Scurvy Masquerading as Juvenile Idiopathic Arthritis or Vasculitis with Elevated Inflammatory Markers: A Case Series

2020 ◽  
Vol 218 ◽  
pp. 234-237.e2 ◽  
Author(s):  
Alexandra Perkins ◽  
Clay Sontheimer ◽  
Jeffrey P. Otjen ◽  
Susan Shenoi
2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1357.1-1357
Author(s):  
S. M. Lao ◽  
J. Patel

Background:Reactive arthritis is a form of spondyloarthritis with aseptic joint involvement occurring after a gastrointestinal or urogenital infection. Most commonly associated with Chlamydia trachomatis, Salmonella, Shigella, Campylobacter, and Yersinia. Syphilis is an infection caused by the spirochete Treponema pallidum and is not usually associated with reactive arthritis. Syphilis is a great imitator of other diseases due to its broad presentation including painless chancre, constitutional symptoms, adenopathy, rash, synovitis, neurological and ocular findings.Objectives:To discuss a patient who presented with symptoms of rheumatoid arthritis (RA) but was later diagnosed with syphilis.Methods:31 year old male, former tobacco smoker, referred to Rheumatology for sudden onset joint pains, elevated anti-cyclic citrullinated peptide (anti-CCP), and elevated inflammatory markers. He reported pain in bilateral wrists, fingers, and right elbow for 6 weeks. Associated with 45 minutes of morning stiffness and new onset lower back pain without stiffness. He denied trauma, fever, chills, skin rash, dysuria, or diarrhea. Initiated trial naproxen 500mg twice a day only to have minimal relief. Patient is sexually active with men and was recently diagnosed with oropharyngeal gonorrhea treated with azithromycin 4 months prior. All other STI screening including syphilis, gonorrhea, HIV were negative at that time. Patient is on emtricitabine and tenofovir disoproxil fumarate for HIV pre-exposure prophylaxis. He denied family history of immune mediated conditions. Exam was significant for mild synovitis of both wrists and bilateral 2nd metacarpophalangeal joints. Initial labs revealed weakly positive anti-CCP 21 (normal <20), sedimentation rate 64 (normal ESR 0-15 mm/hr), C-reactive protein 24 (normal CRP 0-10 mg/L), and negative RF, ANA, HLA B27. During a short trial of prednisone taper, there was temporary improvement in symptoms, however synovitis recurred upon completion. Hydroxychloroquine (HCQ) 200mg twice a day was started for possible RA and he was referred to Ophthalmology for baseline retinopathy screening. Incidentally, he developed right sided blurry vision 2 weeks after initiation of HCQ. He was diagnosed with panuveitis of the right eye with inflammation of the optic nerve head and prednisone 40mg daily was initiated for presumed ocular manifestation of RA. However, further workup of panuveitis revealed reactive Treponema pallidum antibody and RPR quantity 1:32. Prednisone was immediately discontinued and he was referred to the emergency department for possible neurosyphilis.Results:Lumbar puncture showed cerebral spinal fluid with 260 red blood cells, 1 white blood cell, 27mg/dL protein, 60mg/dL glucose, non reactive VDRL, reactive pallidum IgG antibody, and negative cultures. Meningitis and encephalitis panels were negative. Patient completed a 14 day course of IV penicillin G with complete remission of joint pain, visual symptoms, and normalization of anti-CCP, ESR, and CRP.Conclusion:This case highlights how syphilis may mimic signs and symptoms of RA including symmetrical small joint pain, morning stiffness, elevated inflammatory markers, and positive anti-CCP. Anti-CCP is >96% specific for RA but was a false positive in this patient. There have only been few reported cases noting positive anti-CCP with reactive arthritis. This is a rare case of reactive arthritis secondary to syphilis with resolution of symptoms upon treating the syphilis.References:[1]Carter JD. Treating reactive arthritis: insights for the clinician. Ther Adv Musculoskelet Dis. 2010 Feb;2(1):45-54.[2]Cohen SE, Klausner JD, Engelman J, Philip S. Syphilis in the modern era: an update for physicians. Infect Dis Clin North Am. 2013 Dec;27(4):705-22.[3]Singh Sangha M, Wright ML, Ciurtin C. Strongly positive anti-CCP antibodies in patients with sacroiliitis or reactive arthritis post-E. coli infection: A mini case-series based review. Int J Rheum Dis. 2018 Jan;21(1):315-321.Disclosure of Interests:None declared.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1784.1-1784
Author(s):  
R. Dos Santos Sobrín ◽  
M. Martí Masanet ◽  
B. Lopez-Montesinos ◽  
L. Lacruz Pérez ◽  
I. Calvo

