Raynaud's Phenomenon Research Articles

Hyperspectral imaging in systemic sclerosis-associated Raynaud phenomenon

Background/purposeLack of robust, feasible, and quantitative outcomes impedes Raynaud phenomenon (RP) clinical trials in systemic sclerosis (SSc) patients. Hyperspectral imaging (HSI) non-invasively measures oxygenated and deoxygenated hemoglobin (oxyHb and deoxyHb) concentrations and oxygen saturation (O2 sat) in the skin and depicts data as oxygenation heatmaps. This study explored the potential role of HSI in quantifying SSc-RP disease severity and activity.MethodsPatients with SSc-RP (n = 13) and healthy control participants (HC; n = 12) were prospectively recruited in the clinic setting. Using a hand-held camera, bilateral hand HSI (HyperMed™, Waltham, MA) was performed in a temperature-controlled room (22 °C). OxyHb, deoxyHb, and O2 sat values were calculated for 78-mm2 regions of interest for the ventral fingertips and palm (for normalization). Subjects underwent a cold provocation challenge (gloved hand submersion in 15 °C water bath for 1 min), and repeated HSI was performed at 0, 10, and 20 min. Patients completed two patient-reported outcome (PRO) instruments: the Raynaud Condition Score (RCS) and the Cochin Hand Function Scale (CHFS) for symptom burden assessment. Statistical analyses were performed using the Mann-Whitney U test and a mixed effects model (Stata, College Station, TX).ResultsNinety-two percent of participants were women in their 40s. For SSc-RP patients, 69% had limited cutaneous SSc, the mean ± SD SSc duration was 11 ± 5 years, and 38% had prior digital ulcers—none currently. Baseline deoxyHb was higher, and O2 sat was lower, in SSc patients versus HC (p < 0.05). SSc patients had a greater decline in oxyHb and O2 sat from baseline to time 0 (after cold challenge) with distinct rewarming oxyHb, O2 sat, and deoxyHb trajectories versus HCs (p < 0.01). There were no significant correlations between oxyHb, deoxyHb, and O2 sat level changes following cold challenge and RCS or CHFS scores.ConclusionHyperspectral imaging is a feasible approach for SSc-RP quantification in the clinic setting. The RCS and CHFS values did not correlate with HSI parameters. Our data suggest that HSI technology for the assessment of SSc-RP at baseline and in response to cold provocation is a potential quantitative measure for SSc-RP severity and activity, though longitudinal studies that assess sensitivity to change are needed.

Pulmonary veno-occlusive disease in Sjogren's syndrome: a case report

BackgroundPulmonary arterial hypertension (PAH) associated with connective tissue disease (CTD) belongs to Group 1 pulmonary hypertension. Pulmonary veno-occlusive disease (PVOD), which is characterized by venous system aberrations, has been previously reported in CTD-PAH; however, it has rarely been observed in Sjogren’s syndrome (SS).Case presentationOur 28-year-old female patient was admitted to the hospital with recurrent shortness of breath even after minimal physical activity. Her chest high-resolution CT scan demonstrated pulmonary artery dilatation and bilateral ground-glass nodules. A subsequent right heart catheterization confirmed pulmonary hypertension because her mean pulmonary arterial pressure was 62 mmHg. Our inquisitive genomic assessment identified a novel EIF2AK4 mutation at c.1021 C > T (p. Gln341*), the dominant causal gene of PVOD. Histological examination demonstrated stenosis and occlusions in the pulmonary veins. Because she presented with features such as dry eyes and Raynaud's phenomenon, we performed a biopsy on the labial salivary gland, which confirmed SS. Her treatment regimen included PAH-targeted therapies (tadalafil and macitentan) in combination with hydroxychloroquine. Although she was hospitalized several times due to acute exacerbation of PAH, her disease progression was under control, and she did not demonstrate any signs of pulmonary edema even after a three-year treatment period.ConclusionHere, we report the case of an SS-PAH patient with PVOD who carried a novel biallelic EIF2AK4 mutation, and PAH-targeted therapies were well tolerated by our patient.

A deep learning system for quantitative assessment of microvascular abnormalities in nailfold capillary images.

