Search

Background/Objectives: Idiopathic inflammatory myopathies (IIMs) are rare autoimmune disorders characterized by progressive proximal muscle weakness and varying extra-muscular manifestations. The latest 2017 EULAR/ACR criteria classify them into subgroups. This study aims to evaluate the role of nailfold capillaroscopy (NFC) as a diagnostic and prognostic tool in IIMs by comparing capillaroscopic patterns across different IIM subtypes. Methods: We conducted an observational, cross-sectional study at the Institute of Rheumatology in Belgrade, analyzing 90 patients diagnosed with IIMs per the 2017 EULAR/ACR criteria. Patients were categorized into dermatomyositis (DM) (n = 37), polymyositis (PM) (n = 35), amyopathic dermatomyositis (ADM) (n = 13), and juvenile dermatomyositis (JDM) (n = 5). A control group of 35 patients with primary Raynaud's phenomenon was also included. NFC findings, clinical manifestations, and laboratory data were compared across the groups. Results: In DM, 81.9% exhibited a scleroderma capillaroscopic pattern, which was also present in 76.9% of ADM patients. In PM, the most common pattern was nonspecific changes (48.6%). JDM patients showed a high prevalence of scleroderma changes (n = 4 (80%)). Scleroderma patterns correlated with Gottron's papules, heliotrope rash, periungual erythema, Raynaud's phenomenon, and interstitial lung disease (ILD). No significant differences were found in laboratory parameters across capillaroscopic groups, except for a higher prevalence of anti-Jo1 antibodies in patients with nonspecific capillaroscopic changes. Conclusions: NFC is a valuable tool for differentiating IIM subtypes and correlating clinical manifestations with specific capillaroscopic patterns. The high prevalence of scleroderma changes in DM and ADM suggests their potential as a diagnostic and prognostic marker in IIMs. Further research with larger cohorts is warranted to validate these findings.

Background: Diabetic neuropathy is one of the most common complications of diabetes mellitus. The aim of this study is to evaluate the Moveo device, a novel device that uses a machine learning (ML) algorithm to detect and track diabetic neuropathy. The Moveo device comprises 4 sensors positioned on the back of the hands and feet accompanied by a mobile application that gathers data and ML algorithms that are hosted on a cloud platform. The sensors measure movement signals, which are then transferred to the cloud through the mobile application. The cloud triggers a pipeline for feature extraction and subsequently feeds the ML model with these extracted features., Methods: The pilot study included 23 participants. Eleven patients with diabetes and suspected diabetic neuropathy were included in the experimental group. In the control group, 8 patients had suspected radiculopathy, and 4 participants were healthy. All participants underwent an electrodiagnostic examination (EDx) and a Moveo examination, which consists of sensors placed on the feet and back of the participant's hands and use of the mobile application. The participant performs six tests that are part of a standard neurological examination, and a ML algorithm calculates the probability of diabetic neuropathy. A user experience questionnaire was used to compare participant experiences with regard to both methods., Results: The total accuracy of the algorithm is 82.1%, with 78% sensitivity and 87% specificity. A high linear correlation up to 0.722 was observed between Moveo and EDx features, which underpins the model's adequacy. The user experience questionnaire revealed that the majority of patients preferred the less painful method., Conclusions: Moveo represents an accurate, easy-to-use device suitable for home environments, showing promising results and potential for future usage.

Objective: The present systematic review and meta-analysis aimed to evaluate the periodontal health of systemic sclerosis patients compared with non-systemic sclerosis controls., Materials and Methods: Online databases were searched for eligible studies on February 24, 2023. The primary outcomes of interest in systemic sclerosis patients and controls included the clinical attachment level, periodontal probing depth, recession depth, plaque index, bleeding on probing score, gingival index, number of teeth with periodontitis, prevalence of periodontitis and gingivitis, and extent and severity of periodontitis., Results: Fourteen studies met inclusion criteria and were incorporated in the qualitative and quantitative analysis. In comparison with the controls, systemic sclerosis patients had a higher prevalence of periodontitis (OR = 7.63 (1.74-33.50), p = 0.04, I2 = 69%), including more severe forms of periodontitis (OR = 6.68 (3.39-13.15), p = 0.85, I2 = 0%), as well as higher periodontal probing depth ((0.88 (0.45-1.31), p = 0.02, I2 = 99%)), clinical attachment level (1.22 (0.8-1.64), p = 0.003, I2 = 98%), and plaque presence (0.83 (0.13-1.53), p = 0.03, I2 = 96%). There was no statistically significant difference in gingival inflammation (1.14 (0.07-2.21), p = 0.04, I2 = 98%)., Conclusions: The systematic review and the meta-analysis showed that systemic sclerosis patients suffer from worse periodontal health than non-systemic sclerosis individuals., Competing Interests: The authors have declared that no com peting interests exist., (Copyright: © 2024 Sredojevic et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.)