Background:Familial Mediterranean Fever (FMF) is a genetic autoinflammatory disorder caused mostly by mutations in MEFV gene. Its inheritance is autosomal recessive and is the most frequent periodic fever syndrome. First-line treatment is based in colchicine use, so biologics (anti-IL-1) are reserved for refractory cases1, 2.Objectives:To account for clinic and treatment features of patients with FMF in a specialized center as opposed to non-referent centers.Methods:This study was developed in the Pediatric Rheumatology Service in Hospital Universitario y Politécnico La Fe de Valencia. Demographic, clinic and treatment data were collected from patients diagnosed of FMF since January 2004 to September 2019.Results:106 patients met last FMF criteria3. 55% had a pathogenic mutation in genetic analysis. 52% were female. Before 10 years old, 71% of patients had the diagnosis (51% before 4 years old). Arthralgia/myalgia (73%), periodic fever (62%) and abdominal pain (54%) were the most common symptoms. Systemic Juvenile Idiopathic Arthritis (JIA, 6), other forms of JIA (9) and vasculitis (10) were the most prevalent comorbidities. When talking about treatment, 76,4% received Colchicine (60,5% with good response), 22,6% needed a classical disease modifying antirheumatic drug (mostly Methotrexate) and 22 patients got biologic treatment (73% anti-IL-1).Conclusion:When analyzing this case-review, JIA has a strong association with our patients, so it could explain severe disease activity and more articular involvement. This could be an illustration to the higher use of Methotrexate. Also, the most relevant symptom was arthralgia while fever is the most frequent in literature. Likewise, age of diagnosis has been earlier than other case-series (this would be more frequent in other autoinflammatory syndromes, as literature relates)1, 2, 4.References:[1]Ozdogan H, Ugurlu S. Familial Mediterranean Fever. Presse Med. (2019).[2]Ozen S, Demirkaya E, Erer B, et al. EULAR recommendations for the management of familial Mediterranean fever. Ann Rheum Dis 2016;75:644-651.[3]Sag E, Demirel D, Demir S, et al. Performance of the new “Eurofever/PRINTO classification criteria” in FMF patients. Semin Arthritis Rheum. 2019;19:30369-5.[4]Rozenbaum M, Rosner I. Severe outcome of juvenile idiopathic arthritis (JIA) associated with familial Mediterranean fever (FMF). Clin Exp Rheumatol. 2004;22:S75-8.Disclosure of Interests:Raquel Dos Santos Sobrín: None declared, Miguel Martí Masanet: None declared, B Lopez-Montesinos: None declared, Lucía Lacruz Pérez: None declared, Inmaculada Calvo Grant/research support from: Bristol-Myers Squibb, Clementia, GlaxoSmithKline, Hoffman-La Roche, Merck Sharpe & Dohme, Novartis, Pfizer, Sanofi, Speakers bureau: AbbVie, GlaxoSmithKline, Hoffman-La Roche, Novartis


2015 ◽  
Vol 42 (7) ◽  
pp. 1252-1258 ◽  
Author(s):  
Christine Petri ◽  
Steffen Thiel ◽  
Jens Christian Jensenius ◽  
Troels Herlin