Nailfold capillaroscopy is key to timely diagnosis of SSc, but is often not used in rheumatology clinics because the images are difficult to interpret. We aimed to develop and validate a fully automated image analysis system to fill this gap. We mimicked the image interpretation strategies of SSc experts, using deep learning networks to detect each capillary in the distal row of vessels and make morphological measurements. We combined measurements from multiple fingers to give a subject-level probability of SSc.We trained the system using high-resolution images from 111 subjects (group A) and tested on images from subjects not in the training set: 132 imaged at high-resolution (group B); 66 imaged with a low-cost digital microscope (group C). Roughly half of each group had confirmed SSc, and half were healthy controls or had primary RP ('normal'). We also estimated the performance of SSc experts. We compared automated SSc probabilities with the known clinical status of patients (SSc versus 'normal'), generating receiver operating characteristic curves (ROCs). For group B, the area under the ROC (AUC) was 97% (94-99%) [median (90% CI)], with equal sensitivity/specificity 91% (86-95%). For group C, the AUC was 95% (88-99%), with equal sensitivity/specificity 89% (82-95%). SSc expert consensus achieved sensitivity 82% and specificity 73%. Fully automated analysis using deep learning can achieve diagnostic performance at least as good as SSc experts, and is sufficiently robust to work with low-cost digital microscope images.

A CLINICAL STUDY ON NONSPECIFIC MUCOCUTANEOUS MANIFESTATIONS OF SYSTEMIC LUPUS ERYTHEMATOSUS &amp; ITS ASSOCIATION WITH SYSTEMIC INVOLVEMENT

Background : Systemic lupus erythematosus (SLE) is a chronic autoimmune connective tissue disorder which presents with multiple mucocutaneous manifestations along with various internal organ involvement. Skin manifestation may be either specic or nonspecic. At times, they could be an indicator of internal disease activity. To estimate the prevalence and pattern of the nonspecic mucocutaneous manifestations in Aims and objective : systemic lupus erythematosus and association with internal organ involvement. Materials and methods : This is a cross sectional study and hospital based, done at Government Rajaji Hospital, Madurai Medical College, Madurai over a period of 6 months from July 2020 to December 2020. Newly diagnosed patients are selected according to 2012 SLICC criteria and all relevant investigations were carried out. Results : A total of 23 patients were studied and the female to male ratio was 9:1. The mean of onset of the disease is 32 years and belonged to the age group of 4th decade of life. Almost all the patients had specic skin lesions and nonspecic skin lesions were observed in 80% of them (n=18). The common non specic manifestation was palmar erythema (n=7) followed by telogen efuvium in 4 patients. Small vessel vasculitis was observed in 3 patients. Livedo reticularis, sclerodactyly and urticaria were seen in 2 patients each. Features of erythromelalgia, raynauds phenomenon, PAN like vasculitis, alopecia areata, erythema nodosum &amp; rheumatoid nodule were seen in 1 patient each. Musculoskeletal involvement is seen in majority of the patients (n=15). A strong association with signicant p value was seen between palmar erythema and renal involvement. A thorough knowledge of the various cutaneous manifestations, both Conclusion: specic and nonspecic is necessary for the early diagnosis of the disease, which in turn will help in controlling &amp; preventing the progression of the disease. In addition some of the nonspecic cutaneous lesions can be the clinical marker of systemic association. Further studies with large sample size are needed to conrm it which are the limitations in our study.

Central and peripheral mechanisms of pain in fibromyalgia: scoping review protocol.

Fibromyalgia is characterised by widespread musculoskeletal pain, which may present with fatigue, depression, anxiety, sleep and cognitive disturbances. It is the second most prevalent rheumatic disease. An accurate diagnosis is challenging, since its symptoms may resemble diverse conditions such as carpal tunnel syndrome, Raynaud syndrome, Sjögren syndrome, amongst others. Neuropathic pain and autonomic dysfunction in fibromyalgia suggest the involvement of the nervous system. Ion channels, neurotransmitters and neuromodulators may play a role. Small fibre neuropathy (SFN) may also cause chronic widespread pain. SFN may occur in 50% of fibromyalgia patients, but its role in the disease is unknown. Despite several efforts to synthesise the evidence on the mechanisms for pain in fibromyalgia, there are few studies applying an integrative perspective of neurochemical, immunological, and neuroanatomical characteristics, and their relevance to the disease. This protocol aims to clarify the mechanisms of the central and peripheral nervous system associated with pain in fibromyalgia. We will retrieve published studies from Web of Science, MEDLINE, Scopus, EBSCOhost, Ovid and Google Scholar. All clinical studies or experimental models of fibromyalgia reporting imaging, neurophysiological, anatomical, structural, neurochemical, or immunological characteristics of the central or peripheral nervous systems associated with pain will be included. Exclusion criteria will eliminate studies evaluating pain without a standardised measure, studies written in languages different from Spanish or English that could not be appropriately translated, and studies whose full-text files could not be retrieved after all efforts made. A narrative synthesis will be performed.

