Recurrent Arthritis Research Articles

Does an Internal Joint Stabilizer and Standardized Protocol Prevent Recurrent Instability in Complex Persistent Elbow Instability?

Does an Internal Joint Stabilizer and Standardized Protocol Prevent Recurrent Instability in Complex Persistent Elbow Instability?

Infections With Enterohepatic Non-H. pylori Helicobacter Species in X-Linked Agammaglobulinemia: Clinical Cases and Review of the Literature.

The genus Helicobacter is classified into two main groups according to its habitat: gastric and enterohepatic. Patients with X-linked agammaglobulinemia (XLA) appear to be associated with invasive infection with enterohepatic non-Helicobacter pylori species (NHPH), mainly H. cinaedi and H. bilis. Such infections are difficult to control and have a high potential for recurrence. The spectrum of illnesses caused by these species includes recurrent fever, bacteremia, arthritis, osteomyelitis, cellulitis, abdominal abscesses, and pyoderma gangrenosum-like ulcer. The presence of these Helicobacters is particularly difficult to diagnose and eradicate, as they are very fastidious bacteria and present resistance to several types of antibiotics. We report two clinical cases of XLA patients infected with H. bilis. These infections were chronic in these patients and could not be eradicated in one of them. We also review the cases of enterohepatic non-Helicobacter pylori species (NHPH) in patients with this inborn error of immunity.

Clinical, immunological and genomic characteristics of children with X-linked agammaglobulinemia from Kerala, South India

X-linked agammaglobulinemia (XLA) is an X-linked recessive primary immunodeficiency disorder caused due to a pathogenic variant in the Bruton tyrosine (BTK) gene with an incidence of 1:379,000 live births and 1:190,000 male births. Patients affected with XLA present with recurrent infections of the gastrointestinal and respiratory tracts. Here we report the first case series of 17 XLA patients of 10 South Indian families with a wide spectrum of clinical and genetic features. In our cohort, patients presented mainly with recurrent pneumonia, gastrointestinal infection, otitis media, pyoderma, abscesses, empyema, arthritis, and osteomyelitis. Using next-generation and Sanger sequencing we have identified 10 unique pathogenic and likely pathogenic variants in 17 patients. This encompasses three nonsynonymous, two stop-gain, two frameshifts, two structural, and one splicing variant, out of which two of them are novel. Based on the type of variant, patients had variable clinical features and treatment responses. We have also evaluated Btk protein expression for six patients in comparison to the healthy individuals and determined mosaic Btk expression patterns in four mothers. We have also performed family screening in 6 families using Sanger sequencing and identified 19 carriers for the variant. The diagnosis for the patients led to the proper treatment i.e. 15 patients were on intravenous immunoglobulin (IVIG) and the other two had successful hematopoietic stem cell transplantation (HSCT). Unfortunately, two of our patients died due to sepsis, while on IVIG. We envision the present study could help in better understanding of patients with XLA and help in family screening and prenatal diagnosis. To the best of our knowledge, this is the largest case series of patients affected with XLA from South India.

Abstracts from the ISSAID 2021 Periodic Congress

Abstracts from the ISSAID 2021 Periodic Congress

Examination of 18 cases of recurrent pseudogouty arthritis

Examination of 18 cases of recurrent pseudogouty arthritis

Assessment of dental anxiety and fear levels of pediatric patients diagnosed with familial mediterranean fever

Objective: Familial Mediterranean Fever (FMF) is an autoinflammatory disease characterized by recurrent fever, arthritis, and inflammation of the serous membranes, with an autosomal recessive with recurrent attacks and is a disease that begins in childhood and lasts a lifetime. In this study, it was aimed to evaluate the relationship between disease activity and dental fear-anxiety, and dental behaviour level in children with FMF who had pain and hospital experience in early childhood. Materials and Methods: The study included 207 children ranging in age from 5 to 15 years; 93 patients with FMF and 114 healthy controls. Dental anxiety and dental fear were measured using the Frankl Scale and Children’s Fear Survey Schedule-Dental Subscale (CFSS-DS). Age, sex, disease severity activity, and duration of illness were examined. Results: There was no statistical difference in dental anxiety and fear between the FMF and healthy group (p>.05). However, there was a statistically significant difference in the effect of disease severity activity on the CFSS-DS. The anxiety and fear in FMF patients was similar to healthy children, which was low. Conclusions: Dental anxiety and fear level in FMF patients was similar to healthy children, which was low. It is necessary to avoid the idea that all chronic diseases will cause dental anxiety and fear, and they should be evaluated according to the nature of the chronic disease.