Objective.The complement system plays a crucial role in the pathogenesis of inflammatory processes. The lectin pathway of the complement system is activated through the recognition of pathogens by soluble pattern recognition molecules (PRM), i.e., mannan-binding lectin (MBL), collectin-LK, and the ficolins. PRM are reportedly correlated to disease activity in rheumatoid arthritis (RA). The aim was to evaluate the pathogenic role of PRM in juvenile idiopathic arthritis (JIA).Methods.We measured MBL, M-ficolin, H-ficolin, MBL-associated serine proteases (MASP) 1, MASP-2, MASP-3, and 2 alternative splice products, MBL-associated protein (MAp) 44 and MAp19, in plasma and synovial fluid (SF) of children with persistent oligoarticular (n = 109 in plasma, n = 38 in SF) and systemic JIA (n = 19 in plasma, n = 11 in SF). The concentrations of the proteins were measured by in-house time-resolved immunofluorometric assays.Results.We observed significantly higher levels of M-ficolin, MASP-1, MASP-2, and MASP-3 in plasma and SF from patients with systemic JIA compared with persistent oligoarticular JIA (p < 0.001). In paired samples of plasma and SF from 47 patients with oligoarticular and systemic JIA, we observed higher concentrations in plasma for both subtypes for 7 of the measured proteins while the reverse relationship was observed for MASP-3. M-ficolin and MASP-2 correlated to erythrocyte sedimentation rate, C-reactive protein, white blood cell count, and platelet count (p < 0.001). M-ficolin was in addition related to the number of active joints and inversely related to hemoglobin levels.Conclusion.Our results point to plasma M-ficolin and MASP-2 as inflammatory markers in JIA. The levels of all proteins are higher in plasma than in SF, except for MASP-3, indicating that MASP-3 may be produced locally in joints.


2019 ◽  
Vol 37 (15_suppl) ◽  
pp. e17548-e17548
Author(s):  
Vittoria G. Espeli ◽  
Francesco Martucci ◽  
Antonella Richetti ◽  
Alessia Pastore

e17548 Background: the Neutrophil-to-Lymphocytes ratio (NLR) and the Platelet-to-Lymphocytes ratio (PLR) represent potential prognostic markers in different tumor types. The purpose of our study was to investigate the prognostic role of the pretreatment inflammatory markers NLR and PLR in patients with locally advanced squamous cell oropharyngeal cancer (OPSCC) treated with curative concomitant chemoradiotherapy (CRT). Methods: between 2004 and 2016, all patients with OPSCC diagnosed at the University Hospital of Varese and the Oncology Institute of Southern Switzerland were reviewed retrospectively. CRT consisted of Intensity-Modulated Radiation Therapy (IMRT) to a dose of 66-70 Gy concomitant to 3 doses of 3-weekly cisplatin. Pre-treatment NLR and PLR were registered from blood samples obtained within 14 days before treatment. 3-year disease free survival (DFS) was analyzed. Results: ninety-two patients were identified. Median follow-up was 39 months. Median NLR was 3 and median PLR was 153. The 3 year DFS was 82.9%. Univariate analysis showed that HPV status and PLR were statistically significant factors in determining 3-year DFS (p 0.01 and 0.04 respectively). Multivariate analysis showed that only HPV status was a statistically significant factor for 3-year DFS (p 0.009). High NLR and high PLR were correlated with lower DFS: patients with NLR > 4 had a 3-years DFS of 50% versus 75%in the group with NLR ≤ 4(p = 0.04) and patients with PLR > 150 had a 3-years DFS of 65% versus 82% in the group with PLR ≤ 150 (p = 0.04). Conclusions: in our case series, NLR and PLR were significantly associated with clinical outcome. Additional data are necessary to investigate the prognostic value of inflammatory markers and to identify the ideal cut-off of both markers.


2013 ◽  
Vol 2013 ◽  
pp. 1-11 ◽  
Author(s):  
Barbara Głowińska-Olszewska ◽  
Artur Bossowski ◽  
Elżbieta Dobreńko ◽  
Andrzej Hryniewicz ◽  
Jerzy Konstantynowicz ◽  
...  

Objective. We aimed to determine the prevalence of excess body mass in juvenile idiopathic arthritis (JIA) children and to investigate the influence of obesity into the early, subclinical changes in cardiovascular system in these patients.Methods. Fifty-eight JIA patients, aged median 13 years, were compared to 36 healthy controls. Traditional cardiovascular risk factors and inflammatory markers (hsCRP, IL-6, TNFα, adiponectin) were studied together with IMT (intima-media thickness), FMD (flow mediated dilation), and LVMi (left ventricle mass index) as surrogate markers of subclinical atherosclerosis.Results. Thirteen JIA children (22%) were obese and had increased systolic blood pressure, cholesterol, triglycerides, insulin, HOMA, hsCRP, and IL-6 compared to nonobese JIA and controls. FMD was decreased compared to nonobese JIA and controls, whereas IMT and LVMi were increased. In multivariate regression analysis, TNFα, SDS-BMI, and systolic blood pressure were independent predictors of early CV changes in JIA.Conclusions. Coincident obesity is common in JIA children and is associated with insulin resistance, dyslipidemia, and increased levels of inflammatory markers leading to early changes in cardiovascular system. Thus, medical care of children with JIA should include strategies preventing cardiovascular disease by maintenance of adequate body weight.