Critical digital ischemia and biliary cholangitis related to graft versus host disease: A case report and systematic literature review.

Chronic graft versus host disease (cGVHD) is a systemic immune-mediated complication that occurs in approximately half of patients undergoing allogeneic hematopoietic stem cell transplantation (allo-HCT), and remains the leading cause of late morbidity and mortality. cGVHD involves a heterogeneous group of organic manifestations, many of which mimic autoimmune diseases such as scleroderma, primary biliary cholangitis, Sjögren syndrome and polymyositis. A 60-years-old female with a history of allo-HCT developed de novo cGVHD 11 months after allo-HCT with isolated liver involvement. The patient presented with jaundice, cytolysis, cholestasis and concomitant acute digital ischemia. Liver biopsy and autoimmunity tests were performed and were found to be compatible with immune-mediated liver damage. Nailfold capillaroscopy revealed microangiopathy, characterized by avascular areas and some enlarged capillaries resembled an early systemic sclerosis pattern. Biliary cholangitis-like and digital ischemia related to cGVHD. The patient was treated with high-dose prednisone and ursodeoxycholic acid, and extracorporeal photopheresis. The patient required hospital admission for administration of intravenous prostacyclin due to refractory Raynaud syndrome. After 6 to 8 weeks, the patient achieved a good response, with evident clinical improvement and progressive normalization of liver function. cGVHD is a multiorgan pathological condition, and this case emphasizes that a multidisciplinary team, including rheumatologists, should be involved in the follow-up of allo-transplant patients to ensure that the clinical complications are adequately addressed. Early intervention is critical for improving patient' prognosis.In addition, we performed a systemic literature review based on published case articles on hepatic cGVHD and digital ischemia published up to August 2022. To the best of our knowledge, this is the first reported case of such an association.

Does the use of nail cosmetics interfere with the reporting of nailfold capillaroscopy?

Capillaroscopy is a non-invasive tool evaluating the nail-fold capillaries, especially in approach to Raynaud's phenomenon (RP) and scleroderma (SSc) spectrum of diseases. It was recommended that the patients should not use any cosmetic procedure involving the nailfold to avoid misinterpretations. Therefore, we aimed to find the problems of using or recent removal of the nail polishes/artificial nails/henna before doing capillaroscopy. During 10years, we looked for all capillaroscopy reports and nail fold images of patients who referred for capillaroscopy and had used or recently removed nail polish/artificial nail/henna in order to find the presence of any artifacts or misinterpretations in reports. Sixty-three patients were identified that had used or removed the nail cosmetic during 10days before capillaroscopy. In patients who used nail polish, removed their nail polish, used artificial nails, or removed the artificial nails, and those using henna, 16.2%, 36.4%, 3.8%, 0%, and 1.4% of nail folds showed some stains in the upper part of the nail fold area, respectively, that had no interference with the report due to their distinct color. However, few areas were covered with polish stains in patients who removed the polish recently. The presence of nail cosmetics including nail polishes, henna, and artificial nails at the time of capillaroscopy does not induce a significant misinterpretation in capillaroscopy. However, some colored stains which were mostly distinguishable from hemorrhages could be seen. The removal of them 10days before the time of capillaroscopy did not decrease the cosmetic artifacts. Key Points • The presence of nail cosmetics including nail polishes, henna, and artificial nails at the time of capillaroscopy does not induce a significant misinterpretation in capillaroscopy. • The removal of nail polish 10 days before the time of capillaroscopy did not decrease the procedure artifacts. • The removal of nail polish 10 days before capillaroscopy increased the area of artifacts and coverage of the study background.

Diagnostic accuracy and predictive value of autoantibody profiles in patients with systemic sclerosis: a single-center study.

