Musculoskeletal Manifestations Research Articles

COVID-19 and musculoskeletal pain: an overview of the current knowledge.

The Coronavirus disease 2019 (COVID-19) caused by severe acute respiratory syndrome Coronavirus 2 (SARS-CoV-2) has provoked billions of infections worldwide. Several meta-analyses have observed that up to 50% of individuals who had survived to a SARS-CoV-2 acute infection suffer from post-COVID symptoms lasting for weeks or months and up to one year after infection. The prevalence of post-COVID pain ranges between 10% to 20% when assessed with other overall post-COVID symptoms and can reach up to 50% to 60% when investigated specifically. The most common musculoskeletal manifestations of post-COVID-19 condition include fatigue, myalgia, arthralgia or back pain. Despite pain of musculoskeletal origin is one of the most prevalent post-COVID pain symptoms, the exact pathophysiological mechanisms of musculoskeletal post-COVID pain are not completely understood. Studies have reported the complexity of post-COVID pain including immune, biological, and psychological factors, and more recently, they have suggested that genetic and epigenetic factors may also play a potential role, highlighting the need for further investigation into these mechanisms. Its management is still controversial, as no specific guideline for treating musculoskeletal post-COVID pain has been proposed with only general consideration about the relevance of multidisciplinary and multimodal treatment approaches. In this paper we will highlight the clinical features, the mechanism, and the management possibilities of musculoskeletal post-COVID pain.

Not all Erosions are Inflammatory: A Rare Case of Erosive Arthritis with Fibromatosis

Background:: Polyfibromatosis is a rare syndrome characterized by benign fibrous proliferation in different locations. It can be divided into two major categories: superficial and deep. Palmar fibromatosis (Dupuytren's contracture) in variable combinations with plantar fibromatosis (Ledderhose's disease), penile fibromatosis (Peyronie's disease), knuckle pads, and keloids are part of the spectrum seen in polyfibromatosis. There is a rare association of polyfibromatosis with keloids and erosive arthritis. Only four reported cases of polyfibromatosis with erosive arthritis and/or osteolysis have been described in the literature. In this report, we present one such case and review the existing literature on this rare syndrome. We also highlight the possibility of underlying genetic disorders in our patient, such as mucopolysaccharidosis with musculoskeletal manifestation, due to certain dysmorphic features and optic atrophy. Case Presentation:: The patient was a 23-year-old female with palmar and plantar fibromatosis accompanied by erosive arthritis. In addition, the patient had facial dysmorphic features, optic atrophy, and nystagmus. Extensive investigations were conducted, and no evidence of inflammatory rheumatic disease was found. The Patient exhibited features consistent with polyfibromatosis. Discussion:: A rare association between polyfibromatosis and erosive arthropathy with osteolysis has been demonstrated in literature through four case reports spanning from 1979 to 2018. The presence of dysmorphic facial features and ocular complications suggests a possible genetic or metabolic etiology, such as mucopolysaccharidosis. Conclusion:: This patient presents a diagnostic and therapeutic challenge. It is important to recognise the non-inflammatory etiology in order to avoid unnecessary treatment with anti-rheumatic drugs.

Occurrence and prediction of flare after tapering of TNF inhibitors in patients with axial spondyloarthritis.

