Laboratory Remission Research Articles

Case Report: Clinically amyopathic dermatomyositis presenting acutely with isolated facial edema.

A 45-year-old Asian man presented with acute-onset periorbital and facial edema associated with pyrexia. Muscle weakness was absent. Initial laboratory investigations showed an inflammatory reaction, while screening for infections was negative. Serum muscle enzyme levels were normal. He was hospitalized and treated empirically with antibiotics and corticosteroids, pending the result of facial skin and muscle biopsy. He showed a good clinical and laboratory response but an attempt to discontinue corticosteroids led to a prompt relapse of facial edema and pyrexia, associated with rising laboratory indices of inflammation. Biopsy findings were typical of dermatomyositis. Reintroduction of corticosteroid treatment resulted in complete clinical and laboratory remission. Facial edema as the sole clinical manifestation of dermatomyositis is extremely rare. There have been no previous reports of isolated facial edema in the setting of acute, clinically amyopathic dermatomyositis in adults. A high level of suspicion is required to make the diagnosis in the absence of myopathy and the hallmark cutaneous manifestations of the disease (heliotrope rash, Gottron papules).

REMISSION IN AXIAL SPONDYLOARTHRITIS: DEFINITION AND EVALUATION TOOLS (RECOMMENDATIONS OF THE SPONDYLOARTHRITIS STUDY GROUP OF EXPERTS, ALL-RUSSIAN PUBLIC ORGANIZATION «THE ASSOCIATION OF RHEUMATOLOGY OF RUSSIA»)

The paper gives the definition of remission in axial spondyloarthritides (axSpA), which has been developed by the Spondyloarthritis Study Group of Experts. The work used the Delphi technique. At stage 1, based on the analysis of the data available in the literature and on their own clinical experiences, the experts proposed some variants of the definition of remission and ways of its evaluation in patients with axSpA. At Stage 2, the definitions that had received at least 80% of the votes via anonymous voting were selected and adopted without further discussion. Those that had received an equal number of votes were reconsidered and additionally discussed; then there was repeat voting, by choosing the final definition. As a result of their discussion, the experts formulated the definitions of clinical laboratory and magnetic resonance imaging (MRI) remissions. They proposed the following remissions in axSpA: drug and drug-free, clinical laboratory, and MRI remissions, as well as a remission in the presence and absence of structural changes in the locomotor system, as evidenced by imaging techniques. Criteria for clinical laboratory remission and basic tools for its evaluation that can be used in real clinical practice and researches have been elaborated.

Case Report: Acute amyopathic dermatomyositis presenting with isolated facial edema.

A 45-year-old Asian man presented with acute-onset periorbital and facial edema associated with pyrexia. Muscle weakness was absent. Initial laboratory investigations showed an inflammatory reaction, while screening for infections was negative. Serum muscle enzyme levels were normal. He was hospitalized and treated empirically with antibiotics and corticosteroids, pending the result of facial skin and muscle biopsy. He showed a good clinical and laboratory response but an attempt to discontinue corticosteroids led to a prompt relapse of facial edema and pyrexia, associated with rising laboratory indices of inflammation. Biopsy findings were typical of dermatomyositis. Reintroduction of corticosteroid treatment resulted in complete clinical and laboratory remission. Facial edema as the sole clinical manifestation of dermatomyositis is extremely rare. There have been no previous reports of isolated facial edema in the setting of acute amyopathic dermatomyositis. A high level of suspicion is required to make the diagnosis in the absence of myopathy and the hallmark cutaneous manifestations of the disease (heliotrope rash, Gottron papules).

Hemolytic Uremic Syndrome: Single Centre Experience

Introduction: Hemolytic uremic syndrome (HUS) is a clinical syndrome characterized by non-immune microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. In this study, we aimed to evaluate patients diagnosed with HUS in our clinic. Method: Patients who were diagnosed with HUS as clinic and laboratory between August 2012 and October 2017 were included in our nephrology clinic. Biochemistry, haemogram, ANA, anti-Ds DNA, p-ANCA, c-ANCA, anti-GBM antibody, C3, C4 of patients were studied. In some cases, ADAMTS13 was studied and renal biopsy was performed. The demographic features, clinics, and treatments of our cases were reviewed. Results: A total of 18 patients were enrolled in the study, the average age of the patients was 48.3 (21-82) and 10 of the cases were female and 8 of the cases were male. Among the etiologic causes of HUS in patients; the complement factor B mutation, complement factor H polymorphism, breast cancer, herbal medicine, infections and idiopathic were detected. Primer glomerulonephritis was detected in 6 cases of renal biopsy. Conclusion: Clinical and laboratory remission was obtained in 13 patients. End-stage renal failure developed in 3 of our patients.