Diagnosis and Treatment of Gout Arthritis

Gout is a heterogeneous, often familial, metabolic disease associated with abnormal deposits of uric acid in tissues and initially characterized by recurrent acute arthritis, usually monoarticular, and later by chronic deforming arthritis. Urate deposition occurs when serum uric acid is saturated (that is, at greater than 6.8 mg/dL [404.5 mcmol/L]). Hyperuricemia is caused by excess or underexcretion of uric acid, sometimes both. The disease is especially common in the Pacific islands, for example, the Philippines and Samoa. Acute gouty arthritis is sudden in onset and often occurs at night. It may develop without a clear precipitating cause or may follow a rapid increase or decrease in serum urate levels. Common precipitants are excess alcohol (especially beer), changes in medications that affect urate metabolism, and, in hospitalized patients, fasting before medical procedures. This literature review presents gout arthritis, symptoms and signs in general to the prognosis of this disease.

Tofacitinib effectiveness in Blau syndrome: a case series of Chinese paediatric patients

ObjectiveBlau syndrome (BS), a rare, autosomal-dominant autoinflammatory syndrome, is characterized by a clinical triad of granulomatous recurrent uveitis, dermatitis, and symmetric arthritis and associated with mutations of the nucleotide-binding oligomerization domain containing 2 (NOD2) gene. Aim of this study was to assess the efficacy of tofacitinib in Chinese paediatric patients with BS.MethodsTofacitinib was regularly administered to three BS patients (Patient 1, Patient 2, and Patient 3) at different dosages: 1.7 mg/day (0.11 mg/kg), 2.5 mg/day (0.12 mg/kg), and 2.5 mg/day (0.33 mg/kg). The clinical manifestations of the patients, magnetic resonance imaging results, serological diagnoses, therapeutic measures and outcomes of treatments are described in this report.ResultsThe clinical characteristics and serological diagnoses of all BS patients were greatly improved after the administration of tofacitinib treatment. All patients reached clinical remission of polyarthritis and improvements in the erythrocyte sedimentation rate (ESR) and levels of C-reactive protein (CRP) and inflammatory cytokines.ConclusionTofacitinib, a Janus kinase (JAK) inhibitor, is a promising agent for BS patients who have unsatisfactory responses to corticosteroids, traditional disease-modifying antirheumatic drugs, and biological agents.

M155 AUTOINFLAMMATORY DISORDER, IMMUNODEFICIENCY, OR BOTH?

Autoinflammatory disorders result from inborn errors causing innate immune system dysregulation. Clinical features include recurrent fever, rash, arthritis, and lymphadenopathy in the absence of infection. This can be contrasted with primary immunodeficiency, wherein a lack of functional immune response leads to recurrent infections. Nuclear factor-κ B essential modulator (NEMO) deficiency causes a spectrum of disease which can manifest with features of both autoinflammation and immunodeficiency. We report a challenging case with a working diagnosis of NEMO deficiency.

P03 Not everything is lupus

P03 Not everything is lupus

Osteomyelitis on MR imaging as a key predictor of recurrent septic arthritis of the shoulder.