Patients with axial spondyloarthritis (axSpA) in clinical remission tapered Tumor Necrosis Factor inhibitor (TNFi) therapy according to a clinical guideline. During 2-years' follow-up, we aimed to investigate flare frequency, dose at which flare occurred, type of flare and predictors thereof. Patients in clinical remission (Bath ankylosing spondylitis disease activity index (BASDAI)<40, physician global score<40 and without disease activity the previous year) tapered TNFi to 2/3 standard dose at baseline, 1/2 at week (w) 16, 1/3 at w32 and discontinuationed at w48. Flare was defined as: BASDAI flare (BASDAI≥40 and Δ≥20) and/or clinical flare (development of inflammatory back pain, musculoskeletal or extra-articular manifestations and/or ASDAS-CRP increase ≥0.9) and/or MRI flare (≥2 new/worsened inflammatory lesions). Of 108 patients, 106 (99%) flared before 2 years: 29 patients (27%) at 2/3 standard dose, 21 (20%) at 1/2 dose, 29 (27%) at 1/3 dose and 27 (25%) after discontinuation. One-hundred-and-five (99%) had clinical flare, 25 (24%) BASDAI flare and 23 (29% of patients with MRI) MRI flare. Forty-one patients (41%) fulfilled the ASAS-definition of clinically important worsening (≥0.9 increase since baseline). Higher baseline physician global score was an independent predictor of flare after tapering to 2/3 (Odds ratio=1.19 (95% Confidence Interval=1.05-1.41);p=0.011). Changes in clinical and/or imaging variables in the 16 weeks prior to tapering did not predict flare. Almost all (99%) axSpA patients in clinical remission flared during tapering to discontinuation, but above half not before receiving 1/3 dose or less. Higher physician global score was an independent predictor of flare.

THU460 A Case Series Of Hypophosphatasia: Presentation And Response To Asfotase Alfa

Abstract Disclosure: F. Alsarraf: None. D.S. Ali: None. K. Almonaei: None. H. Al-Alwani: None. M. Brandi: Consulting Fee; Self; Aboca, Alexion Pharmaceuticals, Inc., Amolyt, Bruno Farmaceutici, Calcilytix, Kyowa Kirin, UCB. Grant Recipient; Self; Abiogen, Alexion Pharmaceuticals, Inc., Amgen Inc, Bruno Farmaceutici, Echolight, Eli Lilly &amp; Company, Kyowa Kirin, SPA, Theramex, UCB. Speaker; Self; Abiogen, Alexion Pharmaceuticals, Inc., Amgen Inc, Bruno Farmaceutici, Calcilytix, Echolight, Eli Lilly &amp; Company, Kyowa Kirin, SPA, Theramex, UCB. A.A. Khan: Advisory Board Member; Self; Amgen Inc, Amolyt, Takeda. Consulting Fee; Self; Amgen Inc. Grant Recipient; Self; Alexion Pharmaceuticals, Inc., Amgen Inc, Ascendis, Chugai, Radius Health, Inc, Takeda, Ultragenyx. Research Investigator; Self; Amgen Inc, Radius Health, Inc. Speaker; Self; Alexion Pharmaceuticals, Inc., Amgen Inc. Introduction: Hypophosphatasia (HPP) is a rare inherited metabolic bone disorder characterized by a deficiency in the tissue non-specific alkaline phosphatase (TNSALP) due to loss of function mutation in the ALPL gene. HPP is associated with impaired skeletal mineralization due to elevations in inorganic pyrophosphate and altered phosphate: pyrophosphate ratio. Asfotase alfa (AA) was approved for treatment of HPP in 2015. We present a case series of 7 patients with HPP, 5 with pediatric-onset and 2 with adult-onset, who have been treated with AA and describe the efficacy and safety in these patients. Case presentation: 7 patients (4 females, 3 males) aged 19-68 years with HPP were included in this case series. Diagnosis of HPP was confirmed by DNA analysis. AA was administered in doses of 6mg/kg/week with a mean follow-up of 6 months (SD= 5). Management outcome: Improvement in muscle strength, muscle pain, walking ability and walking distance with a reduction in the use of gait aids was seen. Muscle strength and pain improved by up to 70% from baseline as quantified subjectively by patients. Walking distance improved by up to 100%. Patients also reported improved cognition, mood and energy levels, with up to 90% improvement in mood and 75% improvement in energy levels. 4 out of 6 patients first noted clinical signs of improvement after 3 months of being on therapy. 1 out of the 7 patients sustained a toe fracture 10 months from being on AA. AA was well-tolerated with injection site reactions being the most commonly reported adverse effect. Discussion: HPP treatment with AA in individuals with both pediatric and adult-onset forms resulted in significant improvement in musculoskeletal and cognitive manifestations in addition to patients’ quality of life. It was well tolerated in 6 patients. 1 patient discontinued therapy because of minor adverse effects with myalgias. Presentation: Thursday, June 15, 2023

The Effect of an Interdisciplinary Dermatological-Rheumatological Consultation on the Outcome of Patients with Psoriasis with Musculoskeletal Pain: A Prospective, Single-Center Cohort Study.