Elderly-onset rheumatoid arthritis (EORA) is a distinct clinical entity defined as the onset of rheumatoid arthritis (RA) in individuals aged over 60 years. EORA presents unique clinical features, including a more equitable distribution of sexes, a potential predilection for male involvement, a higher incidence of acute onset characterized by constitutional symptoms, a propensity for systemic manifestations, elevated sedimentation rates at disease onset, a reduced occurrence of rheumatoid factor positivity, increased titers of anti-citrullinated protein antibodies, a preference for involvement of large joints, elevated disease activity, the presence of bone erosions, and heightened patient disability. RA is recognized to consist of three partially overlapping subsets. One subset mirrors the classical RA clinical presentation, while the remaining subsets exhibit either a polymyalgia rheumatica-like phenotype or present with remitting seronegative symmetrical synovitis accompanied by pitting edema syndrome. In the initial stages of EORA management, non-steroidal anti-inflammatory drugs (NSAIDs) are not typically the first-line treatment choice, because seniors are much more prone to develop side effects due to NSAIDs, and the use of NSAIDs is in reality contraindicated to the majority of seniors due to comorbidities. Disease-modifying antirheumatic drugs (DMARDs), frequently methotrexate, are introduced immediately after the diagnosis is made. In cases where elderly patients demonstrate resistance to conventional DMARD therapy, the introduction of biological or targeted synthetic DMARDs becomes a viable treatment option. EORA presents a unique clinical profile, necessitating tailored treatment strategies. Our study emphasizes the challenges of NSAID use in seniors, highlighting the imperative shift toward DMARDs such as methotrexate. Future research should explore personalized DMARD approaches based on disease activity, comorbidities, and safety considerations, aiming to optimize treatment outcomes and minimize glucocorticoid reliance, thereby enhancing the quality of care for EORA patients.

Objectives: This study aims to assess the possible relationship between 99m Tc-pertechnetate hand perfusion scintigraphy (HPS) and nailfold capillaroscopy (NC) in systemic sclerosis (SSc) patients., Patients and Methods: The study group consisted of 25 SSc patients (6 males; 19 females; mean age 54.2±9.7 years; range, 32 to 67 years), 18 female patients with primary Raynaud's phenomenon (PRP) (mean age 47.1±9.5 years; range, 34 to 65 years) and 10 healthy individuals (3 males, 7 females; mean age 52.7±12.6 years; range, 37 to 73 years). NC and 99m Tc-pertechnetate HPS were performed in all examinees. The capillaroscopic findings were classified as normal or scleroderma pattern ("early", "active", or "late"). The fingers-to-palm ratios were calculated for both blood flow (BF) and blood pool (BP) phases of the 99m Tc-pertechnetate HPS., Results: Systemic sclerosis patients showed a significantly lower BP ratio than PRP patients and healthy subjects (p=0.004). No statistically significant difference was observed between the SSc and PRP patients in respect to BF ratio. A gradual decrease of BF and BP with the severity of NC microangiopathy pattern ("early", "active" or "late") was found in SSc patients, while the differences were not statistically significant. Patients with diffuse SSc showed lower BF and higher BP than those with limited SSc, while these differences were without statistical significance. There was no significant correlation between BF or BP values and type of SSc (limited or diffuse) (p=0.77 versus p=0.54, respectively) as well as three microangiopathy patterns (p=0.22 versus p=0.54, respectively)., Conclusion: 99m Tc-pertechnetate HPS improves the evaluation of vascular damage in SSc patients. There is no direct relationship between NC and 99m Tc-pertechnetate HPS; however, the two methods complement each other in the assessment of microcirculation in SSc., Competing Interests: Conflict of Interest: The authors declared no conflicts of interest with respect to the authorship and/or publication of this article., (Copyright © 2020, Turkish League Against Rheumatism.)