Healthcare ◽  
2021 ◽  
Vol 9 (9) ◽  
pp. 1144
Author(s):  
Ioana Adriana Muntean ◽  
Irena Pintea ◽  
Ioana Corina Bocsan ◽  
Carmen Teodora Dobrican ◽  
Diana Deleanu

(1) Background: The COVID-19 pandemic has resulted in the exacerbation of various chronic diseases. Due to the potential impact of SARS-CoV-2 on mast cells, we aimed to analyze the relevance of COVID-19 disease on chronic spontaneous urticaria (CSU) clinical presentation and biological profile. (2) Methods: This study is a retrospective case series of patients with CSU diagnosed and treated in the Allergy Department of the Professor Doctor Octavian Fodor RIGH, (Cluj-Napoca, Romania). Patients were assessed for disease activity and level of control with the weekly urticaria activity score and the visual analogue scale. Results were correlated with COVID-19 severity and with nonspecific markers of inflammation during and after the SARS-CoV-2 infection. (3) Results: SARS-CoV-2 impacted a significant proportion (33%) of the CSU patients, of which 71% developed a moderate-severe form of COVID-19. Most of the patients (68%) had moderate-severe forms of CSU and 65% took AH1 treatment (one dose, two-fold dose or four-fold dose). The rest of them (35%) received the second-line treatment (40.3% Omalizumab, 53% Prednisolone and 4.8% Cyclosporine). In Omalizumab treated group of UCS patients we observed that COVID-19 disease was not severe. We established a positive correlation between the severity of the infection and that of the CSU clinical presentation, with most bothersome symptoms of urticaria being experienced by moderate to severe COVID-19 CSU patients (47%). Inflammatory markers were positively correlated (p = 0.01) with a more severe clinical profile of CSU, in accordance with our hypothesis that the level of inflammation triggered by COVID-19 disease has a role in CSU exacerbation. The non-specific inflammatory markers, such as CRP, were positively associated with the UAS7 score (R2 = 0.363; p = 0.001). An increased rate of exacerbation of CSU was observed in moderate-severe COVID-19 infection. 4) Conclusions: COVID-19 disease can result in the exacerbation of chronic spontaneous urticaria, more likely in moderate to severe forms of infection.