To determine diagnostic accuracy and evaluate the predictive value of autoantibody profiles in patients with systemic sclerosis (SSc). A total of 140 patients with SSc (125 female, mean age 54.2 ± 14.2years) were analyzed by a multiplex line immunoassay (Euroimmun) for autoantibodies against 12 SSc-related antigens. Associations between the presence of the autoantibodies and demographic clinical manifestations of patients with SSc were investigated. The sensitivity and specificity of this assay were as follows: 32.9% and 99.4% for anti-Scl-70, 29.3% and 88.9% for anti-CENP A, 28.6% and 87.8% for anti-CENP B, 7.1% and 97.8% for anti-RP11, 5.7% and 100% for anti-RP155, 2.9% and 99.4% for anti-NOR 90, 2.9% and 98.9% for anti-Th/To, 1.4% and 96.7% for anti-PM-Scl-100, 5.0% and 98.3% for anti-PM-Scl-75, and 2.9% and 97.2% for anti-Ku, respectively. Anti-Scl-70 was significantly associated with sine scleroderma (P = 0.003), digital ulcers (P = 0.047), and Raynaud's phenomenon as the first clinical manifestation of onset (P = 0.017). SSc-ILD was more common in patients with anti-Scl-70 (P = 0.029) and less frequent in patients with anti-CENP A (P < 0.001) and anti-CENP B (P < 0.001). There was a significant association between PAH with anti-CENP A (P = 0.008) and anti-CENP B (P = 0.025). Renal involvement was significantly related to anti-NOR90 (P = 0.026) and anti-Th/To (P = 0.026). This study confirmed the important role of autoantibodies in accurately diagnosing SSc. The autoimmune profile of patients with SSc was related to specific disease manifestations. Key Points • Autoantibody profiles were useful for diagnosing SSc and predicting clinical features of patients.

Individualized Net Benefit estimation and meta-analysis using generalized pairwise comparisons in N-of-1 trials.

The Net Benefit (Δ) is a measure of the benefit-risk balance in clinical trials, based on generalized pairwise comparisons (GPC) using several prioritized outcomes and thresholds of clinical relevance. We extended Δ to N-of-1 trials, with a focus on patient-level and population-level Δ. We developed a Δ estimator at the individual level as an extension of the stratum-specific Δ, and at the population-level as an extension of the stratified Δ. We performed a simulation study mimicking PROFIL, a series of 38 N-of-1 trials testing sildenafil in Raynaud's phenomenon, to assess the power for such an analysis with realistic data. We then reanalyzed PROFIL using GPC. This reanalysis was finally interpreted in the context of the main analysis of PROFIL which used Bayesian individual probabilities of efficacy. Simulations under the null showed good size of the test for both individual and population levels. The test lacked power when being simulated from the true PROFIL data, even when increasing the number of repetitions up to 140 days per patient. PROFIL individual-level estimated Δ were well correlated with the probabilities of efficacy from the Bayesian analysis while showing similarly wide confidence intervals. Population-level estimated Δ was not significantly different from zero, consistently with the previous Bayesian analysis. GPC can be used to estimate individual Δ which can then be aggregated in a meta-analytic way in N-of-1 trials. GPC ability to easily incorporate patient preferences allow for more personalized treatment evaluation, while needing much less computing time than Bayesian modeling.

Raynaud's Phenomenon: A Brush Up!

Raynaud's Phenomenon: A Brush Up!

Clinical Features and Risk Factors of Rapidly Progressive Systemic Sclerosis in a Single Center in China: Anti-RNA Polymerase III Antibodies as a Predictor.

