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1.
Occup Med (Lond) ; 74(1): 128-130, 2024 02 19.
Article in English | MEDLINE | ID: mdl-37796654

ABSTRACT

This is the first known case report of COVID-19-related Raynaud's phenomenon (RP) identified at routine health surveillance of a male exposed to hand-transmitted vibration. The temporal relationship with COVID infection followed the course previously reported, being a late feature associated with mild COVID, and followed by gradual spontaneous recovery. It is not known whether the RP, in this case, is solely due to COVID or represents a summative effect of COVID and vibration exposure. A prudent approach is recommended in such circumstances with limitation of vibration exposure and continuing frequent surveillance pending improvement of the RP, with further prompt investigation if the expected recovery does not occur.


Subject(s)
COVID-19 , Raynaud Disease , Humans , Male , Vibration/adverse effects , COVID-19/complications , Hand , Raynaud Disease/complications
2.
Rheumatology (Oxford) ; 62(SI): SI74-SI81, 2023 02 06.
Article in English | MEDLINE | ID: mdl-35441683

ABSTRACT

OBJECTIVES: Silver fibre gloves transport heat from the palm to the fingers, possibly reducing the burden of RP in SSc patients. We aim to evaluate the clinical efficiency of this intervention. METHODS: A multicentre, double-blind, randomized trial was performed, accounting for interindividual differences and external factors using a crossover design. Patients were randomized in two groups: group 1 wore 8% silver fibre gloves in period 1 and normal gloves in period 2 and group 2 vice versa. Each period lasted 6 weeks. The primary outcome was the Raynaud Condition Score (RCS) over time (minimal clinical important difference 1.4), assessed three times per week using an online questionnaire. Secondary outcomes included vascular complications and Scleroderma-Health Assessment Questionnaire (SHAQ). Outcomes were evaluated before unblinding using linear mixed models. RESULTS: A total of 85 SSc patients were included, with 76 completing the study. The mean RCS during 2 weeks before the study (i.e. without gloves) was 6.4 (s.d. 1.6). Both with silver fibre gloves and normal gloves the mean RCS decreased to 3.9 (s.d. 2.3) with a similar course over time. There was no difference in mean RCS over time between the type of gloves [ß = 0.067 (95% CI -0.006, 0.19)]. Of secondary outcomes, total SHAQ [ß = 0.036 (95% CI 0.026, 0.046)] was slightly higher with silver fibre gloves, which is clinically irrelevant. Three patients developed new digital ulcers with normal gloves vs one patient with silver fibre gloves [odds ratio 3.2 (95% CI 0.32, 31.1)]. CONCLUSIONS: Wearing gloves in SSc patients clearly decreases the RP burden. Our results do not support the hypothesis that increased heat transport of 8% silver fibre gloves is associated with less disease burden as measured in this study by the RCS compared with normal gloves. CLINICAL TRIAL REGISTRATION NUMBER: Netherlands Trial register (https://www.trialregister.nl/) NL7904.


Subject(s)
Raynaud Disease , Scleroderma, Localized , Scleroderma, Systemic , Humans , Cross-Over Studies , Silver , Scleroderma, Systemic/complications , Scleroderma, Localized/complications , Raynaud Disease/complications
3.
Rheumatology (Oxford) ; 62(2): 824-828, 2023 02 01.
Article in English | MEDLINE | ID: mdl-35894657

ABSTRACT

OBJECTIVE: Our main aim was to investigate the effect of a single oral dose of C21, a selective angiotensin II type 2 receptor agonist, on cold-induced vasoconstriction in SSc-related RP. METHODS: This was a phase IIa, randomized, double-blind, cross-over, single-dose, placebo-controlled, single-centre study. Twelve female patients with SSc (median age 58.5 years, median duration of RP 19.0 years) attended on four occasions: screening, treatment visits 1 and 2 (separated by 3-7 days) and follow-up. At the first treatment visit, patients were randomized to receive either a single oral dose of C21 (200 mg) or placebo, then the opposite treatment on the second visit. Forty min after each treatment, each patient underwent a standard hand cold challenge. The primary end point was the area under the curve (AUC) for rewarming for each finger (eight fingers) over 15 min. Secondary end points included the maximum finger temperature after rewarming (MAX). Statistical analyses were performed by multiplicative ANCOVA models. RESULTS: For all eight fingers combined, mean AUC for rewarming was higher after treatment with C21 than after placebo (geometric mean 20 046°C*s vs 19 558°C*s), but not significantly (P = 0.380) and MAX (at 15 min) was also higher (geometric mean 23.5°C vs 22.5°C; P = 0.036). C21 was well tolerated. CONCLUSION: Despite the small trial size, a signal emerged suggesting that even in patients with established SSc, C21 may confer benefit for RP and deserves further investigation. TRIAL REGISTRATION: ClinicalTrials.gov, https://clinicaltrials.gov, NCT04388176.