The study aims to analyze the effects of induction treatment with cyclophosphamide (CYC) pulse therapy followed by maintenance treatment with other mild immunosuppressive agents on lung function in scleroderma (SSc) patients. Thirty patients with SSc (mean age 52 years, mean disease duration < 2 years) with forced vital capacity (FVC) ≤ 80% and/or diffusing capacity of carbon monoxide (DLco) ≤ 70% were included. Monthly CYC pulses were given for 6 months (induction treatment), followed by 3-monthly maintenance pulses for the next 18 months, and during the next 5 years patients received other mild immunosupressive therapy brought by the competent rheumatologist. The efficacy was evaluated by comparing FVC% and DLco% after 6, 24, and 84 months from the baseline. All patients completed induction and maintenance treatment with CYC. Three patients were lost to follow-up. The rest of 27 patients, during the next 5 years, received other immunosupressive agents (14 azathioprine, 9 methotrexate, and 4 mycophenolate mofetil). Three patients died in the 4 years of follow-up. By 6, 24, and 84 months, the mean FVC and DLco changes were + 0.47 and + 2.10, + 3.30 and - 2.49, and + 1.53 and - 3.76%, respectively. These changes were not significantly different from the baseline values. CYC does not appear to result in clinically significant improvement of pulmonary function but fulfilled criteria of stable disease. Maintenance treatment with other mild immunosupressive agents preserves the benefits achieved during CYC treatment.

Silicone has been widely used in the manufacture of medical implants. It is well tolerated in most cases. However, in this paper we report the cases of three women who developed autoimmune/inflammatory syndrome induced by adjuvants (ASIA syndrome), namely with silicone breast implants. The symptoms in these cases include arthralgia, arthritis, myalgia, sleep disturbances, the appearance of autoantibodies, miscarriage, Raynaud's phenomenon, and involvement of autoimmune diseases (scleroderma and undifferentiated connective tissue diseases). In one patient, breast implants were removed, but no improvement was seen after the removal. The remaining two patients received the updated information about their condition, and they decided not to remove the implants. In conclusion, earlier reports that silicone is biologically relatively inert have recently been challenged with the description of ASIA syndrome.

We assess the usefulness of 99m Tc-pertechnetate hand perfusion scintigraphy in patients with Raynaud's phenomenon (RP). The study population consisted of 18 patients with primary RP, 25 patients with secondary RP within systemic sclerosis (SSc), and ten healthy individuals. Gamma camera dynamic first-pass study during the first 60 s and a static scintigraphy after 5 min were recorded following a bolus injection of 99m Tc-pertechnetate via a cubital vein. Regions of interest were drawn on the summed images around the fingers and the palmar region. The fingers-to-palm ratios were then calculated. The mean fingers-to-palm ratio for dynamic study (blood flow) was 0.58 ± 0.19 for the healthy group, 0.45 ± 0.18 for the primary RP, and 0.43 ± 0.21 for the SSc patients. The mean fingers-to-palm ratio for static study (blood pool) was 0.44 ± 0.06 for the healthy group, 0.42 ± 0.06 for the primary RP, and 0.36 ± 0.07 for the SSc patients. Analysis of variance showed these differences to be significant (p = 0.039 from blood flow and p = 0.004 from blood pool). The receiver operating characteristic curve showed sensitivity of 80% and a specificity of 60% when using cutoff values of 0.40 for blood flow and sensitivity of 79% and a specificity of 70% when using cutoff values of 0.37 for blood pool. Our method is able to differentiate between patients with normal and those with abnormal microcirculation of the hands. Dynamic study separates the healthy subjects from patients with RP, while static study separates primary from secondary RP.

Klippel-Feil syndrome is defined as congenital fusion of two or more cervical vertebrae. In this article, we report a 55-year-old male patient with one-year history of neck pain, headaches, and one episode of syncope after a severe trauma. X-rays and magnetic resonance imaging of cervical spine revealed fused vertebral bodies of C2-C5. The major anomalies associated with Klippel-Feil syndrome (small stature, thoracic kyphoscoliosis, lumbar scoliosis, restricted opening mouth, and bilateral sensorineural hearing loss) as well as multiple minor anomalies (mild face asymmetry, high arched palate, rhinoscoliosis, high nasal bridge, inclined septi nasi, and thin upper lip) were detected. This is a rare case describing the anomalies of the nose in Klippel-Feil syndrome patients. Our patient had no central cord impairment following a severe trauma., Competing Interests: Conflict of Interest: The authors declared no conflicts of interest with respect to the authorship and/or publication of this article.