Systemic sclerosis (SSc) have been classified in two clinical subsets (diffuse and limited) based on the extend of skin thickening. In this study, we classified a novel subset of SSc defined rapidly progressive systemic sclerosis (RPSSc), which based on the rate of skin thickening progression and the progressive of interstitial lung disease (ILD). We aimed to evaluate RPSSc clinical characteristics and predictive factors in a Chinese single center. Overall, 75 patients diagnosed with SSc, classified into RPSSc (n = 14) and non-rapidly progressive SSc (non-RPSSc, n = 61) were retrospectively included in the study. Clinical characteristics, disease severity and autoantibodies were collected. Logistic regression, least absolute shrinkage, and selection operator (LASSO) regression analysis was used to identify RPSSc predictors. Receiver operating characteristic (ROC) analysis and Delong test was conducted to evaluate and compare different indexes. RPSSc rate was 18.7%. ILD (64.3%), cardiac involvement (42.9%) were the most common organ system involvement of RPSSc, while Raynaud's phenomenon incidence significantly decreased. Disease duration (12 vs 72, months), sex (42.9% vs 11.5%, male %), SSc subset (85.7% vs 27.9%, diffuse cutaneous SSc (dsSSc) %), modified Rodnan total skin score (mRSS) (20.5 vs 6), Raynaud's phenomenon (64.3% vs 98.4%), cardiac involvement (42.9% vs 18%), higher incidence with malignancy (28.6% vs 1.6%) and positive anti-RNA polymerase III antibodies (ARA) (64.3% vs 1.6%) were statistically significant differences among the RPSSc groups and non-RPSSc groups (p < 0.05). Univariate analysis showed that positive ARA, male, dsSSc and malignancy were RPSSc risk factors, while long-disease duration, Raynaud's phenomenon was RPSSc protective factors. ARA was the strongest factor associated to RPSSc (OR 108, 95% CI 11.287-1033.327, p < 0.001). LASSO logistic regression model identified six factors: Disease duration, dsSSc, malignancy, cardiac involvement, positivity of ARA were RPSSc risk factors, Raynaud's phenomenon was RPSSc protective factors. RPSSc is an SSc clinical category which should be accounted for early detection of organ involvement and close follow-up of malignancy. ARA might be used as a predictor for RPSSc and organ involvement.

Rheumatoid Arthritis Presenting with Raynaud’s Phenomenon in an Elderly: End of Road?

Rheumatoid Arthritis (RA) is an autoimmune and chronic inflammatory disorder where the immune system of the body attacks the healthy cells and causes inflammation. Raynaud’s Phenomenon (RP) is a disorder of small blood vessels supplying the distal parts of the limbs where decrease in blood supply leads to infarction of that area. It occurs secondary to other disorders as in this case with RA. The report is about an 80-year-old female patient who presented with chief complaints of bluish discolouration over distal parts of hands and feet as well as pain in joints of all limbs. She had gangrene developing over the tips of fingers and toes, ulcer over the lower end of right foot, cold extremities, swelling over elbow and knee joints, oedematous hands and feet, and loss of pin prick needle sensations. With all the clinical presentations and investigation findings, the patient was diagnosed with RA presenting with RP. The patient was first started with the treatment for RA and then was managed symptomatically for vasculitis. Due to the old age factor and the advanced stage of the disease, the patient developed septicaemia and died due to cardiac arrest.

Predicting cardiovascular risk in a Chinese primary Sjögren's syndrome population: development and assessment of a predictive nomogram.

Patients with primary Sjögren's syndrome (pSS) are at increased risk of cardiovascular morbidity as compared with the general population. A retrospective study on 349 Chinese patients with pSS was conducted to identify potential risk factors for cardiovascular events and develop a cardiovascular risk nomogram. This is a retrospective observational study. The study included 349 patients who were diagnosed with pSS at Tongji Hospital, School of Medicine, Tongji University, China from January 2010 to March 2022. The least absolute shrinkage and selection operator (LASSO) was used to select features for the cardiovascular risk model. The features selected in LASSO were used to build the cardiovascular risk model in a multivariate logistic regression analysis. C-index, receiver operating characteristic (ROC) curve, calibration plot, and decision curve analysis were used to assess the predictive model. Internal validation was performed by bootstrapping. Sex, joint pain as an initial symptom, dry mouth, oral ulcers, dental caries, Raynaud's phenomenon, fatigue, diabetes, elevated thyroid-stimulating hormone (TSH) level, and elevated systolic blood pressure were included in the nomogram for the prediction of cardiovascular risk. Our model had good discrimination (C-index: 0.824, 95% confidence interval: 0.712-0.936) and good calibration (C-index in the interval validation: 0.8). Decision curve analysis indicated that our nomogram demonstrated clinical usefulness for intervention in a cardiovascular disease possibility threshold of 3%. The cardiovascular risk nomogram incorporating sex, initial joint pain, dry mouth, oral ulcer, dental caries, Raynaud's phenomenon, fatigue, diabetes, elevated TSH, and systolic blood pressure could be used in the prediction of cardiovascular risk in patients with pSS and the guidance of further treatment.