Subject(s)
Raynaud Disease , Scleroderma, Systemic , Humans , Female , Middle Aged , Receptor, Angiotensin, Type 2/therapeutic use , Scleroderma, Systemic/complications , Scleroderma, Systemic/drug therapy , Scleroderma, Systemic/diagnosis , Fingers , Body Temperature , Raynaud Disease/etiology , Raynaud Disease/complications
4.
BMC Pediatr ; 23(1): 574, 2023 11 17.
Article in English | MEDLINE | ID: mdl-37978357

ABSTRACT

BACKGROUND: Kaposiform hemangioendothelioma (KHE) is a rare vascular neoplasm affecting infants or young children. KHE includes a spectrum of lesions, ranging from small and superficial tumors to large and invasive lesions with Kasabach-Merritt phenomenon (KMP). Currently, no published studies have reported a KHE presenting as thrombocytopenia and Raynaud phenomenon. CASE PRESENTATION: A 2-year-old boy with right hand swelling and thrombocytopenia was admitted to our hospital. His right hand turned swelling and red, even occasionally cyanotic. This condition became worse in response to cool environments and improved with warming, and platelet counts were between 50 ~ 80 × 10^9/L. Physical examination on admission revealed the swelling and frostbite-like rash of the right-hand fingers, and the skin temperature of the right hand was lower than the left. On day 3 of admission, chest CT results showed an irregular mass on the right side of the spine. The puncture biopsy demonstrated positive CD31, D2-40, and FLI1 immunohistochemical staining, but negative GLUT1 staining, confirming the diagnosis of KHE. Furthermore, endothelin-1 (ET1) expression levels significantly increased, and eNOS and A20 expression levels significantly decreased comparing with control patients. The patient received methylprednisolone and sirolimus treatments, and his condition gradually improved during the follow-up. CONCLUSIONS: We reported the first case of KHE presenting with thrombocytopenia and Raynaud phenomenon. The development of Raynaud phenomenon could be associated with increased ET-1 and reduced eNOS and A20 expressions. Careful differential diagnosis of hidden KHE should be considered in children with thrombocytopenia and Raynaud phenomenon.


Subject(s)
Hemangioendothelioma , Kasabach-Merritt Syndrome , Raynaud Disease , Sarcoma, Kaposi , Infant , Child , Male , Humans , Child, Preschool , Kasabach-Merritt Syndrome/complications , Kasabach-Merritt Syndrome/diagnosis , Kasabach-Merritt Syndrome/pathology , Hemangioendothelioma/complications , Hemangioendothelioma/diagnosis , Hemangioendothelioma/pathology , Sarcoma, Kaposi/complications , Sarcoma, Kaposi/diagnosis , Sarcoma, Kaposi/pathology , Raynaud Disease/complications , Raynaud Disease/diagnosis
5.
BMC Surg ; 23(1): 313, 2023 Oct 14.
Article in English | MEDLINE | ID: mdl-37838733

