Current Rheumatology Reviews - Volume 18, Issue 4, 2022

Previous research has shown conflicting reports about the effect of systemic sclerosis (SSc) on bone metabolism, especially considering bone mineral density (BMD), bone microarchitecture, and risk of fracture. The objective of this review is to analyze data from previous articles to investigate the differences in BMD and fracture risk between SSc and non-SSc populations and to discuss potential underlying mechanisms. The main factors investigated have been BMD (mean and standard deviation), t-scores and z-scores at the lumbar spine, femoral neck, and total hip measured by dual-energy X-ray absorptiometry (DEXA), bone remodeling markers, fracture prevalence, and incidence, trabecular bone score (TBS), musculoskeletal involvement with particular correlation to SSc skin subtype and extent, disease duration, serological pattern, and vitamin D levels. Since microvascular alterations evaluated through nailfold videocapillaroscopy (NVC) of SSc patients have recently been correlated with decreased BMD and bone microarchitecture, the vascular impairment in SSc has been proposed as a remarkable contributing element in bone remodeling, and the role of hypoxia has been investigated.

Introduction: Fibrolipoma of the median nerve is a rare benign lesion responsible for carpal tunnel syndrome. Fibrolipoma is often misdiagnosed. This article aimed to review and summarize current knowledge regarding fibrolipoma of the median nerve. We emphasize the clinical and imaging features of this disease. Methods: To examine the characteristics of fibrolipoma of the median nerve, we performed a litera-ture review using MEDLINE. The search included only English studies published from database in-ception to June 2021. Results: Forty-six cases of fibrolipoma of the median nerve were included. Fibolipoma is characterized by diffuse infiltration of peripheral nerves by normal-appearing fibrous and adipose tissues. The fibrolipoma of the median nerve can be responsible for macrodactyly, numbness, paresthesia, and weakness within the median nerve distribution. Ultrasonography shows a fusiform hyperechoic mass along the nerve containing hypoechoic bands corresponding to nerve fascicles. Magnetic resonance imaging is the gold standard for the diagnosis of fibrolipoma. It typically shows a contrast between the low signal nerve fibers and the high signal fatty tissues, revealing a characteristic « cable-like » appearance on axial sections and a «spaghetti-like» appearance on coronal sections. Conclusion: Fibrolipoma should be considered in young patients with carpal tunnel syndrome. This review emphasizes the clinical and radiological features of fibrolipoma. We highlight the images of ultrasonography in the diagnosis of rare structural causes of carpal tunnel syndrome.

Background: It is often unclear whether systematic reviews and primary studies are de-signed to elucidate the efficacy or effectiveness of interventions. This may compromise the use of the information in clinical or policy decisions. Objective: This overview aimed to evaluate the methodological profiles of studies on fibromyalgia pharmacotherapy in terms of the quality and nature of the interventions (efficacy versus effective-ness). Methods: The protocol was registered in the International Prospective Register of Systematic Re-views database. Seven databases were searched for relevant publications. Systematic reviews inves-tigating the effectiveness or efficacy of fibromyalgia pharmacotherapy were included. Methodolog-ical quality was investigated using A MeaSurement Tool to Assess Systematic Reviews (AM-STAR), and efficacy andeffectiveness were evaluated using Rating of Included Trials on the Effica-cy-effectiveness Spectrum (RITES). Results: In this overview, 4,107 studies were initially identified. 8 systematic reviews and 34 prima-ry studies remained after overlaps were removed. Of the eight systematic reviews, 4.76% (n=3) and 7.93% (n=5) were of moderate and high quality, respectively. An analysis of systematic reviews clearly showed the criteria “participants characteristics” and “trial setting" with the most frequent answers as scales 1 and 2 (strong emphasis on efficacy or rather strong emphasis on efficacy), re-spectively. RITES analysis revealed that the most frequent response was “strong emphasis on effi-cacy” in 68% (92/136) of primary studies. Conclusion: This analysis showed, in both systematic reviews and primary studies, a predominantly strong emphasis on efficacy, suggesting the need for methodological quality improvement in future studies, especially those designed to provide evidence related to effectiveness. The protocol for this overview has been registered in the International Prospective Register of Sys-tematic Reviews (PROSPERO; CRD42018095943).