ROLE OF HUMAN DISKOIDIN DOMAIN RECEPTOR 1 IN THE PROGRESSION OF CHRONIC PYELONEPHRITIS IN CHILDREN

The purpose: to evaluate the role of collagen receptors Human Discoidin Domain Receptors (DDR1) as mediators of inflammation, proliferation and fibrosis in children with chronic pyelonephritis (CP), to reveal their relationship to the clinical form of the disease and the characteristics of its flow.
 Materials and methods: The levels of DDR1, transforming growth factor (TGF-β1), insulin-like growth factor (IGF-1) in the serum, β2 - microglobulin ( β2- MG) in the serum and urine were identified during the study of 40 children, ages 6 to 16 with CP in a state of clinical and laboratory remission.
 Results: Clinical and laboratory remission HP was associated with significant increased levels of DDR1 sera from long ill patients with frequent exacerbations, as well as 2-3 degree of activity last exacerbation, with family history. Found a strong inverse correlation between the levels of DDR1 and IGF-1, and the line with TGF-β1 and β2-MG of blood and urine. In patients with obstructive HP DDR1 level was significantly higher than in patients with non-obstructive clinical form.
 Conclusions: Increased serum DDR1 shows the progression of kidney damage with active fibrogenesis and inflammation in certain categories of patients with CP in a state of clinical and laboratory remission.

PECULIARITIES OF CYTOKINES AND THEIR PROGNOSTIC VALUE IN PATIENTS WITH CHRONIC GLOMERULONEPHRITIS

Introduction. Cytokines are important to take part in immunogenesis and progression of glomerulonephritis (GN), therefore the analysis of these indices in dependence of type of GN forms as prognosys results of treatment.
 Aim of the work is to determine the peculiarities of pro-inflammatory cytokines (TNFα, MCP-1, IL-17, -18, -23) and anti-inflammatory TGF- β1 and their association with form GN with nephritic syndrome (NS) and possibility use cytokines as prognostic markers.
 Materials and methods. Using IFA, the level of the above mentioned cytokines was studied in the blood serum of 117 patients with proliferative (33) and non-proliferative (84) CGN, NS forms. There was also analyzed the changes of these indices in the patients with subsequent clinical laboratory remission (59) and patients without one (58).
 Results. The GN, NS patients showed statistically higher level of the pro- inflammatory cytokines – TNFα, MCP-1, IL-17 (with more high indices last one in the proliferative forms of GN, and IL-17 in nonsensitive to immunotherapy patients). High level profibrogenic TGFβ, and the ratio TNFα /TGFβ reflect the prevalence of anti-inflammatory reactions and high activity of monocytes - macrophages and T-helpers17. The positive effect of the treatment associates with the statistically decreased TNFα, MCP-1 and TGFβ1 levels in the patients with proliferative forms GN; the TGFβ1 - for patients with non-proliferative GN forms.
 Conclusion. The changes cytokine profiles may be use as predictors of results the immunosuppressive therapy.