Psoriatic arthritis (PsA), a disease with complex inflammatory musculoskeletal manifestations, complicates psoriasis in up to 30% of patients. In this study, we aimed to determine the effect of an interdisciplinary dermatological-rheumatological consultation (IDRC) for patients with psoriasis with musculoskeletal symptoms. This prospective study enrolled 202 patients with psoriasis. Patients with musculoskeletal pain (MSP) (n = 115) participated in an IDRC 12weeks after enrollment. The outcome was evaluated after 24weeks. In 12/79 (15.2%) patients seen in the IDRC, the prior diagnosis was changed: eight with a first diagnosis of PsA, four with a diagnosis of PsA rescinded. Treatment was modified in 28% of patients. Significant improvements in Psoriasis Area and Severity Index (PASI) (from 5.3 to 2.0; p < 0.001) and Dermatology Life Quality Index (DLQI) (from 6.7 to 4.5; p = 0.009) were observed. By comparing changes in PASI and DLQI over the study period, an improvement in PASI of 0.7 ± 1.4 points (p = 0.64) and in DLQI of 2.9 ± 1.5 points (p = 0.051) could be attributed to participation in the IDRC. An IDRC of patients with psoriasis with MSP leads to a valid diagnosis of PsA and improvement in quality of life. Based on these results, an IDRC is a valuable and time efficient way for psoriasis patient with MSP to receive optimal care.

P25 TB or not TB? An unusual case of inflammatory arthritis in an anti-CCP positive patient

P25 TB or not TB? An unusual case of inflammatory arthritis in an anti-CCP positive patient

The International X-Linked Hypophosphatemia (XLH) Registry: first interim analysis of baseline demographic, genetic and clinical data

BackgroundX-linked hypophosphatemia (XLH) is a rare, hereditary, progressive, renal phosphate-wasting disorder characterized by a pathological increase in FGF23 concentration and activity. Due to its rarity, diagnosis may be delayed, which can adversely affect outcomes. As a chronic disease resulting in progressive accumulation of musculoskeletal manifestations, it is important to understand the natural history of XLH over the patient’s lifetime and the impact of drug treatments and other interventions. This multicentre, international patient registry (International XLH Registry) was established to address the paucity of these data. Here we present the findings of the first interim analysis of the registry.ResultsThe International XLH Registry was initiated in August 2017 and includes participants of all ages diagnosed with XLH, regardless of their treatment and management. At the database lock for this first interim analysis (29 March 2021), 579 participants had entered the registry before 30 November 2020 and are included in the analysis (360 children [62.2%], 217 adults [37.5%] and 2 whose ages were not recorded [0.3%]; 64.2% were female). Family history data were available for 319/345 (92.5%) children and 145/187 (77.5%) adults; 62.1% had biological parents affected by XLH. Genetic testing data were available for 341 (94.7%) children and 203 (93.5%) adults; 370/546 (67.8%) had genetic test results; 331/370 (89.5%) had a confirmed PHEX mutation. A notably longer time to diagnosis was observed in adults ≥ 50 years of age (mean [median] duration 9.4 [2.0] years) versus all adults (3.7 [0.1] years) and children (1.0 [0.2] years). Participants presented with normal weight, shorter length or height and elevated body mass index (approximately − 2 and + 2 Z-scores, respectively) versus the general population. Clinical histories were collected for 349 participants (239 children and 110 adults). General data trends for prevalence of bone, dental, renal and joint conditions in all participants were aligned with expectations for a typical population of people with XLH.ConclusionThe data collected within the International XLH Registry, the largest XLH registry to date, provide substantial information to address the paucity of natural history data, starting with demographic, family history, genetic testing, diagnosis, auxology and baseline data on clinical presentation.