Clinical characteristics of myositis patients with isolated anti-U1 ribonucleoprotein antibody resemble immune-mediated necrotizing myopathy.

Anti-U1 ribonucleoprotein (U1RNP) antibodies were associated with connective tissue diseases (CTDs), but the clinical characteristics of this antibody in Chinese myositis patients have not been studied. We aim to analyze the clinical features of myositis patients who test positive for anti-U1RNP antibodies and delineate a subgroup of myositis. This is a retrospective cohort study. We reviewed the clinical data of myositis patients with anti-U1RNP antibodies and compared them to those with anti-signal recognition particle (SRP) and hydroxy-3-methylglutaryl-CoA reductase (HMGCR) antibody-associated immune-mediated necrotizing myopathy (IMNM). A total of 30 adult cases were identified; median age was 47.5 years and 24 (80%) were females, and 12 patients did not coexist with myositis-specific antibodies (MSAs) (isolated anti-U1RNP). The serum creatine kinase (CK) was significantly higher in patients with isolated anti-U1RNP (2182.5 U/L versus 289 U/L, p = 0.01), and the number of patients with CK > 2000 U/L was higher compared to that in anti-U1RNP antibody patients coexisting with MSAs (66.7% versus 16.7%, p = 0.009). The prevalence of IMNM in patients' muscle pathology with isolated anti-U1RNP was significantly higher (75%, p = 0.003). Skin rashes were less common in isolated anti-U1RNP group (p < 0.05). Of the 25 individuals with available pulmonary high-resolution CT (HRCT), 14 (56%) were diagnosed with interstitial lung disease (ILD). The incidence of muscular weakness, dysphagia, or levels of CK was not different between the isolated anti-U1RNP antibody individuals and the anti-HMGCR or SRP-IMNM groups (p > 0.05). But the frequency of Raynaud's phenomenon, arthritis, and membrane attack complex (MAC) deposits in myositis patients with isolated anti-U1RNP antibodies were higher than in anti-HMGCR and SRP-IMNM (all p < 0.005). There was no difference between anti-U1RNP patients with and without Ro-52 (p > 0.05). Isolated anti-U1RNP individuals showed marked improvements in muscle strength, and the remission rate in 1 and 2 years was significantly higher than that in anti-HMGCR and SRP-IMNM (p < 0.05). The clinical and pathological features of myositis patients with isolated anti-U1RNP antibodies were similar to IMNM. Arthritis and ILD are the most common extramuscular clinical features. Most respond well to treatment and have a good prognosis.

Screening tool development for hand surgery referrals in systemic sclerosis

BackgroundSystemic Sclerosis (SSc) patients may need hand surgery. ObjectiveTo develop a screening tool for rheumatologists to identify potential candidates with systemic sclerosis for hand surgery, optimizing referrals. MethodsA pilot cross-sectional study from January 2015 to December 2016. Sample size: 51 participants. Inclusion criteria: ≥ 18 years old, meeting the 2013 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification criteria for SSc and hand impairment. Data collected: age, sex, race, disease duration, SSc subtypes, vasodilator use, skin thickness, finger stiffness, presence of Digital Ulcers (DU) and/or calcinosis, presence of Raynaud's Phenomenon (RP) attacks, health status and disability, disease status, pain intensity and functional status of the hands. Data were analyzed by a multivariate logistic regression model. ResultsFulfillment of surgical criteria: 68.8%. The surgical group had higher scores on the HAQ-DI (1.39 vs. 0.96, p = 0.032) and CHFS (25.0 vs. 12.0, p = 0.005) questionnaires, and a higher frequency of DU (91.43% vs. 18.75%, p < 0.0010), calcinosis (60.0% vs. 0.0%, p < 0.001), use of vasodilators (100.0% vs. 75.0%, p = 0.007) and digital stiffness (28.57% vs. 0.0%, p = 0.017). The presence of DU increased the chance of surgical indication by 46.2 times (ORIC 95% = 8.23 to 259.49). The statistical model showed good accuracy (86.3%, p < 0.001), sensitivity (91.4%), and specificity (81.2%). ConclusionThe presence of DU in SSc could be used as a screening feature for early identification and referral of potential candidates for hand surgery.

