of "doctor's-diagnosed asthma" increased significantly from 3.4 % in 1995 to 9.6 % in 2001. This trend may be due to the real increase in the prevalence of asthma and also may be a result of better physician's diagnosis and/or better parents' health education. A substantial increase of asthma-related symptoms (post-exercise breathlessness, wheezing and dyspnoea) was also observed between these years (8.3-17.7 %, 6.2-13.2 % and 7.6-13.3 %, respectively). These results suggest that asthma in Polish schoolchildren is still underdiagnosed.

[Vasculitis with coagulopathic complications in the course of Coxsackie A9 infection in a 13-year-old boy]. / Zapalenie naczyn z zespolem wykrzepiania wewnatrznaczyniowego w przebiegu zakazenia wirusem Coxsackie A9 u 13-letniego chlopca.

Viral infections are well known factors responsible for vasculitis. In our report we present the case of a 13-year-old boy with a history of life-threatening vasculitis complicated with coagulophathy. The only possible etiological agent was Coxsackie A9 infection. We were unable to find a report of a similar case in the available literature.

[Role of mycoplasma pneumoniae infection in aetiopathogenesis of juvenile idiopatic arthritis]. / Udzial zakazen Mycoplasma pneumoniae w etiopatogenezie mlodzienczego idiopatycznego zapalenia stawów.

UNLABELLED: Although more and more is known about chronic autoimmune diseases, attempts to establish one trigger factor have been unsuccessful. The role of endogenic factors is beyond doubt. But it is emphasized that environmental factors are necessary to cause the disease. Infections are taken under consideration as trigger mechanism in the development of autoimmune diseases including chronic arthritis. Both numerous viruses and bacteria are among the microorganism mentioned. We considered it sensible to conduct research on Mycoplasma pneumoniae infections in a group of patients with juvenile idiopathic arthritis (JIA). MATERIALS: 19 patients diagnosed with JIA aged between 6-17 were investigated for Mycoplasma pneumoniae infection whose blood was examined for antibodies against Mycoplasma pneumoniae in class IgG and IgM. The control group comprised 20 children of similar age admitted to hospital with digestive tract complaints. METHODS: Serologic tests were made in serum. Marking of antigens of class IgM and IgG were made by Elisa method using commercial kits produced by Scientific Point. Quantitative calculations of a level of antigens were done using appropriate standards, positive and negative serum of reference standard and calibration curve. RESULTS: In 11 patients positive reaction for Mycoplasma pneumoniae in class IgG was observed and only in 2 in class IgM with low titer. In the control group positive reaction was observed in 3 children (15%). The fact that 58% of patients were infected (contact either with Mycoplasma pneumoniae) indicates that in the group of our patients with JIA, infections with these bacteria might have had a role in triggering the disease.

[A case of relapsing polychondritis with severe involvement of the lower airway in 10 years old boy]. / Nawrotowe zapalenie chrzastki z powaznym zajeciem dolnych dróg oddechowych u dziesiecioletniego chlopca.

Relapsing polychondritis is a very rare disease of the connective tissue in children. Inflammation may affect all cartilaginous structures. The study presents the case of a 10 years old boy with bilateral conchitis as a prominent symptom of the disease. In our patient lower airways were seriously affected. The detection of the degree of the disease in respiratory tract was facilitated to a large degree by pulmonary function tests and high resolution computed tomography. Histopathological picture of the inflamed cartilage of the tracheobronchial tree finally confirmed the diagnosis.