Background: Fibromyalgia syndrome is characterised by extensive muscular pain and chronic fatigue. Among the pharmacologic and other nutrient supplements that have been studied, Vitamin D has garnered attention owing to the critical role it plays in inflammatory and pain pathways. We conducted a systematic literature review to examine the efficacy of vitamin D supplementation in improving the clinical status of the patients and alleviating the symptoms of fibromyalgia. Methods: We searched Cochrane CENTRAL, PubMed, Science Direct, Scopus, grey literature (medrXiv and biorXiv) for observational studies, randomized controlled trials, case-control studies, and case reports published in English from January 2011 to May 2021, using the terms vitamin D and fibromyalgia or FMS. References were reviewed manually and articles were only included if they were specific about the diagnosis of fibromyalgia. Results: 2651 studies were retrieved, with 12 studies fulfilling the inclusion criteria. 11 out of these 12 studies were of high quality and showed low risk of bias. 11 of these also demonstrated definitive improvement in clinical status and various outcome measures following supplementation with Vitamin D. Conclusion: Our study emphasises an association between supplementation of vitamin D and improvement of the clinical condition of fibromyalgia through a systematic review of high-quality studies. The study also identified areas for future scope for research needed for standardising the detection and treatment of this chronic condition through cost-effective supplements such as Vitamin D.

Background: Differentiating between seronegative rheumatoid arthritis (RA) and psoriatic arthritis (PsA) presenting only with the involvement of the small joints of the hands can be challenging. Implementing musculoskeletal ultrasound (US) to reveal specific patterns of joint and tendon involvement may have an added value in the management of early arthritis. Objective: The aim was to investigate whether a seven-joint US score was able to distinguish between patients with RA and PsA. Materials and Methods: One hundred and forty-one patients with RA, 65 patients with PsA, and 45 healthy controls (HC) were included in the current study. US assessment of the wrist, second and third metacarpophalangeal, second and third proximal interphalangeal joint, second and fifth metatarsophalangeal joint was performed, and the following scores were calculated: synovitis and tenosynovitis/ paratenonitis scores on grayscale ultrasound (GSUS) and on power Doppler (PD) US, erosion score, US7 score. Results: RA patients had significantly higher median scores of GS synovitis, PD synovitis, erosions, and US7 than PsA patients (p < 0.001). PsA patients had significantly higher median scores of GS tenosynovitis/paratenonitis and PD tenosynovitis/paratenonitis (p < 0.001). All US scores were significantly higher for both patient groups as compared to the HC group (p < 0.001). Conclusion: Sonographic evaluation by a seven-joint score can be helpful in the differentiation between rheumatoid and psoriatic arthritis.

Background: Gout is one of the most common inflammatory arthritis, where identification of MSU crystals in synovial fluid is a widely used diagnostic measure. Ultrasonography has a great sensitivity in detecting signs of MSU deposits, such as tophi and double contour (DC), as mentioned in the latest gout criteria, allowing early clinical diagnosis and therapy. Objective: The objective of this study was to evaluate the changes in ultrasound of gout patients’ knee and 1st metatarsophalangeal joint (MTP1) after initiation of urate-lowering therapy (ULT) drugs in the six-month period. Methods: Forty-three patients, fulfilling the ACR/EULAR 2015 criteria of gout with a score of >8, were enrolled; they were in between attacks and not on ULT for the last 6 months, or SUA concentration (SUA) of >6.0 mg/dL. Full examination, evaluation of joints pain by visual analog scale (VAS), ultrasonography (US) for tophus and DC at the knee, and MTP1 were performed at baseline and at 3 and 6 months (M3, M6) after starting ULT. Result: After 6 months of treatment, patients reached the target SUA level showed higher disappearance of DC sign (p<0.05) and a decrease in tophus size (p<0.05). The percentage of tophus size at 6th month was 26.4% and 3% for DC sign disappearance, which was more at MTP1. Conclusion: Ultrasound examination in screening for gout tophi or DC sign before starting ULT and during follow-up is important and complements clinical examination.