ABSTRACT

BACKGROUND: Raynaud's syndrome (RS), also referred to as Raynaud's phenomenon, is a vasospastic disorder causing episodic color changes in extremities upon exposure to cold or stress. These manifestations, either primary Raynaud's phenomenon (PRP) or associated with connective tissue diseases like systemic sclerosis (SSc) as secondary Raynaud's phenomenon (SRP), affect the quality of life. Current treatments range from calcium channel blockers to innovative surgical interventions, with evolving efficacy and safety profiles. METHODS: In this retrospective study, patients diagnosed with RS were selected based on complete medical records, ensuring homogeneity between groups. Surgeries involved microscopic excision of sympathetic nerve fibers and stripping of the digital artery's adventitia. Postoperative care included antibiotics, analgesia, oral nifedipine, and heat therapies. Evaluation metrics such as the VAS pain score and RCS score were collected bi-weekly. Data analysis was conducted using SPSS 26.0, with significance set at p < 0.05. RESULTS: In total, 15 patients formed the experimental group, with five presenting fingertip soft tissue necrosis and ten showing RS symptoms. Comparative analysis of demographic data between experimental and control groups, both containing 15 participants, demonstrated no significant age and gender difference. However, the "Mean Duration of RP attack" in the experimental group was notably shorter (9.47 min ± 0.31) than the control group (19.33 min ± 1.79). The RS Severity Score also indicated milder severity for the experimental cohort (score: 8.55) compared to the control (score: 11.23). Postoperative assessments at 2, 4, and 6 weeks revealed improved VAS pain scores, RCS scores, and other measures for the experimental group, showing significant differences (p < 0.05). One distinctive case showcased a variation in the common digital nerve and artery course in an RS patient. CONCLUSION: Our retrospective analysis on RS patients indicates that microsurgical techniques are safe and effective in the short term. As surgical practices lean towards minimally invasive methods, our data supports this shift. However, extensive, prospective studies are essential for conclusive insights.


Subject(s)
Quality of Life , Raynaud Disease , Humans , Retrospective Studies , Prospective Studies , Raynaud Disease/surgery , Raynaud Disease/complications , Minimally Invasive Surgical Procedures/adverse effects , Pain/complications
6.
Rheumatology (Oxford) ; 61(9): 3686-3692, 2022 08 30.
Article in English | MEDLINE | ID: mdl-35020814

ABSTRACT

OBJECTIVE: This study analysed the very early disease of SSc (VEDOSS) characteristics in a group of 217 patients with RP and at least one manifestation of SSc in search of predictors for the progression to SSc. METHODS: This was a cross-sectional single-centre analysis of patients presenting with RP with a specific SSc clinical manifestation or SSc autoantibody or SD pattern at nailfold capillaroscopy (SD-NFC), without skin involvement, who attended a scleroderma outpatient clinic between 2010 and 2019. The performance of VEDOSS and the importance of the combination of VEDOSS characteristics to predict the progression to SSc were evaluated. RESULTS: Among 217 patients, 153 (70.5%) were classified as SSc, including 65 (30%) in the first investigation; 69.3% of the SSc patients met VEDOSS criteria compared with 6.3% of patients who did not progress to SSc. The combinations most associated with progression to SSc were RP + puffy fingers (PF) + positive ANA + SD-NFC and/or SSc-specific antibody (VEDOSS level 2), with an odds ratio (OR) of 19.52 (95% CI 4.48, 85.06; P < 0.001) and RP + PF + positive ANA (VEDOSS level 1; 'red flags') (OR 15.45; P < 0.001), while combinations without non-RP clinical symptoms, as RP + SD-NFC (OR 0.03; P < 0.001) and RP + anticentromere + SD-NFC (OR 0.06; P = 0.006) were associated with non-progression to SSc. CONCLUSION: Among patients with RP with at least one manifestation of SSc, without skin involvement, combinations of VEDOSS characteristics were the strongest predictors of progression to SSc at a median follow-up of 4 years.


Subject(s)
Raynaud Disease , Scleroderma, Systemic , Cross-Sectional Studies , Early Diagnosis , Humans , Microscopic Angioscopy , Raynaud Disease/complications , Scleroderma, Systemic/complications
7.
Rheumatology (Oxford) ; 61(4): 1709-1716, 2022 04 11.
Article in English | MEDLINE | ID: mdl-34282441