Ускладнення ендовезикальної корекції міхурово-сечовідного рефлюксу у дітей

Objective. To reduce the percentage of complications in children after intravesical correction of VUR. Material and methods. The clinical material covers 270 patients with uni- and bilateral VUR II-IV grades, aged from 9 months to 14 years. Patients with VUR were collected complaints and anamnesis, a clinical examination, general clinical laboratory examinations with an assessment of the functional status of the glomerular apparatus and the degree of nephrosclerosis, ultrasound of the urinary tract and bladder with Doppler effect, retrograde cystography, excretory urography, dynamic angiography and renography with 99mTc-DTPA, cystoscopy. At the stage of clinical and laboratory remission patients underwent intravesical restored urodynamics – STING (subureteric injection), HIT (hydrodistention implantation technique), Double HIT, and, if necessary, their combination. Results. In the early postoperative period 8 (2.96%) patients had a disorder of urodynamics with exacerbation of pyelonephritis, requiring more intensive and prolonged parenteral antibiotic therapy – more than 3 days. In 4 patients a dilatation of the ureter and pyelocaliceal system (PCS) with signs of urinary tract obstruction was revealed via the ultrasonography. These patients performed an intravesical internal stenting of ureter and PCS with JJ-stent for 1-3 months. In 2 patients in the late postoperative period an obstructive ureterohydronephrosis was diagnosed according to the urological X-ray examination, which required the ureterocystoneostomy. The clinical case of minimally invasive removal of concrement fragments from the left ureter after intravesical treatment of VUR III grade in both segments of the left double kidney in a 3-year-old patient, who was twice operated and the defect was successfully corrected. Obstructive uropathy after the injection of implant under the urethra orifice is the results of neuromuscular dysplasia of the urinary tract, which associated with fibrous tissue changes in the area of the uretero-vesical junction. Conclusions. The endoscopic method is the most optimal way of treating VUR in children and its complications. However, intravesical treatment of VUR, as well as other methods of surgical intervention, also has various complications.

Evaluation of Infliximab Therapy in Children with Crohn's Disease Using Trough Levels Predictors

Background: In adults, infliximab (IFX) levels correlate with disease activity, and antibodies to IFX (ATIs) predict treatment failure. We aimed to determine the association of IFX levels and ATIs with disease activity in a paediatric population. We prospectively collected blood, stool, and clinical data from 65 patients (age 10.5-15.1 years) with Crohn's disease (CD) before IFX administration, and measured IFX trough levels, ATIs, and faecal calprotectin levels (CPT). Samples were collected during maintenance therapy. We used multivariate analysis to identify the predictors of IFX levels. Summary: Lower levels of IFX were associated with ATIs positivity (OR 0.027, 95% CI 0.009-0.077). Higher C-reactive protein (CRP) level, erythrocyte sedimentation rate, and CPT levels were found in patients with lower IFX levels. The optimal combination of sensitivity (0.5) and specificity (0.74) for disease activity was calculated for IFX levels ≥1.1 µg/mL using CRP level <5 mg/L as a marker of laboratory remission. In a model that used CPT ≤100 µg/g as the definition of remission, the optimal IFX trough level was 3.5 µg/mL. No independent association between remission and ATIs was found in our study population. However, we found an independentz association between IFX levels and serum albumin levels (OR 1.364, 95% CI 1.169-1.593), p < 0.001. Key Messages: The paediatric population was similar to adult populations in terms of the association between IFX and ATIs as well as between IFX and disease activity.

Juvenile Idiopathic Arthritis in Adults: Long-Term Observation of Ukrainian Patients