2019 ◽  
Vol 104 (7) ◽  
pp. e2.26-e2
Author(s):  
Octavio Aragon Cuevas

BackgroundNon JIA related HLH is a life-threatening complication that is increasingly recognised in paediatric patients, particularly in those who are unwell in the paediatric intensive care unit (PICU). Untreated or insufficiently treated HLH has a significant mortality rate (up to 53%).1AimTo review the evidence base for the use of anakinra in paediatric patients with non-JIA HLH refractory to systemic corticosteroids in patients who are not fit for treatment as per HLH 2004 protocol.MethodsA PubMed search with words ‘anakinra’ and ‘hemophagocytic lymphohistiocytosis’ was carried out on July 2018 to find out the evidence base with regards to the use of anakinra in non-JIA related HLH. Any published peer reviewed clinical studies or trials (including but not limited to retrospective or prospective controlled trials, comparative studies and observational/cohort studies) were considered. Case reports and series were considered if better evidence studies were not available. A recent case study from a tertiary paediatric centre will be used to illustrate the pathway followed to diagnose non-JIA related HLH and funding options.ResultsAlthough a protocol exists for primary HLH treatment (HLH 2004), including chemotherapy and stem cell transplantation,2 there is no consensus on how to treat secondary HLH. The literature mainly showed case reports and small case series,3 describing the use of anakinra collectively for 35 patients (median age 14 to 48 years) who met the HLH 2004 diagnostic criteria with an overall survival rate of up to 88% at time of discharge from the PICU3. Anakinra was used at standard doses always in combination with corticosteroids. Some patients also received intravenous immunoglobulin (IVIG) and ciclosporin at the discretion of the medical teams.ConclusionThe evidence for use of anakinra in non JIA secondary HLH is limited to retrospective observational studies and mostly restricted to adult populations. Despite this caveat, these studies have demonstrated that anakinra therapy alongside other non-etoposide immunomodulatory therapies is associated with an improvement in short term survival. In patients with multi-organ dysfunction, who are too unstable to receive the existing etoposide based HLH-2004 treatment regimen due to concerns regarding significant treatment toxicity, personalised non-etoposide therapies including dexamethasone, IVIG, ciclosporin and anakinra may be better tolerated and provide a bridge to future more standardised treatment. Evidence to date shows that relapse of secondary HLH is possible with ciclosporin therapy. In JIA related HLH, anakinra was considered better than ciclosporin at inducing remission and having a lower incidence of adverse effects,4 and NHS England granted funding for the treatment based on these findings. The available evidence did not show any serious adverse events related to anakinra.RecommendationsThis tertiary centre approved the use of anakinra for this patient and future patients with this indication despite lack of reimbursement from NHS England for the drug. An urgent interim policy review will be put together by a team of the British Society of Paediatric and Adolescent Rheumatology (BSPAR) and presented to the NHS England commissioners to seek funding for anakinra for paediatric patients with this indicationReferencesMiettunen, et al. Successful treatment of severe paediatric rheumatic disease-associated macrophage activation syndrome with interleukin-1 inhibition following conventional immunosuppressive therapy: case series with 12 patients. Rheumatology (Oxford) 2011;50:417–9Henter JI, Horne A, Arico M, Egeler RM, Filipovich AH, Imashuku S, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2007;48:124–31.Kumar B, Aleem S, Saleh H, Petts J, Ballas ZK. A Personalized Diagnostic and Treatment Approach for Macrophage Activation Syndrome and Secondary Hemophagocytic Lymphohistiocytosis in Adults. J Clin Immunol 2017;37:638–643Boom V, et al. Evidence-based diagnosis and treatment of macrophage activation syndrome in systemic juvenile idiopathic arthritis. Pediatr Rheumatol Online J 2015;13:55.


2020 ◽  
Vol 18 (1) ◽  
Author(s):  
Bassam Atallah ◽  
Wasim El Nekidy ◽  
Saad I. Mallah ◽  
Antoine Cherfan ◽  
Laila AbdelWareth ◽  
...  

Abstract Background Hospitals in the Middle East Gulf region have experienced an influx of COVID-19 patients to their medical wards and intensive care units. The hypercoagulability of these patients has been widely reported on a global scale. However, many of the experimental treatments used to manage the various complications of COVID-19 have not been widely studied in this context. The effect of the current treatment protocols on patients’ diagnostic and prognostic biomarkers may thus impact the validity of the algorithms adopted. Case presentation In this case series, we report four cases of venous thromboembolism and 1 case of arterial thrombotic event, in patients treated with standard or intensified prophylactic doses of unfractionated heparin or low molecular weight heparin at our institution. Tocilizumab has been utilized as an add-on therapy to the standard of care to treat patients with SARS-CoV-2 associated acute respiratory distress syndrome, in order to dampen the hyperinflammatory response. It is imperative to be aware that this drug may be masking the inflammatory markers (e.g. IL6, CRP, fibrinogen, and ferritin), without reducing the risk of thrombotic events in this population, creating instead a façade of an improved prognostic outcome. However, the D-dimer levels remained prognostically reliable in these cases, as they were not affected by the drug and continued to be at the highest level until event occurrence. Conclusions In the setting of tocilizumab therapy, traditional prognostic markers of worsening infection and inflammation, and thus potential risk of acute thrombosis, should be weighed carefully as they may not be reliable for prognosis and may create a façade of an improved prognostic outcome instead. Additionally, the fact that thrombotic events continued to be observed despite decrease in inflammatory markers and the proactive anticoagulative approach adopted, raises more questions about the coagulative mechanisms at play in COVID-19, and the appropriate management strategy.


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