ABSTRACT

OBJECTIVE: Anti-NOR90 antibodies are usually found in patients with SSc; however, their clinical relevance remains obscure. We developed an ELISA for measuring them to investigate the clinical features of patients with anti-NOR90 antibodies. METHODS: Serum samples from 1252 patients with various conditions from Nagoya University Hospital and 244 patients with idiopathic interstitial pneumonia (IIP) from Tosei General Hospital were included. Anti-NOR90 antibodies were assayed by an ELISA using the recombinant protein produced by in vitro transcription/translation. RESULTS: Five (0.4%) patients in the Nagoya University Hospital cohort had anti-NOR90 antibodies. One patient with diffuse cutaneous SSc, three with limited cutaneous SSc, and one with Raynaud's disease were positive for anti-NOR90 antibodies. Anti-NOR90 antibodies were found more frequently in patients with systemic scleroderma-spectrum disorders (SSDs) than without SSDs (5/316 vs 0/936, P <0.00101) and were found more frequently in patients with SSc than without SSc (4/249 vs 0/528, P <0.0104) in the systemic autoimmune rheumatic diseases cohort. Three of the four anti-NOR90-positive SSc patients had interstitial lung disease (ILD), and two of those four had cancer. Three (1.2%) patients in the Tosei General Hospital cohort had anti-NOR90 antibodies. All three of the anti-NOR90-positive IIP patients had gastrointestinal tract involvement, and two of those three had cancer or skin lesions observed in SSc. CONCLUSIONS: Although anti-NOR90 antibodies are rarely found in clinics, our ELISA is useful for their detection. Further studies are needed to confirm the association of anti-NOR90 antibodies with ILD and cancer in SSc and IIP patients.


Subject(s)
Idiopathic Interstitial Pneumonias , Lung Diseases, Interstitial , Raynaud Disease , Scleroderma, Systemic , Cohort Studies , Humans , Idiopathic Interstitial Pneumonias/complications , Lung Diseases, Interstitial/complications , Raynaud Disease/complications
8.
Rheumatology (Oxford) ; 61(5): 1975-1985, 2022 05 05.
Article in English | MEDLINE | ID: mdl-34463711

ABSTRACT

OBJECTIVE: In patients with RP, capillaroscopy is useful for discriminating primary from secondary causes. There are certain capillaroscopy and lab values as predictive factors leading to a known CTD. We conducted the present study to evaluate the causes of RP in our area and followed the studied subjects to find prognostic factors indicating a definite CTD or remaining a UCTD. METHODS: In this retrospective cohort study we included all adult patients with RP who were referred for capillaroscopy from 2010 to 2019. All the patients with primary and secondary RP with follow-up were evaluated for demography, laboratory results and capillaroscopy to find the risk factors of their progression to a CTD. RESULTS: A total of 760 of 776 patients were included, with 679 being female (89.3%) and 81 (10.7%) male. There were 660 subjects (90.8%) with secondary RP [mostly UCTD (48.2%) and then SSc (16.4%)] and 67 (9.2%) with primary RP; 109 patients were followed up and 42 (42%) of those with secondary RP developed a definite CTD. The scleroderma pattern and some capillary changes on capillaroscopy and/or positive ANA had statistically significant differences for CTD transition. CONCLUSION: We had a small number of patients with primary RP. The most prevalent causes of secondary RP in our patients were UCTD and SSc. Some capillaroscopy and laboratory results alone or in combination could be used as a predictive marker for the transition of patients with UCTD to CTD.


Subject(s)
Connective Tissue Diseases , Raynaud Disease , Adult , Capillaries/diagnostic imaging , Connective Tissue Diseases/complications , Connective Tissue Diseases/diagnostic imaging , Female , Humans , Male , Microscopic Angioscopy , Raynaud Disease/complications , Retrospective Studies
9.
Clin Exp Dermatol ; 47(2): 282-288, 2022 Feb.
Article in English | MEDLINE | ID: mdl-34342883

ABSTRACT

BACKGROUND: Although melanoma differentiation associated (MDA)-5 autoantibodies have been widely explored in dermatomyositis (DM), most studies have relied on MDA-5 autoantibody testing performed in research settings, rather than the now-available commercial laboratory tests. AIM: To characterize the clinical and histopathological data in patients with DM and circulating MDA-5 autoantibodies, as defined by commercially available testing. METHODS: This was a retrospective review of patients with DM who underwent MDA-5 antibody testing. All available skin biopsy slides were reviewed. RESULTS: Cutaneous features more prevalent in MDA-5-positive DM included Raynaud phenomenon (RP) (P < 0.001), cutaneous ulcerations (P = 0.01), mechanic hands (P < 0.02), palmar papules (P < 0.01), oral ulcers (P = 0.024) and alopecia (P = 0.03). Joint and pulmonary involvement were more common in patients with MDA-5-positive DM (both P < 0.001) as was dysphagia (P < 0.01). Myopathy (P = 0.4) and malignancy (P = 0.34) were not statistically different between the cohorts. Vasculopathy was more common in MDA-5-positive DM (P < 0.01), while spongiosis was less common (P < 0.02). CONCLUSION: This study not only confirms some known associations between disease manifestations and MDA-5 autoantibody status, as determined by commercially available tests, but also identifies new associations, including RP and dysphagia.