The assessment of long-term outcome of functional disability and disease activeness in adult patients with juvenile idiopathic arthritis appears to be complicated due to the absence of a unified approach to the classification and estimation of disease activeness, as well as the loss of supervision over a patient because of remission or his/her transition from pediatric to adult rheumatic service. The objective of the research was to determine how adults with the history of juvenile idiopathic arthritis fulfill the classification criteria for adult rheumatic diseases, as well as to assess activeness of these diseases, the degree of functional disorders, and social activeness of patients in Ukraine. Materials and methods. Patients with juvenile idiopathic arthritis older than 18 years and with more than 3 years of disease duration living in different parts of Ukraine were included into the study. Data regarding sociodemographic features, fulfillment of adult classification criteria, Health Assessment Questionnaire, articular and extra-articular Juvenile Arthritis Damage Index and disease activity were analyzed.Results. We observed 122 adult patients with the history of juvenile idiopathic arthritis irrespective of the presence of active inflammation at the moment of the examination. This group included patients from different regions of Ukraine diagnosed with juvenile idiopathic arthritis during 1984-2013. An adult rheumatologist examined all patients and the diagnosis was revised according to the adult classification of rheumatic diseases. Typical diagnostic criteria for rheumatoid arthritis were estimated in 32.8% of patients, ankylosing spondylitis – in 31.1% of patients, undifferentiated arthritis – in 13.9% of patients, Still’s disease – in 4.9% of patients, psoriatic arthritis – in 0.8% of patients, steady clinical laboratory remission – in 16.5% of patients. Most patients (81.8%) with rheumatoid factor positive polyarticular juvenile idiopathic arthritis fell under rheumatoid arthritis criteria in adulthood, and in 85% of patients with enthesitis-related arthritis as well as 53.8% of patients with extended oligoarthritis ankylosing spondylitis developed in adulthood. 68.8% of patients with systemic juvenile idiopathic arthritis, 68% of patients with rheumatoid factor negative polyarthritic subtype and 55% of patients with enthesitis-related arthritis had disability and incapacitation. Minimal disorders of the patients’ general condition according to the Health Assessment Questionnaire in adult age were found in most subtypes of juvenile idiopathic arthritis classified according to the International League of Associations for Rheumatology (extended and persistent oligoarthritis, rheumatoid factor positive polyarthritis, systemic subtype); moderate disorders of the general condition were found in enthesitis-related arthritis and rheumatoid factor negative polyarthritis. Side effects of juvenile idiopathic arthritis according to the articular Juvenile Arthritis Damage Index included severe articular damage being most frequently found in systemic and rheumatoid factor positive polyarthritis subtypes of juvenile idiopathic arthritis, while side effects of juvenile idiopathic arthritis according to the extra-articular Juvenile Arthritis Damage Index included extra-articular damage being found in systemic and rheumatoid factor negative polyarthritis subtypes of juvenile idiopathic arthritis, that was confirmed by the assessment of physical health according to the Short Form Health Survey-36, which was the worst in patients with systemic (40.3±12.6) and rheumatoid factor negative polyarthritis (38.9±9.4) subtypes of juvenile idiopathic arthritis.Conclusions. Further research of remote consequences of juvenile idiopathic arthritis in adult age and long-term observation of such patients require a detailed study to improve diagnostics and provide adequate treatment of rheumatic diseases with juvenile onset in adult age.

URINE CYTOKINES ARE NONINVASIVE MARKERS OF CLINICAL AND LABORATORY REMISSION IN CHILDREN WITH CHRONIC PYELONEPHRITIS

THE AIM:to evaluate diagnostic significance of clinical and laboratory resistance in determining urine cytokines in patients with chronic pyelonephritis (CP).PATIENTS AND METHODS.This prospective study included 110 children with CP aged 6 to 16 years in a state of clinical and laboratory remission. The control group consisted of 20 apparently healthy children. Laboratory tests used to determine EGF, TGF-β1, IGF-1, β2 – MG, IL-4, IL-10, IL-17 and IL-12 by ELISA in the morning urine of patients.RESULTS.Comparison of cytokines values depending on the clinical form of the disease revealed the prevalence of levels of TGF-β1 and IL-17 in the urine of patients with obstructive CP. The highest uTGF-β1 / uCr and uIL-17 / uCr were obtained in patients, which have vesicoureteral reflux in anamnesis. Level uIL-10 / uCr almost 2 times was elevated in children with both clinical forms of CP. In patients with the disease experience up to 6 years, were identified unidirectional changes of urine cytokines – increase in both pro- and anti-inflammatory parameters. As the duration of the microbial-inflammatory processes more than 6 years there was a significant decrease in the levels uIGF-1 / uCr and uEGF-1 / uCr relative to the control group, with no significant changes in the uIL-12 / uCr and uIL-10 / uCr at continuing higher levels uTGF-β1 / uCr, uIL-17 / uCr, uIL-4 / uCr. In patients having one or less exacerbation of CP per year, regardless of the duration of the disease, no pathological changes in cytokine levels have been detected. Most of the studied parameters were dependent on renal function.CONCLUSION.Increased concentration of studied urine cytokines indicates the presence of a latent inflammatory process more than in half of studied patients.