Diabetes Mellitus Is a Possible Risk Factor for the Development of Trochanteric Bursitis-A Large-Scale Population-Based Study.

Trochanteric Bursitis (TB) is a common reason to seek primary care, previously shown to be associated with female gender and obesity. Diabetes mellitus (DM) has several musculoskeletal manifestations, but was never found to be associated with TB. To explore the association between DM and TB, based on a large database. The secondary aim was to explore the influence of gender and insulin usage on the occurrence of TB. cross-sectional study. A population-based cohort consisting of 60,610 patients (55,428 without DM and 5182 with DM), of whom 5418 were diagnosed with TB. A logistic regression model was applied to estimate propensity scores. The odds of individuals with DM being diagnosed with TB were 55.8% higher compared to the odds of patients without DM (OR: 1.558, 95% CI: [1.429, 1.70], p < 0.0001). We found that insulin users had a lower risk of TB than patients not using insulin (log-rank p < 0.0001). Females are 3.3 times more likely to have TB than males (RR: 3.337, 95% CI: [3.115, 3.584], p < 0.0001). DM is a risk factor for developing TB. Insulin had a protective effect against TB, suggesting that better glycemic control might prevent this painful infliction.

Musculoskeletal manifestations associated with transthyretin-mediated (ATTR) amyloidosis: a systematic review

BackgroundHereditary and wild-type transthyretin-mediated (ATTRv and ATTRwt) amyloidoses result from the misfolding of transthyretin and aggregation of amyloid plaques in multiple organ systems. Diagnosis of ATTR amyloidosis is often delayed due to its heterogenous and non-specific presentation. This review investigates the association of musculoskeletal (MSK) manifestations with ATTR amyloidosis and the delay from the onset of these manifestations to the diagnosis of ATTR amyloidosis.MethodsThis systematic review utilized Medline and EMBASE databases. Search criteria were outlined using a pre-specified patient, intervention, comparator, outcome, time, study (PICOTS) criteria and included: amyloidosis, ATTR, and MSK manifestations. Publication quality was assessed utilizing Joanna Briggs Institute (JBI) critical appraisal checklists.The search initially identified 7,139 publications, 164 of which were included. PICOTS criteria led to the inclusion of epidemiology, clinical burden and practice, pathophysiology, and temporality of MSK manifestations associated with ATTR amyloidosis. 163 publications reported on ATTR amyloidosis and MSK manifestations, and 13 publications reported on the delay in ATTR amyloidosis diagnosis following the onset of MSK manifestations.ResultsThe MSK manifestation most frequently associated with ATTR amyloidosis was carpal tunnel syndrome (CTS); spinal stenosis (SS) and osteoarthritis (OA), among others, were also identified. The exact prevalence of different MSK manifestations in patients with ATTR amyloidosis remains unclear, as a broad range of prevalence estimates were reported. Moreover, the reported prevalence of MSK manifestations showed no clear trend or distinction in association between ATTRv and ATTRwt amyloidosis.MSK manifestations precede the diagnosis of ATTR amyloidosis by years, and there was substantial variation in the reported delay to ATTR amyloidosis diagnosis. Reports do suggest a longer diagnostic delay in patients with ATTRv amyloidosis, with 2 to 12 years delay in ATTRv versus 1.3 to 1.9 years delay in ATTRwt amyloidosis.ConclusionThese findings suggest that orthopedic surgeons may play a role in the early diagnosis of and treatment referrals for ATTR amyloidosis. Detection of MSK manifestations may enable earlier diagnosis and administration of effective treatments before disease progression occurs.