Subject(s)
Autoantibodies/blood , Dermatomyositis/pathology , Interferon-Induced Helicase, IFIH1/immunology , Skin/pathology , Biopsy , Deglutition Disorders/complications , Dermatomyositis/complications , Dermatomyositis/immunology , Female , Humans , Male , Raynaud Disease/complications , Retrospective Studies
10.
Int J Clin Pract ; 2022: 7025811, 2022.
Article in English | MEDLINE | ID: mdl-35936062

ABSTRACT

Objective: The present study aims to (1) analyze the clinical characteristics and related influencing factors of knee bone infarction in systemic lupus erythematosus (SLE) and (2) improve the understanding of SLE complicated with knee bone infarction. Methods: The data of patients with SLE complicated with knee bone infarction were retrospectively analysed; patients with SLE during the same period who matched in age, gender, and disease duration were selected as control subjects, with a 1 : 1 ratio with the SLE group. The clinical data were collected to analyze the risk factors for SLE complicated with knee bone infarction. Results: In a total of 36 (6.4%) of 563 patients aged 19-33 (25.8 ± 4.8) years who had SLE during the same period, the disease was complicated with knee bone infarction. The diagnosis of knee bone infarction was made at an SLE duration of 7-65 (26.2 ± 15.7) months. During the SLE course, knee bone infarction occurred within 1 year in 6 cases (16.7%), within 1-5 years in 28 cases (77.8%), and in >5 years in 2 cases (5.6%). Raynaud's phenomenon incidence and anti-nRNP antibody positivity were significantly higher in the knee bone infarction group than in the control group (P < 0.01 and P < 0.05, respectively). The cumulative glucocorticoid dose at 1, 3, and 6 months was significantly higher in the knee bone infarction group than in the control group (P < 0.05). SLE complicated with knee necrosis had a statistically significant rank correlation with Raynaud's phenomenon (r = 0.445, P < 0.001), anti-nRNP antibody (r = 0.309, P=0.008), and renal injury (r = 0.252, P=0.032). The multivariate analysis of SLE complicated with knee bone infarction showed that Raynaud's phenomenon was an independent influencing factor for the complicated knee bone infarction in SLE patients (OR = 4.938, P=0.004), and the probability of SLE complicated with knee bone infarction in Raynaud's phenomenon positive patients was 4.938 times that of Raynaud's phenomenon negative patients. Conclusions: The risk of knee bone infarction was relatively high in patients with SLE within a 5-year disease course and in young patients. The risk factors were Raynaud's phenomenon, anti-nRNP antibody positivity, and early high-dose glucocorticoid therapy.


Subject(s)
Lupus Erythematosus, Systemic , Raynaud Disease , Glucocorticoids/therapeutic use , Humans , Infarction/complications , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/drug therapy , Raynaud Disease/complications , Raynaud Disease/epidemiology , Retrospective Studies
11.
Vascular ; 30(5): 859-866, 2022 Oct.
Article in English | MEDLINE | ID: mdl-34256627