Rheumatology : what's new in 2022

In rheumatology, this year has seen an expansion of knowledge about the treatment of rheumatoid arthritis, with the availability of results from randomized trials evaluating a new molecule targeting IL-6, and regarding the safety profile of tofacitinib compared to TNF-alpha inhibitors. Interesting data on the outcome of pregnancy in patients with spondylarthritis have also been published. New molecules and different treatment strategies have shown promising results in psoriatic arthritis and systemic lupus erythematosus. The utility of botulinum toxin A injections for Raynaud's phenomenon and the efficacy of transplantation of autologous adipose-derived regenerative cells for the treatment of hand dysfunctions have been questioned by 2 randomized controlled trials of patients with systemic sclerosis.

PS-BPR02-2: ECULIZUMAB FOR RESCUE OF THROMBOTIC MICROANGIOPATHY IN MALIGNANT HYPERTENSION ASSOCIATED WITH MCTD

Introduction: Eculizumab is effective in atypical hemolytic uremic syndrome (aHUS) caused by complement abnormalities, but its effect on secondary thrombotic microangiopathy (TMA) is obscure. We here report a case of TMA associated with malignant hypertension, who was successfully treated with an ACE inhibitor, plasma exchange and eculizumab. Case report: The patient was a 40-year-old woman presenting with severe blood pressure elevation (BP 240/140 mmHg), nausea, fatigue, headache, and visual disturbances. She had a history of systematic lupus erythematosus (SLE) and had been stable by the treatment with 5 mg/day of prednisolone and 2 mg/day of tacrolimus for a long period. This time, she exhibited Raynaud's phenomenon, mild skin lesions, and heliotrope eruption on the eyelids, together with positive anti-U1-RNP antibodies. She was diagnosed with mixed connective tissue disease (MCTD), instead of SLE. Acute kidney injury (serum creatinine, 3.24 mg/dL), anemia (hemoglobin, 8.8 g/dL) and thrombocytopenia (platelets, 137,000/μL) were observed. LDH was elevated at 780 IU/L. The haptoglobin level was decreased to less than detection sensitivity and schistocytes were observed in her peripheral blood. The plasma renin activity was beyond the highest limit. On head MRI, posterior reversible encephalopathy syndrome (PRES) was suggested. A renal biopsy was performed, and a histological diagnosis of malignant hypertensive arteriolosclerosis was obtained. Based on these findings, she was diagnosed as accelerated malignant hypertension, possibly due to TMA with a background of MCTD. She was initially treated with nicardipine, enalapril and aliskiren, but this treatment failed to ameliorate the progression of kidney injury and TMA. Therefore, plasma exchange therapy was performed three days in a row, and then hemodialysis and administration of eculizumab were introduced. After weekly induction dose (900 mg once a week) was given four times, administration of 900–1200 mg of eculizumab every 2 weeks were continued. As the treatment was continued, her renal function was improved gradually. Fifteen months after her onset of AKI and TMA, the patient was withdrawn from dialysis and eculizumab therapy. Conclusion: A body of evidence is growing that activation of the complement system may be involved in scleroderma renal crisis. However, the efficacy of eculizumab in TMA, except for aHUS, remains controversial. Our case suggest a possible efficacy of eculizumab along with plasma exchange, as the treatment of malignant hypertension due to scleroderma that was caused by MCTD.

Nailfold Capillaroscopy in Early Stages of Mixed Connective Tissue Disease in a Sample of Egyptian Patients