COMBINATION OF THE ULCERATIVE COLITIS AND VON WILLEBRAND DISEASE (A CASE REPORT)

In the article the clinical observation of a rare combination of ulcerative colitis with von Willebrand’s disease in 33-year-old patient. Comorbidity require specific types of the disease, taking into account the peculiarities of its course, monitoring the main hematological parameters for the prevention of massive blood loss during the treatment of ulcerative colitis, helped damage colonic mucosa (Activity II by Schroeder) and the possibility of spontaneous gastrointestinal bleeding. The article demonstrated features of the diagnostic and treatment process, described diagnosis, assessment of severity of main and concomitant diseases, as well as the dynamics of the patient’s condition during treatment. The choice of treatment tactics was complicated because of the multi-directional impact of both diseases on hemostasis, but the definition of the main hematological parameters allowed to forecast a stable course and not to use drug therapy of von Willebrand disease. The organization of the medical process in ulcerative colitis, associated with von Willebrand’s disease, a comprehensive approach that includes Standard treatment for ulcerative colitis srednetyazhёlogo flow was used: systemic glucocorticosteroids mesalazine sulfosalazin locally and orally, as well as other products in accordance with current clinical guidelines. Close cooperation between two specialists (gastroenterologist and hematologist) in charge of one patient allowed to achieve clinical and laboratory remission

Looking Beyond Mucosal Healing: Effect of Biologic Therapy on Transmural Healing in Pediatric Crohn's Disease.

Crohn's disease is a chronic inflammatory disease characterized by a progressive transmural bowel damage leading to complications. Anti-TNFα therapy is effective in achieving mucosal healing (MH), but its efficacy on transmural inflammation has been poorly investigated. The aim of this study is to evaluate, in pediatric Crohn's disease, the efficacy of anti-tumor necrosis factor α agents in inducing transmural healing (TH) as assessed by ultrasonography (US). Children with Crohn's disease requiring anti-tumor necrosis factor α therapy were prospectively enrolled. Clinical activity, laboratory tests, endoscopic activity, and transmural disease assessed by small intestine contrast US (SICUS) were evaluated at baseline (T0) and then after 9 to 12 months of therapy (T1). We evaluated US quantitative and qualitative parameters: disease extension (centimeters), bowel wall (BW) thickness >3 mm, BW vascularity and stratification strictures, and prestenotic dilatation. TH was defined as a BW thickness <3 mm and normalization of all US parameters at T1. Thirty-two patients were included. Patients with mucosal healing (MH) showed a significant decrease of BW thickness and disease extension at T1 (4.3 ± 1.4 mm and 8 ± 6.3 cm versus 6.1 ± 2.3 mm and 13 ± 5 cm at baseline, respectively) (P < 0.001). Increased vascularity of the BW was found in 80% of patients at T0 and in 18% at T1 (P < 0.001). These parameters did not change in patients without MH, despite clinical and laboratory remission. The presence of stenosis and prestenotic dilatation did not modify in any group. A complete TH was achieved in 14% of patients, all of them showing complete MH. Biologics induce clinical and laboratory remission and MH in pediatric CD. Although caution is needed due to the small sample size, our data suggest that transmural inflammation also improves during therapy, but a complete TH is achieved only in a small percentage of patients.

Analysis of Complete Remission in Systemic Lupus Erythematosus Patients Over a 32‐Year Period

Systemic lupus erythematosus (SLE) is characterized by an unpredictable and fluctuating course. Although various methods have been developed to measure disease activity, there is still a lack of consensus about the optimal criteria for SLE remission. The principal aim of our study was to identify the number of lupus patients achieving a complete remission (implying that for 3 years there were no clinical or serologic features and no treatment with steroids and immunosuppressive drugs) in a single cohort of patients followed for a period of up to 32 years. In addition, we have identified patients in clinical but not serologic remission (known as serologically active, clinically quiescent disease [SACQ]) and vice versa. We were particularly interested to determine the factors associated with complete remission. Eligible patients were followed up in the University College Hospital Lupus cohort from January 1978 until December 2010 for a period of at least 3 years. Complete remission was defined as a period of at least 3 years with clinical inactivity (British Isles Lupus Assessment Group scores of C, D, or E only) and laboratory remission (no antibodies to double-stranded DNA and normal complement C3 levels), and being off-treatment with corticosteroids and immunosuppressants. Antimalarial and nonsteroidal antiinflammatory drugs were allowed. Of 624 lupus patients at our hospital, a total of 532 patients met the strict inclusion criteria for the study. Of these 532 patients, 77 patients (14.5%) achieved complete remission for at least 3 years, and 23 (4.3%) achieved complete remission for a minimum period of 10 years. Ten of these 77 patients were subsequently lost to followup, and, interestingly, flares occurred subsequently in 15 of the 67 remaining patients (22.4%). Three patients relapsed after the tenth year of remission. Forty-five patients (8.5%) fulfilled the requirement for SACQ, and 66 patients (12.4%) achieved only serologic remission. Our study indicated that 14.5% of lupus patients achieved a complete remission for 3 years. However, flares may continue to occur beyond 10 years of remission. Long-term followup of SLE is therefore mandatory.