To investigate the clinical and radiologic predictors of postoperative recurrent septic arthritis of the shoulder (SAS) using multivariable analysis. Forty-three patients (mean age, 65years; 24 women) who underwent surgery for SAS between January 2011 and October 2019 were retrospectively enrolled. An orthopedic surgeon assessed clinical (age, sex, comorbidity, duration from symptom onset to MR imaging and surgery, surgical method, antibiotic usage), laboratory (serum white blood cell [WBC] count, C-reactive protein [CRP] level, synovial cell count), and surgical findings (culture/biopsy results). Two musculoskeletal radiologists evaluated MR imaging findings (bone marrow [reactive bone marrow edema, osteomyelitis, osteochondral erosion] and soft tissue [synovitis, bursitis, muscle edema, abscess] abnormalities). Recurrent SAS was evaluated at ≥ 12months postoperatively. Univariable and multivariable analyses were performed to determine the best predictor of recurrent SAS. The overall recurrent SAS rate was 33% (14/43). On univariable analysis, mean age (without recurrence vs. recurrence: 68 vs. 59years, p = .04), mean duration from symptom onset to surgery (18 vs. 25days, p = .02), serum WBC count (12,000 vs. 9,000 cells/mL3, p = .04), CRP level (13 vs. 6mg/L, p = .01), and osteomyelitis on MR imaging (p < .01 for both readers) significantly differed between patients with and without recurrence; on multivariable analysis, only osteomyelitis on MR imaging was significantly associated with recurrent SAS for both readers (p = .02 and .01 for each reader respectively). The inter-reader agreement was good (κ = .62-1.0) for all MR imaging findings, except for muscle edema (fair, κ = .37). Osteomyelitis on MR imaging was the best predictor of recurrent SAS. • Osteomyelitis on preoperative MR imaging was the best predictor associated with recurrent septic arthritis of the shoulder on multivariable analysis including clinical, laboratory, and MR findings. • In multivariable analyses focused on each bone marrow abnormality, with adjustment for clinical and laboratory parameters, the presence of reactive bone marrow edema and osteochondral erosion on MR imaging showed no significant association with recurrent septic arthritis of the shoulder.

Autoinflammation leading to autoimmunity in adult-onset Still\u2019s disease: more than simple coincidence?

BackgroundAdult-onset Still’s disease (AOSD) should be considered in the differential diagnosis of patients with endocarditis, with or without a cardiac decompensation.Case presentationWe report the case of a 68-year-old Caucasian male diagnosed with AOSD after an initial acute manifestation of endocarditis with severe aortic acute manifestation of endocarditis with severe aortic insufficiency. The histological findings revealed Libman–Sacks endocarditis. He was treated with the IL-1 receptor inhibitor anakinra. Two years later the patient developed a symptomatic dilated cardiomyopathy with reduced ejection fraction (23.5%) and functional anti-beta-1-adrenergic receptor antibodies, which was initially treated with plasmapheresis; anakinra was maintained. While his AOSD symptoms responded well, our patient presented with recurrent arthritis in multiple joints, dual-energy CT showed urate deposition compatible with a gouty arthropathy. Over 7 years, he presented with recurrent episodes of arthritis and the adjustment of dosages of colchicine and febuxostat was needed. In 2018, our patient died due to a deterioration of his underlying cardiac disease.ConclusionsOnly two cases with initial endocarditis prior to AOSD diagnosis have been published, and we are not aware of any other cases reporting -β1AR-Ab development with DCM and gout in the setting of AOSD treated with anakinra.

Early and advanced stages in palindromic rheumatism patients: Test characteristics of three classification criteria and discrimination potential

Aim of the workIt is to identify the classification characteristics of diagnostic criteria in the early (≤3 year) and advanced stage (>3 year) of palindromic rheumatism (PR). Patients and methods269 patients of PR were selected out of those with recurrent intermittent arthritis and were divided into two groups with ≤3 year (n = 99) and >3 year (n = 170) disease duration. The clinical characteristics and the positive incidence according to 3 classification criteria: Pasero and Barbieri (1986); Hannonen et al. (1987) and Guerne and Weisman (1992) were compared. Furthermore, the positive incidences of each criterion were calculated. ResultsThe mean age of the patients was 39.2 ± 10.8 years and M:F 1.2:1. The agreement of the Hannonen et al. (1987) and Guerne and Weisman (1992) criteria was the highest with 0.63 of κ value. When the positive incidences by each criteria were compared between groups of the ≤3 year and >3 year, both Hannonen et al criteria (76.8% and 91.2%) and Guerne and Weisman (62.6% and 85.8%) were significantly different (p = 0.001). The criterion that showed the obvious difference between early and advanced stages were as follow: (Pasero and Barbieri) >5 attacks in the last 2 years and ≥3 joints involved in different attacks; (Hannonen et al.) subsequent attacks in at least three different joints and for (Guerne and Weisman) six-month history of brief-sudden-onset and recurrent episodes of monoarthritis and ≥3 joints involved in different attacks. ConclusionsThe individual PR criteria showed different classification characteristics in ≤3 year and >3 year.