Introduction: Mixed connective tissue disease (MCTD) is a systemic autoimmune disease with a high titer of anti-U1 small nuclear ribonucleoprotein particles and a number of clinical pictures, mainly Raynaud's phenomenon. Nailfold capillaroscopy (NFC) is a noninvasive diagnostic tool for patients with different connective tissue diseases that permit detection of local microvascular changes that correlate with systemic vascular involvement. Aim of the Study: The aim of this study was to compare the results of NFC in patients with systemic sclerosis (SSc) and MCTD to determine the chief characteristics of skin microangiopathy in early MCTD and attempt to describe a characteristic MCTD pattern in Egyptian patients. Methodology: This cross-sectional study included forty patients diagnosed with MCTD according to Alarcón-Segovia and Villareal criteria and twenty patients with confirmed systemic sclerosis according to the American College of Rheumatology and the European League against Rheumatism classification criteria for Systemic Sclerosis. Nailfold examination for the study subjects was performed describing architectural derangement, capillary density changes, mega capillary and enlarged loops, microhemorrhages, and angiogenesis. Results: Of the sixty patients studied, 49 (81.7%) patients were females and 11 were males with a mean age of 31 years with a median of 29.5 and range 16–52 years. Three of the twenty patients diagnosed with systemic sclerosis had arthritis. Out of sixty patients, 53.3% had thickened skin, 19 patients exhibited puffy fingers, six patients showed rash, and none had swollen joints. The skin was significantly thicker in systemic sclerosis patients (85%) compared to 37.5% in the MCTD population. Patients presented with various features, the most common of which was fatigue (26.7%) and myositis (23% of patients). There is a significant negative correlation of −0.508 (P = 0.022) between the enlargement scores and illness duration in systemic sclerosis patients. Those patients also exhibited a statistically significant positive correlation of 0.520 (P = 0.019) between hemorrhage score and the number of tender joints. Alternatively, patients diagnosed with MCTD exhibited a significant positive correlation between the architectural changes scores of their joints and both the duration of their illness (0.347; P = 0.028) and the number of swollen joints (0.424; P = 0.006). MCTD patients also showed a significant correlation of 0.423 (P = 0.007) between their hemorrhage scores and their age at the time of the study. Conclusion: In this study, the results obtained were qualitatively satisfactory for clear delineation of the nailfold capillaries features in MCTD. Therefore, it aids in the recognition of alternations in nailfold capillaries making an early prediction of course of illness in MCTD patients possible and suggests a potential ability to differentiate from early SSc, thus helps in preventing morbidities and sequelae of the disease.

Real-world nailfold videocapillaroscopy in a referral centre in north-western Colombia: A real-life cohort

IntroductionNailfold videocapillaroscopy is a non-invasive tool for the assessment of peripheral microcirculation, the main indication is the study of Raynaud's phenomenon, poorly standardized outside of this context. There is no clear information in real-life about the reasons for referral, the presence of clinical findings of autoimmune diseases, the frequency of patterns of autoantibodies, and specific capillaroscopic findings. ObjectiveThe purpose of this survey is to describe the sociodemographic, clinical, paraclinical, and angioscopy findings of a cohort of subjects referred to a capillaroscopy service in North-western Colombia. MethodsA retrospective study was conducted, from 2015 to 2018. Categorical variables were expressed in frequency and percentage and quantitative variables in mean and standard deviation or median with interquartile range, depending on the distribution of the data. ResultsA total of 318 capillaroscopies were performed for the first time. The main referral reason was Raynaud`s phenomenon (n = 134; 42.1%). The most frequent baseline capillaroscopic pattern found was normal (n = 123; 38.7%). Of the 12 capillaroscopies that presented a non-specific pattern at a 6-month follow-up, only one (8.3%) progressed to a scleroderma pattern. In the subjects with systemic sclerosis, the most frequent clinical finding was sclerodactyly (n = 34; 37.8%), and 42/44 individuals (95.4%) had positive antinuclear antibodies; the most frequent pattern was centromere (n = 27; 64.3%) ConclusionsIn a real-world setting, the main referral reason for capillaroscopy was Raynaud`s phenomenon; more than a third of the subjects had normal capillaroscopic findings. Sclerodactyly was the most frequent clinical finding in patients with scleroderma capillaroscopic pattern.

A Case of Cutaneous Vasculitis in the Setting of Systemic Lupus Erythematosus and Cocaine Use.

Cocaine, one of most prevalent illicit substances in the United States, affects a multitude of organ systems and precedes numerous negative health outcomes. Many of the consequences of cocaine are linked to induction of vasoconstriction. For this reason, cocaine users are placed at considerable risk of ischemic stroke, myocardial infarction, and cardiac arrhythmias. Furthermore, a prominent contaminant, levamisole, has been widely implicated in predisposing individuals to developing or exacerbating cutaneous vasculitides. This report details a 31-year-old woman with acute, localized necrotic skin lesions after cocaine use. Her clinical picture was complicated by a 17-year history of systemic lupus erythematosus (SLE) and Raynaud's phenomenon. This case examines the challenge of forming a differential diagnosis, initiating an appropriate workup, and interpreting serologic-based and immunologic-based studies to differentiate between SLE and drug-based etiologies of skin necrosis. Finally, we discuss appropriate treatment plans to mitigate symptoms and reduce future instances of drug-induced vasculitis.