Background: It has been over a year since the first documented case of the COVID-19 virus was recorded. Since then, our understanding of this virus has continually evolved, however, its wide-ranging effects are still unfolding. Similar to previously studied viral infections, severe acute respiratory syndrome-associated coronavirus-2 (SARS-CoV-2) has been shown to lead to a degree of autoimmunity in patients who are recovering from its effects. Due to its effects on the innate immune system, such as the toll-like receptors and complement system, a varying degree of proinflammatory markers can become widespread in those who continue to recover from the virus. This case series offers a unique perspective on how COVID-19 has had dramatic effects on those already suffering from inflammatory rheumatic conditions, such as rheumatoid arthritis, systemic lupus erythematosus, or fibromyalgia. As the ever-lasting effects of COVID-19 are still unfolding, this case series is one of few to discuss the development and changes of patients with rheumatic conditions. This study hopes to encourage larger studies to be conducted on the effects of COVID- 19 on autoimmune conditions. Case Presentation: Seven patients were identified with new manifestations of rheumatic conditions, which included 3 cases of rheumatoid arthritis, 2 cases of polymyalgia rheumatica, 1 case of reactive arthritis, and 1 case of cutaneous lupus. Post-COVID syndrome was also diagnosed in 7 other patients. Patients with rheumatoid arthritis presented with symptoms 4-5 weeks after being diagnosed with COVID-19. Symptoms of polyarticular joint pain, swelling, and morning stiffness were reported in this group. These patients were treated with disease-modifying anti-rheumatic drugs and experienced an improvement in symptoms on follow-up. Two cases of polymyalgia rheumatica were identified in patients that were previously diagnosed with COVID-19 six weeks prior. One patient had no significant past medical history and the other patient had a history of rheumatoid arthritis, which was well controlled. These patients experienced weakness and tenderness in the proximal joints with elevated levels of ESR and CRP. They were treated with prednisone and showed improvement. Reactive arthritis was diagnosed in 1 patient who presented with swelling in both hands and wrists 2 days after being diagnosed with COVID-19. This patient began to experience symptoms of reactive arthritis 2 days after resolution of initial COVID-19 symptoms and this persisted for 3 months. The patient was managed with methylprednisolone injections and NSAIDs, which improved her symptoms. Post-COVID syndrome was identified in 7 patients. All patients were female and had a history of well-controlled fibromyalgia. Patients generally experienced fatigue, headaches, and memory fog, which had variable onset from a few days and up to 4 weeks after being diagnosed with COVID-19. One patient had a complete recovery of her symptoms at follow-up 3 months after the initial presentation. The other 6 patients continued to report symptoms of post-COVID syndrome at follow-up. Patients were managed with lifestyle modifications and their previous fibromyalgia treatment. Conclusion: While cases of COVID-19 continue to rise, complications of this disease are still being discovered. Those who initially recover from COVID-19 may experience new-onset rheumatic conditions, worsening of previously diagnosed rheumatic conditions, or post-COVID syndrome. As we continue to learn more about the effects of COVID-19, the awareness of these manifestations will play a key role in the appropriate management of these patients.

Aims: To study the clinical and laboratory findings between patients with primary Antiphospholipid Syndrome (pAPS) with and without LR. Background: Livedo Reticularis (LR) is a common manifestation of Antiphospholipid Syndrome (APS). Although no previous study evaluated patients with and without LR. Methods: A transversal study including 66 pAPS patients was performed. Demographical, anthropometric, medication use, antiphospholipid antibodies profile data were evaluated, and LR's clinical and laboratory features. Patients were subdivided into one of two groups: pAPS with LR and pAPS without LR. Results: Both groups were alike concerning demographics and anthropometrics. Interestingly, the frequency of stroke (28.5 vs. 7.5%, p=0.04), as well as of Sneddon’s syndrome (100 vs. 30.0%, p<0.0001), were higher in pAPS with LR than the other group. Conversely, patients in the pAPS without LR group had more thyroidopathy than those in the pAPS with LR group (80% vs. 50% %, p=0.03). Conclusion: Patients with pAPS and LR have more stroke and seem to be protected from thyroidopathy. Careful follow-up of these patients is therefore advised.

Background: Necrotizing fasciitis (NF) is a life-threatening disease. Arthroplasty patients with underlying or preexisting conditions and chronic medication use are prone to infection. The current report aimed to present a 53 years old female patient who developed necrotizing fasciitis one month after total hip arthroplasty. Case Presentation: On the postoperative 15th day, she had wound discharge. She was given oral anti- biotherapy. At postoperative 1 month, her complaints increased. At emergency service, she experienced extreme pain in her lower extremity with pseudoparalysis, and a hip X-ray revealed gas images. She used prednisolone for 10 years and insulin for 15 years because of pemphigus vulgaris and diabetes mellitus. Clinical and laboratory findings led us to the diagnosis of necrotizing fasciitis. Despite aggressive treatment, including debridement, prosthesis removal, and antibiotherapy, unfortunately, the patient died 13 days after admission. Conclusion: Patients with an immune suppressive condition, long-term corticosteroid use, or multiple underlying diseases should be closely monitored after total hip arthroplasty surgery to avoid such a devastating complication.