The Challenging Differentiation of Psoriatic Arthritis from Other Arthropathies and Nonspecific Arthralgias in Patients with Psoriasis: Results of a Cross-Sectional Rheumatologic Assessment of a Large Dermatologic Cohort.

Aiming to identify the potential challenges in the classification of musculoskeletal manifestations in patients with psoriasis (PsO), this study analyzed the outcomes of a cross-sectional rheumatologic assessment of 1057 PsO patients. In total, 209 had a previous diagnosis of psoriatic arthritis (PsA). Out of the remaining 848 subjects, 293 (35%) were classified as suspected PsA cases according to the rheumatologist's judgment and/or Early PsA Screening Questionnaire score (EARP) ≥ 3. However, only 14% received a PsA diagnosis, 49% had a PsA-alternative diagnosis, and the remaining 37% had nonspecific arthralgias. Most of the newly diagnosed PsA patients had a symptoms duration ≥1 year (72%) and moderate disease activity (55%) with active oligoarthritis (85%), dactylitis, or enthesitis (35%) as the most frequent clinical pattern. The most frequent PsA-alternative diagnoses were osteoarthritis and fibromyalgia (44% and 41%). The only factors with significant (p < 0.05) utility in discriminating PsA from other diseases and nonspecific arthralgias were young age and EARP score with a history of morning stiffness, swollen joints, or dactylitis. These results demonstrated a high prevalence of suspected musculoskeletal symptoms in PsO patients, with, however, only a small proportion due to PsA. Close collaboration between the dermatologist and rheumatologist plays a crucial role in the differential diagnosis of PsA, as well as in monitoring nonspecific arthralgias for the potential transition to overt PsA.

Clinical improvement in early onset interstitial lung disease using rituximab in children with anti-MDA5 positive juvenile dermatomyositis.

Children with juvenile dermatomyositis (JDM) and antibodies to melanoma differentiation associated gene 5 (anti-MDA5) are at increased risk of severe disease complications, including interstitial lung disease (ILD). Data regarding treatment of disease complications in this patient population is limited. In this study, we examined the disease course of children with JDM and anti-MDA5 antibodies before and after treatment with rituximab (RTX). Patients (age 2-21 years) seen at the Children's Hospital at Montefiore between July 2012 and August 2021, with a diagnosis of JDM, positive anti-MDA5 positive antibodies, evidence of ILD, and who were treated with rituximab were eligible for inclusion. Retrospective clinical and laboratory data were reviewed. Five out of 8 patients with positive anti-MDA5 antibodies had evidence of ILD (62.5%). Four patients had data available for review. All patients received at least 5 courses of RTX infusions, with discontinuation of steroids by an average of 12 months after starting RTX and a decrease to fewer than two concurrent medications by fifth course of RTX. Indicators of ILD on high-resolution computed tomography (HRCT) and pulmonary function tests (PFTs) either improved or fully resolved over the course of RTX treatment for all patients. Patients also demonstrated resolution of active cutaneous manifestations and musculoskeletal disease activity. This is the first study to examine the use of RTX in children with JDM and anti-MDA5 antibodies, with notable improvements ILD, cutaneous and musculoskeletal manifestations. Further studies are needed to better understand the efficacy of RTX on JDM disease related complications.

Surgical Challenges in Infective Endocarditis: State of the Art.