Temporomandibular Joint Ankylosis in A Child: Uncommon Etiology “Buckley’s Syndrome”

Temporomandibular joint (TMJ) ankylosis is characterized by bone and/or fibrous union between the condyle and the glenoid fossa, restricting joint movements. The etiology of ankylosis may be related to infectious processes. We present the case of the bilateral temporomandibular joint ankylosis in Buckley's syndrome. It is suggested to be ankylosis secondary to recurrent arthritis of the temporomandibular joint. The treatment was surgical include arthroplasty of the joint cavity, coronoidectomy and reconstruction with autogenous costochondral rib graft.

Comparison of the efficacy and safety of various anti-inflammatory drugs in urate-lowering therapy initiation in patients with gout (preliminary data)

Non-steroidal anti-inflammatory drugs (NSAIDs), colchicine and glucocorticoids (GC) are used to prevent arthritis attacks in gout, but data on their comparative effectiveness is lacking.Objective: to compare the efficacy and safety of various anti-inflammatory drugs at the start of treatment in patients with gout.Patients and methods. Of the 108 patients with gout, observed in the V.A. Nasonova Research Institute of Rheumatology, 97 (94%), mostly men, entered a single-center prospective study. The observation period was at least 24 weeks, patients received combined urate-lowering and prophylactic anti-inflammatory therapy unintermittingly. The control group included 12 patients in whom anti-inflammatory drugs were contraindicated. At the start of urate-lowering therapy, allopurinol 100 mg per day was prescribed, followed by dose titration until the target uric acid (UA) level of &lt; 360 μmol/L level was reached, in patients with severe tophus gout it was &lt; 300 μmol/L. The maximum dose of allopurinol was 900 mg per day; in patients with reduced glomerular filtration rate (&lt;60 ml/min/1.73m2 ) – 300 mg per day. For the prevention of arthritis attacks, the drug of choice was colchicine 0.5 mg per day, when contraindicated and/or poorly tolerated, NSAIDs were prescribed in the minimum effective dose. If there were any NSAIDs use restriction or intolerance – prednisone 7.5 mg per day was administered. The effectiveness of anti-inflammatory therapy (NSAIDs, colchicine or GC) was evaluated after 3 and 6 months which included analysis of the frequency of exacerbations and the duration of arthritis attacks, the intensity of pain according to the visual analogue scale (VAS) during a gout attack.Results and discussion. Of 97 patients with gout, 85 (88%) received anti-inflammatory therapy: NSAIDs – 16 (19%), colchicine – 60 (71%), GC – 9 (10%). In 65% of patients who were administered anti-inflammatory therapy, there were no recurrence of arthritis, whereas in 12 patients of the control group who did not receive this treatment, recurrence of arthritis was absent only in 25% of cases (p=0.008). After 3 and 6 months of follow-up, the duration of gout attacks and the intensity of joint pain according to the VAS for recurrent arthritis were significantly less (p&lt; 0.05 in both cases) in the treatment group (with any anti-inflammatory drug) than without treatment. Arthritis attacks were more likely to be absent when taking NSAIDs than when using colchicine and GC both after 3 (63%) and 6 (81%) months (p&lt; 0.05 in both cases). After 3 months of observation, the intensity of pain according to the VAS during the development of a gout attack was less for NSAIDs therapy than for colchicine and GC (p&lt; 0.05 in both cases), and after 6 months there was no difference between NSAIDs, colchicine and GC. Within first 3 months, there was no difference in the duration of arthritis attacks among anti-inflammatory drugs, whereas after 6 months duration of attacks was the smallest for NSAID therapy (p&lt; 0.05). Adverse reactions occurred only in NSAID and colchicine therapy in 2 and 5 patients, respectively. The patient's refusal to continue therapy without cause occurred in 13 (15%) cases, more often when taking colchicine.Conclusion. The proposed algorithm of anti-inflammatory drugs prescription for prevention of arthritis recurrence can reduce the risk of gout attacks development with good tolerance of the therapy. At the same time, better control of the frequency and duration of arthritis attacks, as well as the intensity of pain according to the VAS, is observed with the use of NSAIDs than with the use of colchicine and GC.