Introduction: Retroperitoneal fibrosis (RPF) is a rare disease characterized by fibroinflammatory tissue proliferation in the retroperitoneum. It results in a chronic inflammatory and fibrosis condition, possibly leading to compression of the retroperitoneal structures, especially to encasement of the ureters and the inferior vena cava. It may have an idiopathic or a secondary origin. Spondyloarthritis (SpA) is one of the rare conditions described among the secondary forms. Case Presentation: Herein, we report a new case of RPF in a patient with AS presented with acute abdominal pain radiating to the lumbar region and the left testicle. On clinical examination, we found a mild stiffness of the lumbar spine and a decrease in chest expansion. Sacroiliac joint pain was also found. The rest of the physical examination was normal. Laboratory tests showed inflammation with increased C-reactive protein (130 mg/l) and creatinine (112 micromol/l) levels. The computed tomography scan revealed a soft tissue density mass located around the sub-renal aorta. Diagnosis of idiopathic RPF associated with AS was retained. The patient was treated with a daily dosage of 1 mg/kg of oral glucocorticoid with a good outcome. Conclusion: RPF is a rare condition that can be either idiopathic or secondary. Its association with spondyloarthritis, mainly in its ankylosing spondylitis form, seems to be more than anectodal. Treatment may involve medical therapy and/or surgical management. Key Message: In the presence of back pain, fatigue, weight loss, and low grade fever in spondyloarthritis patients, physicians should screen for retroperitoneal fibrosis as it could be a possible cause.

Background: Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg Strauss syndrome, is an uncommon vasculitis associated with antineutrophil cytoplasmic antibody (ANCA). The hallmarks of the disease are asthma, eosinophilia, and systemic vasculitis with varying degrees of neurological, cutaneous, cardiac, gastrointestinal, and renal involvement. Diagnosis is often difficult since the symptoms are diverse, and a number of differentials need to be excluded. Case Presentation: In this report, we describe a 60-year-old patient who presented with mononeuritis multiplex and a painful skin rash. A history of late-onset asthma, which was poorly controlled, led us to suspect EGPA. Laboratory data showed leukocytosis, eosinophilia (>10%), elevated ESR, CRP, and IgE, normal chest Xray, positive rheumatoid factor (RA), perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA), and evidence of extravascular eosinophils in histopathology report of skin biopsy. She was treated with methylprednisolone and cyclophosphamide pulse therapy with a satisfactory response. Conclusion: Diagnosis of EGPA requires a combination of clinical and histopathological findings to meet the diagnostic criteria. A history of poorly controlled or late-onset asthma may guide us to the diagnosis that is frequently overlooked. Due to the wide heterogeneity of EGPA patients' phenotypes, sharp, professional judgment is needed for early disease detection and treatment in order to avoid irreversible changes and poor outcomes.

Background: Cryopyrin-Associated Periodic Syndrome (CAPS) is a variety of clinical variants of autoinflammatory diseases. The pathology is based on a mutation in the NLRP3 gene encoding the cryopyrin protein, which leads to the uncontrolled production of interleukin-1β. Particular attention should be paid to the rarity of this disease and the lack of clinical knowledge about it in therapeutic and rheumatological practice, which leads to an erroneous diagnosis and the appointment of ineffective treatment for a long time, leading to the progression of the disease and disability of the patient. Case Presentation: This article describes a clinical case of this disease. The first manifestations of the disease in a woman appeared from the age of 2 years, in the form of a rash and fever. Since school age, there have been signs of arthritis. By the age of 24, sensorineural hearing loss and pain in the spine were evident. The disease occurred under the clinical manifestations of spondyloarthritis. Its treatment with anti-inflammatory therapy did not give a stable result. Conclusion: From the analysis, we can conclude that patient M. from early childhood suffers from a severe Neonatal-onset Multisystem Inflammatory Disease of a genetic nature. For a long time, the patient was diagnosed with ankylosing spondylitis, and appropriate treatment was carried out without significant success. The correct diagnosis of CAPS was made only in 2018. This patient has conditions of both CAPS and AS together, which is a very rare association in rheumatological practice. The only treatment method that could stop the manifestations of the disease and prevent life-threatening kidney damage (amyloidosis) is the use of genetically engineered biological drugs, i.e., IL-1β inhibitors. The only drug of this group registered in Russia is canakinumab (Ilaris®). From the moment of diagnosis to the present day, the patient is treated with the genetically engineered drug canakinumab (Ilaris®) at a dose of 150 mg once every 8 weeks. 6 months after taking the drug, the patient went into complete clinical and laboratory remission.