Infective endocarditis (IE) is still a life-threatening disease with frequent lethal outcomes despite the profound changes in its clinical, microbiological, imaging, and therapeutic profiles. Nowadays, the scenario for IE has changed since rheumatic fever has declined, but on the other hand, multiple aspects, such as elderly populations, cardiovascular device implantation procedures, and better use of multiple imaging modalities and multidisciplinary care, have increased, leading to escalations in diagnosis. Since the ESC and AHA Guidelines have been released, specific aspects of diagnostic and therapeutic management have been clarified to provide better and faster diagnosis and prognosis. Surgical treatment is required in approximately half of patients with IE in order to avoid progressive heart failure, irreversible structural damage in the case of uncontrolled infection, and the prevention of embolism. The timing of surgery has been one of the main aspects discussed, identifying cases in which surgery needs to be performed on an emergency (within 24 h) or urgent (within 7 days) basis, irrespective of the duration of antibiotic treatment, or cases where surgery can be postponed to allow a brief period of antibiotic treatment under careful clinical and echocardiographic observation. Mainly, guidelines put emphasis on the importance of an endocarditis team in the handling of systemic complications and how they affect the timing of surgery and perioperative management. Neurological complications, acute renal failure, splenic or musculoskeletal manifestations, or infections determined by multiresistant microorganisms or fungi can affect long-term prognosis and survival. Not to be outdone, anatomical and surgical factors, such as the presence of native or prosthetic valve endocarditis, a repair strategy when feasible, anatomical extension and disruption in the case of an annular abscess (mitral valve annulus, aortic mitral curtain, aortic root, and annulus), and the choice of prosthesis and conduits, can be equally crucial. It can be hard for surgeons to maneuver between correct pre-operative planning and facing unexpected obstacles during intraoperative management. The aim of this review is to provide an overview and analysis of a broad spectrum of specific surgical scenarios and how their challenging management can be essential to ensure better outcomes and prognoses.

Musculoskeletal pain and quality of life in patients undergoing hemodialysis: a single-center study

Objectives: In this study, we aimed to research the frequency of musculoskeletal manifestations of patients receiving hemodialysis treatment, and evaluate the relationship between quality of life and musculoskeletal manifestations of these patients. Methods: Patients undergoing hemodialysis for at least 6 months were included in our study. Patients were asked to complete a questionnaire that formed by the investigators including demographic, clinical features, and musculoskeletal symptoms that frequently seen in patients who receive hemodialysis. Kidney Disease Quality of Life Short Form Version 1.3 (KDQOL-SF 1.3) and Short Form Health Survey (SF 36) questionnaires were completed by the patients and the control group. Blood urea nitrogen, serum creatinine, serum albumin, C-reactive protein (CRP), parathormone (PTH), ferritin, calcium, phosphate, hemoglobin, and Kt/V were measured. Also, the patients were divided into two subgroups according to musculoskeletal symptoms and these subgroups were compared in terms of dialysis adequacy, quality of life scoring and laboratory findings. Results: Seventy-four patients (42 males, 32 females) were enrolled in our study. The mean age of the patients was 60.85 ± 12.29 years. Six-five (87.83%) patients had musculoskeletal symptoms. There was statistically major difference in terms of smoking between subgroups (p = 0.046). We did not detect any correlation between two groups in terms of Kt/V (p = 0.411). Pain in shoulder/neck (41.9%), pain in limbs (58.1%) and pain in back (56.8%) were the most detected musculoskeletal symptoms of the patients. There was statistically significant difference between genders in terms of joint swelling, muscle cramps, pain in limbs, back and neck /shoulder (p = 0.015, p = 0.001, p = 0.008, p = 0.001, and p = 0.004, respectively). We detected that all subunits of KDQOL scores were higher in control group than patients who were included in our study. There was statistically significant relation between emotional role and energy subunits of KDQOL scores and CRP (p = 0.031 and p = 0.025, respectively). Conclusion: The results of our study were not as significant as the results of similar studies, however they are valuable because they show demographic, clinical characteristics, and quality of life of patients receiving hemodialysis in our region.

Identification of the first signs or symptoms in different spondyloarthritis subtypes and their association with HLA-B27: data from REGISPONSER and RESPONDIA registries

ObjectiveTo describe and analyse the initial symptoms attributable to patients with spondyloarthritis (SpA) and their association with HLA-B27 status.MethodsThis was an observational, cross-sectional and multicentre study with patients who fulfilled...