Presentation of a new mutation in FMF and evaluating the frequency of distribution of the MEFV gene mutation in our region with clinical findings

Familial Mediterranean Fever (FMF), which is an autosomal recessive disease characterized by recurrent self-limiting fever, peritonitis, pleuritis, arthritis and erysipelas-like erythemas, has been common among ethnic groups such as Turkish, Armenian, Arabic and Jewish. The clinical presentation is caused by mutations in the MEFV gene encoding the Pyrin protein. In this study, we aimed to present a new mutation that has not been previously defined from the mutations in the MEFV gene which is responsible for the genetic pathology of familial Mediterranean fever and to evaluate the frequency of distribution of the MEFV gene mutation among different ethnic groups living in our region. In present retrospective study, a total of 2639 clinically suspected FMF patients who were referred to Hatay Mustafa Kemal University Hospital between 2010 and 2017 were recorded. MEFV gene mutations were observed using DNA sequence analysis. MEFV mutations were found in 2079 of the 2639 patients (78.7%) Among these patients 184 (6.97%) were homozygous, while 1365 (51.72%) were heterozygous. The most frequently observed mutation was R202Q (1319, 19.55%) followed by E148Q (n = 476, 7.05%), M694V (n = 439, 6.51%), V726A (n = 146, 2.16%) and M680I (n = 135, 2%). In a case clinically diagnosed as FMF, a new mutation called S145G (p. Ser145Gly, c.433A > G) was identified in exon 2 of the MEFV gene. Besides, addition of a new pathogenic MEFV variant to the literature, the relationship between the FMF clinic and homozygous form of R202Q, which was previously considered as a polymorphism, was highlighted.

Joint Manifestations in Children with Familial Mediterranean Fever in Armenia: Clinical and Genetic Characteristics

Familial Mediterranean fever (FMF) is an autosomal-recessive autoinflammatory disease in the group of Hereditary periodic fever syndromes (HPFS), characterized by recurrent, self-limited attacks of fever and polyserositis, which last 2-4 days. It manifests mainly in childhood and often has early onset, resolve without sequels. Genetic testing of FMF is efficient and allows diagnosing of atypical cases. FMF is a significant health care problem in Armenia because of high frequency of carriers of MEFV mutations 1:3 - 4 (0.21) and marked FMF prevalence 54.7 per 10`000 of the total population. Articular symptoms are found in about 45% of cases. They usually manifest as acute recurrent arthritis (ARA) or arthralgia, but more rarely also chronic arthritis can be found. We aimed to investigate clinical and genetic characteristics of the joint manifestations in Armenian children with FMF. The charts of all 715 patients with proven diagnosis of FMF at the National Pediatric Center (NPC) for FMF were reviewed for joint manifestations. The diagnosis of FMF and disease severity were determined according to the Tel-Hashomer criteria and molecular-genetic detection of 12 MEFV mutations common for Armenians. The characteristics of joint manifestations and overall FMF disease were then compared to MEFV mutation analysis. There were 438 boys and 277 girls with an age at diagnosis between 3 months and 17 years (mean age: 8.64±0.17). Joint involvement was observed in 56.4% of all 715 cases. The manifestation was ARA in 30.5%, arthralgia in 21.2% and chronic arthritis (CA) in 4.7%, which would also qualify for a diagnosis of JIA. The frequency of ARA was associated with M694V mutation, mainly M694V homozygous and M694V heterozygous genotypes. The risk of CA depended on MEFV genotype of the FMF patients and was the highest in M694Vheterozygotes and M694Vhomozygotes. M694V heterozygous genotype was noticed significantly more frequently among FMF patients with spondyloarthritis in comparison to those without it. The probability of the development of CA among FMF patients without M694V mutation was significantly higher also in heterozygotes in comparison to compound-heterozygotes. We concluded, that the frequency of joint involvement among Armenian pediatric FMF patients was high - 56.4%, especially for CA. Chronic arthritis may be the first manifestation of FMF and occurred usually in patients with severe M694V mutations. Patients of Armenian origin with refractory arthritis should be asked for isolated febrile attacks, hemorrhagic vasculitis, episodes of pleuritis and family history to rule out FMF.