Giant cell arteritis and polymyalgia rheumatica as first manifestation of typical pulmonary carcinoid tumor

Giant cell arteritis (GCA), a systemic vasculitis of unknown origin, may appear rarely as a paraneoplastic syndrome. Cases secondary to pulmonary neuroendocrine tumors have not been reported. A 75-year-old female developed prednisone-responsive GCA/polymyalgia rheumatica (PMR) shortly followed by syndrome of inappropriate antidiuretic hormone secretion. An 8 mm carcinoid lung tumor with positron emission tomography normal uptake was found. After a thoracoscopic tumor resection the patient experienced complete clinical and laboratory remission. This is the first report of GCA with PMR in the context of carcinoid lung tumor. It emphasizes the role of paraneoplastic vasculitis as a possible cause of GCA.

A novel genomic inversion in Wiskott-Aldrich–associated autoinflammation

A novel genomic inversion in Wiskott-Aldrich–associated autoinflammation

Sa1968 Post-Induction Adalimumab Drug Levels Predict Clinical and Laboratory Remission at Week 24 in Patients With Crohn's Disease

Sa1968 Post-Induction Adalimumab Drug Levels Predict Clinical and Laboratory Remission at Week 24 in Patients With Crohn's Disease

Практическая значимость оценки клеточного и гуморального иммунитета при сочетании осложнённого кариеса и пародонтита

Patients with caries and its complications in a combination with periodontitis were investigated by clinical and immune methods in our work. The interrelation between the clinical and immune indicators was revealed at complicated caries in a combination with average and heavy degree periodontitits accordingly. The recommendations were formulated for increase of treatment efficiency, reduction of the complex pathologies treatment terms, relapses prevention and prolongation of clinical and laboratory remission in addition to traditional medical complex in stomatologic practice. Results of our work will help to stop inflammatory and destructive process, stimulate osteogenesis, destroy odontogenic centers of infection and normalize immunity in oral cavity.

P426. Post-induction adalimumab drug levels predict clinical and laboratory remission at week 24 in patients with Crohn’s disease

P426. Post-induction adalimumab drug levels predict clinical and laboratory remission at week 24 in patients with Crohn’s disease

Remission in ankylosing spondylitis and axial spondyloarthritides: A modern understanding of the problem

The paper discusses possible approaches to determining remission in ankylosing spondylitis (AS) and other axial spondyloarthritides (axSpA). At present, there is no single definition of the concept of remission in axSpA and AS, which is due to both the diversity of manifestations of axSpA and a large number of tools to measure disease activity and the nonsimultaneous change in the degree of clinical and laboratory symptoms, signs of acute inflammation, as evidenced by imaging techniques, and signs of progressive structural changes in the locomotor apparatus in the same patient. Clinical, laboratory, magnetic resonance imaging, and radiographic remissions in a patient cannot be in time, which in turn affects the choice of optimal therapy. The case report demonstrates problems with treatment correction in a patient who has achieved clinical and laboratory remission in the presence of persistent inflammatory signs in the locomotor apparatus, as shown by imaging techniques. Since the guidelines for the follow-up and treatment of patients with nonradiographic axSpA and AS are similar today, the paper considers remission as a general problem for all subtypes of axSpA.

Experience in the use of tocilizumab in patient with systemic juvenile idiopathic arthritis and type 1 diabetes

научный биометрический журнал, который знакомит читателя с клиническими рекомендациями и особенностями применения лекарственных и вакцинных средств у детей, предоставляет исчерпывающую информацию о воздействии лекарственных средств на плод, о проводимых в