Extra-musculoskeletal manifestations driving the therapeutic decision-making in patients with Spondyloarthritis: a 12-month follow-up prospective cohort study

BackgroundThe extra-musculoskeletal manifestations (EMMs) such as recurrent acute anterior uveitis (rAAU), psoriasis (Ps), and inflammatory bowel disease (IBD), are related to the Spondyloarthritis (SpA), as well as they are associated with disease activity and poor prognosis. However, there are no data addressing its relevance regarding therapeutic decision-making in clinical practice.ObjectiveTo evaluate the impact of EMMs to drive the treatment decision-making in patients with SpA in a 12-month follow-up.Patients and methodsSpA patients, according to the axial and peripheral ASAS classification criteria, as well as CASPAR criteria, with any active EMM, defined as main entry criteria, were included in this longitudinal cohort study. Individuals with a history of any disease or condition that could be associated with some of the studied endpoints, including neoplasms and infectious diseases, were excluded. Specific tools related to each EMM, including Psoriasis Area Severity Index (PASI), ophthalmologic evaluation, according to the Standardization of Uveitis Nomenclature (SUN) criteria, and gut complaints were used at baseline and during the 3-, 6- and 12-month of follow-up as outcomes measures over time. Descriptive and inferential analyses were used appropriately, including Pearson’s correlation test, chi-squared test, and ANOVA. P value less than 0.05 was considered as significant.ResultsA total of 560 patients were enrolled, of whom 472 meet the eligibility criteria. The majority (N = 274; 59.6%) had one or more EMM related to SpA umbrella concept. Among the EMM, the one that most influenced therapeutic decision-making was psoriasis (28.5%), followed by uveitis (17.5%) and IBD (5.5%), regardless of musculoskeletal manifestations. Clinical improvement of EMMs outcomes was observed in most patients over 12-month follow-up, especially in those with rAAU and IBD (P < 0.001).ConclusionOur results showed that EMMs guided the therapeutic decision-making in half of SpA patients, regardless of musculoskeletal condition, suggesting the inter-disciplinarity among the rheumatologist, ophthalmologist, dermatologist, and gastroenterologist plays a crucial role to manage them.

Musculoskeletal symptoms in juvenile onset systemic lupus erythematosus

In juvenile onset systemic lupus erythematosus (SLE), musculoskeletal involvement is one of the most common manifestations. Musculoskeletal symptoms may be due to disease activity itself, organ damage due to SLE activity, drug exposure, or comorbidities. Although they are not life-threatening, they still have a significant impact on disability severity and social exclusion. This article discusses the clinical features of various causes of musculoskeletal manifestations in SLE with onset in childhood and adolescence, the adequate interpretation of which is important for diagnosing and evaluating SLE activity and for correcting the treatment of the disease, thereby improving the long-term prognosis and quality of life of patients.

Socioeconomic disparities in musculoskeletal oncology.

Musculoskeletal oncology is a clinical specialty dealing with a diverse population of patients with metastatic bone disease, hematological malignancies with musculoskeletal manifestations, primary bone malignancies and soft tissue sarcomas. There are wide-spread disparities including socioeconomic (SES) and insurance-related disparities reported in the literature. In this review, we'll summarize the disparities surrounding the musculoskeletal oncology.

Musculoskeletal Manifestations of Transthyretin Cardiac Amyloidosis

Transthyretin Cardiac Amyloidosis (ATTR-CA) is caused by the deposition of misfolded transthyretin fibrils, leading to restrictive cardiomyopathy and eventually death if untreated. ATTR-CA is associated with certain musculoskeletal conditions, notably carpal tunnel syndrome, lumbar spinal stenosis, nontraumatic biceps tendon rupture, trigger finger and hip, knee and shoulder arthropathies. These musculoskeletal manifestations could provide a clue and potentially help in the timely diagnosis of ATTR-CA.