331. Septic arthritis: when is the joint clean enough?

BackgroundSeptic arthritis is an orthopedic emergency that requires debridement. Previous authors reported that patients with inflammatory arthropathy, diabetes, infection with S. aureus, involvement of a large joint, and synovial fluid WBC >85,000 are associated with >1 debridements. The purpose of this study was to determine factors associated with 1 vs >1 debridements.MethodsThis is a retrospective cohort of adult patients hospitalized at Denver Health Medical Center with large joint septic arthritis between 7/1/2012 and 4/13/20. Patients with implanted orthopedic material, osteomyelitis, and recurrent septic arthritis were excluded. Septic arthritis was defined as a patient presenting with acute arthritis and positive culture OR negative culture and no other etiology. Both electronic capture and manual chart review were performed. Descriptive statistics were used to characterize the population. Statistical analyses included bivariate and multivariate analyses.ResultsForty-four cases were included (26 knee [59.1%], 4 hip [9.1%], 6 elbow [13.6%], and 8 shoulder [18.2%]. The median age was 55.7 years (41.3–64.1], and 79.5% were male. The most common organisms were S. aureus (n=20, 45.5%) and beta-hemolytic Streptococcus (n=10, 22.7%). Three patients had no surgical debridement, 21 had 1 debridement, and 20 had >1 debridements.As compared to those who had 1 debridement, those with >1 debridements were more likely to be male (95% vs 61.9%, p=0.02) and to have a higher synovial fluid leukocyte count (102,761 vs 49,154, p=0.001), CRP at admission (162.5 vs 97.7, p=0.039), and WBC the day prior to debridement (13.4 vs 9.8, p=0.007). Intra-operative purulence trended to association with >1 debridements. Pre- to post-operative changes in opiate use, temperature, and ability to work with physical therapy were not associated with 1 vs >1 debridements. Both higher synovial fluid leukocyte counts and CRP value at admission were independently associated with >1 debridements (OR 2.31, p=0.015; OR 1.01, p=0.036 respectively).ConclusionPatients with higher synovial fluid leukocytes and CRP at admission were more likely to have >1 debridements. Additional studies with functional outcome scores are necessary to determine if >1 debridements are associated with better clinical outcomes.Disclosures All Authors: No reported disclosures

Клинический случай поздней диагностики анкилозирующего спондилита

Clinical observation of late diagnosis of ankylosing spondylitis, about the disease started with recurrent uveitis and peripheral arthritis, without clinical and instrumental signs of axial skeletal damage. “Classic” manifestations of the disease: inflammatory back pain and signs of sacroiliitis by MR appeared 5 years later from the first eye symptoms.

Omission of Hypertrophic Osteoarthropathy Delays the Early Diagnosis of a Lung Neoplasm

Abstract Introduction. Hypertrophic osteoarthropathy (HOA) is a syndrome characterized by periostitis of the long (tubular) bones, clubbing of the digits and arthritis. Less than 4,5% of the lung cancer patients developed hypertrophic osteoarthropathy as a paraneoplastic manifestation. Case presentation. We present a case of a 59 year old man, smoker presents for 18 months recurrent symmetric arthritis of knee. He lost 6 kilograms and he had no pulmonary symptoms. Physical examination revealed digital clubbing with watch-glass nails painless of all fingers, joint effusion of the knees. Laboratory results revealed only inflammatory syndrome and anaemia of chronic disease. The X-rays revealed periosteal appositions at the tibia, fibula and femur, mass in the left lower lobe. Computed tomography confirmed lung tumor and biopsy showed the malignant character of the tumor. Conclusion. The index of suspicion for OAH should be high in a patient with symmetrical arthritis who associates Hippocratic fingers.