Objectives: To distinguish patients (pts) with enthesitis having spondyloarthritis (SpA) from pts with enthesitis without SpA by ultrasound (US) enthesitis score., Methods: The study sample included 127 pts with enthesitis (76 pts with SpA, 26 pts with rheumatoid arthritis, 25 pts with mechanically-related enthesitis). The entheses of plantar fascia, Achilles, patellar, quadriceps and common extensor tendon on lateral epicondyle were examined by US. Two operators, blinded to clinical diagnosis and enthesitis symptoms, assessed enthesis thickness, echogenicity, enthesophytes, power Doppler signal and erosions. Logistic regression and receiver operating characteristic (ROC) curve analysis were used to determine the predictive value of each enthesitis lesion for diagnosis of SpA. The best predictive value for SpA was accomplished when absence and presence of increased thickness, hypoechogenicity and enthesophytes were scored as 0 and 1; absence and presence of PD and erosions were scored as 0 and 4. Belgrade Ultrasound Enthesitis Score (BUSES) represents a cumulative score of derived enthesitis lesion scores at examined entheses. Independent-samples t-test was used for BUSES comparison between pts with and without SpA. Validity of BUSES for SpA diagnosis was evaluated by sensitivity and specificity. Cut-off point was chosen as the smallest value with specificity of at least 90%. The reliability was analysed by intra-class-correlation coefficient (ICC)., Results: BUSES was 9.9 ± 12.4 (mean ± SD) in SpA pts and 3.1 ± 4.2 in pts without SpA (p<0.001). BUSES cut-off point ≥ 7 achieved excellent specificity (90.2%) and fair sensitivity (47.4%). ICC was 0.99., Conclusions: BUSES is highly specific, valid and reliable to identify patients with SpA.

To assess the prognostic value of the age at onset of Raynaud's (RP) and of a history of exacerbation of RP attacks for the development of connective tissue disease (CTD) in patients initially found to have primary Raynaud's. 3,035 patients with primary RP (2,702 women and 333 men) were followed for an average of 4.8 years (range from 1 to 10 years). At baseline and every 6 months, they were screened for signs and symptoms of CTD. At 4.8 years of follow-up, 54.7 % patients remained as primary RP, 8.1 % had developed suspected secondary RP, and 37.2 % had developed a definite CTD. Primary RP patients had an earlier onset of RP (mean age of 32.2 years) than those with suspected (mean age 36.5 years, P = .007) or definite secondary RP associated with CTD (mean age of 39.8 years, P = .004). RP beginning before the age of forty was not significantly associated with the development of CTD. Conversely, the appearance of RP after the age of 40 was significantly associated with the development of CTD (P = .00001). Worsening of RP attacks predicted the development of CTD, especially systemic sclerosis (relative risk [RR] of 1.42), scleroderma overlap syndrome (RR of 1.18), and mixed CTD (RR of 1.18). Patients whose onset of RP occurred past 40 years of age and those with worsening RP attacks were at risk for the future development of CTD.

The aim of this study was to assess the association between Raynaud's phenomenon (RP) and specific capillaroscopic findings in patients with SLE and particular clinical manifestations of the disease. A total of 79 patients with SLE were included in the study: 44 of them (43 women) with RP and 35 (32 women) age-, sex-, and disease-duration-matched patients with SLE without RP. Demographic variables, clinical manifestations, laboratory and nailfold capillaroscopy findings were compared between the two groups. Central nervous systemic involvements (P = 0.0038) and peripheral neuropathy (P = 0.0336) were significantly more common in SLE patients with RP, while secondary Sjögren's syndrome (P = 0.0363) was more common in SLE patients without RP. RP occurred in 52 % of patients before SLE onset while 48 % of patients developed RP after they had been diagnosed with SLE. Arthritis/arthralgia (P = 0.0073) was significantly more common in patients who had been diagnosed with RP before the onset of SLE, while mucosal ulcers were more common in patients who contracted RP after the onset of SLE (P = 0.0258). Enlarged capillaries (P = 0.0482), presence of avascular areas (P = 0.0476), capillary hemorrhages (P = 0.0482), and granular blood flow (P = 0.0482) were more common in patients with SLE who also suffered from RP, than in patients with SLE without RP. The frequency of normal (63.6 vs. 82.9 %, P = 0.100) and nonspecific (25 vs. 17.1 %, P = 0.5696) capillaroscopy findings were similar in either groups. Scleroderma-like pattern of capillaroscopy finding was only found in patients with RP [(11.4 %), P = 0.0482]. RP in our patients with SLE was associated with specific clinical manifestations, indicating that prognostic relevance of RP in SLE should be evaluated.