Clinical features and long-term outcomes of patients with systemic polyarteritis nodosa diagnosed since 2005: Data from 196 patients

BackgroundThe landscape of polyarteritis nodosa (PAN) has substantially changed during the last decades. Recent data regarding causes, characteristics, and prognosis of systemic PAN in the modern era are lacking. MethodsThis retrospective study included patients with systemic PAN referred to the French Vasculitis Study Group between 2005 and 2019. Characteristics, associated conditions and outcomes were collected, and predictors of relapse and death were analyzed. Results196 patients were included. Main clinical symptoms were constitutional (84%), neurological (59%), skin (58%) and musculoskeletal (58%) manifestations. Secondary PAN accounted for 55 (28%) patients, including myelodysplastic syndrome (9%), solid cancer (7%), lymphoma (4%) and autoinflammatory diseases (4%). No patient had active HBV infection. All treated patients (98.5%) received glucocorticoids (GCs), alone (41%) or in combination with immunosuppressants (59%), with remission achieved in 90%. Relapses were independently associated with age >65 years (HR 1.85; 95% CI1.12–3.08), gastrointestinal involvement (1.95; 95% CI1.09–3.52) and skin necrotic lesions (HR 1.95; 95%CI 1.24–3.05). One-, 5- and 10-year overall survival rates were 93%, 87% and 81%, respectively. In multivariate analyses, age >65 years (HR 2.80; 95%CI 1.23–6.37), necrotic purpura (HR 4.16; 95%CI 1.62–10.70), acute kidney injury (HR 4.89; 95% 1.71–13.99) and secondary PAN (HR 2.98; 95%CI 1.29–6.85) were independently associated with mortality. ConclusionLandscape of PAN has changed during the last decades, with the disappearance of HBV-PAN and the emergence of secondary PAN. Relapse rate remains high, especially in aged patients with gastrointestinal and cutaneous necrosis, as well as mortality.

Multicenter study of the natural history and therapeutic responses of patients with chikungunya, focusing on acute and chronic musculoskeletal manifestations – a study protocol from the clinical and applied research in Chikungunya (REPLICK network)

BackgroundChikungunya is associated with high morbidity and the natural history of symptomatic infection has been divided into three phases (acute, post-acute, and chronic) according to the duration of musculoskeletal symptoms. Although this classification has been designed to help guide therapeutic decisions, it does not encompass the complexity of the clinical expression of the disease and does not assist in the evaluation of the prognosis of severity nor chronic disease. Thus, the current challenge is to identify and diagnose musculoskeletal disorders and to provide the optimal treatment in order to prevent perpetuation or progression to a potentially destructive disease course.MethodsThe study is the first product of the Clinical and Applied Research Network in Chikungunya (REPLICK). This is a prospective, outpatient department-based, multicenter cohort study in Brazil. Four work packages were defined: i. Clinical research; ii) Translational Science – comprising immunology and virology streams; iii) Epidemiology and Economics; iv) Therapeutic Response and clinical trials design. Scheduled appointments on days 21 (D21) ± 7 after enrollment, D90 ± 15, D120 ± 30, D180 ± 30; D360 ± 30; D720 ± 60, and D1080 ± 60 days. On these visits a panel of blood tests are collected in addition to the clinical report forms to obtain data on socio-demographic, medical history, physical examination and questionnaires devoted to the evaluation of musculoskeletal manifestations and overall health are performed. Participants are asked to consent for their specimens to be maintained in a biobank. Aliquots of blood, serum, saliva, PAXgene, and when clinically indicated to be examined, synovial fluid, are stored at -80° C. The study protocol was submitted and approved to the National IRB and local IRB at each study site.DiscussionStandardized and harmonized patient cohorts are needed to provide better estimates of chronic arthralgia development, the clinical spectra of acute and chronic disease and investigation of associated risk factors. This study is the largest evaluation of the long-term sequelae of individuals infected with CHIKV in the Brazilian population focusing on musculoskeletal manifestations, mental health, quality of life, and chronic pain. This information will both define disease burden and costs associated with CHIKV infection, and better inform therapeutic guidelines.