To assess the prognostic value of scleroderma pattern of nailfold capillary changes for the development of connective tissue diseases (CTD) in subjects with primary Raynaud's phenomenon (RP). The study included 3,029 consecutive patients with primary RP who had been followed at 6-month intervals during the mean of 4.8 years. The pathological features of nailfold capillaroscopy were recorded in all patients who had neither clinical nor serological signs of a CTD. In patients who developed CTD, capillary changes obtained 6 months prior to diagnosis were analyzed. A possible relationship between capillary changes and the presence of associated CTD was assessed. At the end of follow-up, 1,660 (54,8%) patients have still the primary RP, 246 (8,1%) had suspected secondary RP, and 1,123 (37,1%) patients developed CTD (363 undifferentiated CTD, 263 systemic sclerosis, 143 systemic lupus erythematosus, 106 rheumatoid arthritis, 102 Sjögren's syndrome, 61 overlap syndrome, 30 vasculitides, 24 mixed CTD, 19 polymyositis, 7 dermatomyositis, and 5 primary antiphospholipid syndrome). Scleroderma pattern were significantly associated with the development of systemic sclerosis [P = .00001, sensitivity 94%, specificity 92%, positive predictive value 52%, negative predictive value 99%, and odds ratio 163 (95% CI, 97,9-271,5)], as well as dermatomyositis (P = .0004), overlap syndrome with signs of systemic sclerosis (P = .0001), and mixed connective tissue disease (P = .007). Capillary microscopy is effective method for differentiation between primary and secondary RP and useful tool for the prediction of scleroderma spectrum disorders in RP patients.

Objective: To examine the difference in clinical signs of peripheral vasculopathy in patients (pts) with limited (lcSSc) and diffuse cutaneus systemic sclerosis (dcSSc)., Patients and Methods: Ninety one patients with systemic sclerosis (39 with lcSSc and 52 with dcSSc) have been assessed for the presence of clinical signs of vascular injury: Raynaud's phenomenon, severity of capillary damage on capillaroscopy, presence or absence of finger-tip ulcers or pitting scars, presence of telangiectasias and radiographic signs of finger-tip osteolysis. Statistical significance of difference in clinical manifestations of peripheral vasculopathy in pts with lcSSc and dcSSc was assessed using the Mann-Whitney and X2-test., Results: Duration of Raynaud's phenomenon before manifestation of skin or internal organ damage, was significantly longer (z=-2.54, p=0.004) in patients with lcSSc (5.4 years) than in patients with dcSSc (1.9 years). Using the technique of nailfold capillaroscopy, we found normal capillaries or non-specific capillary change in 10.2% pts with lcSSc and only in 2.0% pts with dcSSc. Enlarged capillaries without significant loss of capillaries were found in 38.5% pts with lcSSc, and 11.5% pts with dcSSc (p=0.05). But severe capillary damage, with significant loss of capillaries, was noticed more frequently in pts with dcSSc (dcSSc/lcSSc: 86.5%/51.3%, p=0.002). Pitting scars or digital ulcers were found in 46.2% pts with lcSSc and 67.3% pts with dcSSc (p=0.04). We did not notice significant difference in frequency of finger-tip osteolysis (lcSSc/dcSSc: 23.1%/21.2%, p>0.05) and telangiectasias (lcSSc/dcSSc: 46.2%/53.8%, p>0.05)., Conclusion: Severe capillary damage and digital ulcers are more common in patients with diffuse cutaneus systemic sclerosis, but finger-tip osteolysis and telangiectasias are equally frequent in both form of disease.