ABSTRACT

INTRODUCTION: Digital ischemia with subsequent severe pain and tissue loss is often difficult to treat, with no obvious guidelines or strong evidence in the literature to support a specific treatment modality. Patients who fail medical treatment remain with very limited surgical options due to the difficulty of any intervention in this "no man's land" area of the hand, as described since 1918. Extended distal periarterial sympathectomy is reported as an effective treatment option since the eighties of last century. The procedure entails large incisions and major technical difficulties. In this study, we describe a less invasive approach with very promising results and equally high success rates. MATERIALS AND METHODS: This was a prospective study. All patients with severe digital ischemia manifesting with bluish discoloration, ulceration, and/or dry gangrene who failed medical treatment underwent distal periarterial sympathectomy for the radial and ulnar arteries, with added digital sympathectomy in very severe cases. Primary endpoints were ulcer healing and improvement in pain scores assessed by Visual Analog Scale pain scoring system. Secondary endpoints included complications and amputation rates. RESULTS: This study recruited 17 patients between January 2019 and January 2020. The mean follow-up was 14.6 months. The mean age was 33.71 (±SD 13.14) years. 41% were males. 59% suffered from vasculitis, 35% of patients had dry gangrene, and 71% had ulcers. Periarterial radial and ulnar sympathectomy was performed for all cases, with digital sympathectomy for 12 fingers. We had 50% complete ulcer healing within 1 month (p = 0.031), and 100% were completely healed at 6 months (p < 0.001). Pain scores showed significant reductions at 1 (p = 0.001) and 6 months (p < 0.001) of follow-up. CONCLUSION: Distal periarterial sympathectomy demonstrates high success rates in terms of pain relief and ulcer healing in severe digital ischemia.


Subject(s)
Raynaud Disease , Adult , Female , Fingers/blood supply , Fingers/surgery , Gangrene/complications , Gangrene/surgery , Humans , Ischemia/diagnostic imaging , Ischemia/surgery , Male , Pain , Prospective Studies , Raynaud Disease/complications , Raynaud Disease/surgery , Sympathectomy/adverse effects , Sympathectomy/methods , Ulcer/surgery , Ulnar Artery
12.
Medicina (Kaunas) ; 58(7)2022 Jul 17.
Article in English | MEDLINE | ID: mdl-35888663

ABSTRACT

Background and Objectives: Systemic lupus erythematosus (SLE) is a chronic systemic autoimmune disease that affects predominantly women in the childbearing years. Patients may seek complementary therapies to manage their health and to reduce symptoms. However, to our knowledge, no studies have explored the association between clinical manifestations of SLE and complementary therapies. Therefore, this study aimed to investigate the association of complementary therapies with common clinical manifestations in Taiwanese female patients with SLE. Materials and Methods: A cross-sectional study was conducted at a regional teaching hospital in southern Taiwan. Outpatients from the rheumatology clinic who met the inclusion criteria were consecutively recruited. Demographic data, clinical manifestations of SLE, and types of complementary therapy use were determined using paper-based questionnaire. Multiple logistic regression analyses were conducted to investigate the use of complementary therapies associated with clinical manifestations of SLE. Results: Of the 317 female patients with SLE, 60.9% were 40 years or older. The five SLE clinical manifestations with the highest prevalence were Raynaud's phenomenon (61.2%), photosensitivity (50.2%), Sjögren's syndrome (28.4%), arthralgia and arthritis (22.1%), and renal involvement (14.5%). Multiple logistic regression analyses revealed that Raynaud's phenomenon was significantly associated with fitness walking or strolling (adjusted odds ratio [aOR] 1.77; p = 0.027) and fish oil supplements (aOR 3.55, p < 0.001). Photosensitivity was significantly and inversely associated with the use of probiotics (aOR 0.49; p = 0.019). Renal involvement was significantly associated with the use of probiotics (aOR 2.43; p = 0.026) and visit to the Chinese medicine department in a hospital (aOR 3.14, p = 0.026). Conclusions: We found that different clinical manifestations of SLE were associated with the use of different complementary therapies. Health care providers should have up-to-date knowledge of common complementary therapies and be ready to provide evidence-based advice to patients with SLE.


Subject(s)
Complementary Therapies , Lupus Erythematosus, Systemic , Raynaud Disease , Sjogren's Syndrome , Cross-Sectional Studies , Female , Humans , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/epidemiology , Lupus Erythematosus, Systemic/therapy , Male , Raynaud Disease/complications , Sjogren's Syndrome/complications
13.
Rheumatology (Oxford) ; 60(6): 2615-2628, 2021 06 18.
Article in English | MEDLINE | ID: mdl-33599244

ABSTRACT

OBJECTIVES: To identify clinical factors associated with cancer risk in the idiopathic inflammatory myopathies (IIMs) and to systematically review the existing evidence related to cancer screening. METHODS: A systematic literature search was carried out on Medline, Embase and Scopus. Cancer risk within the IIM population (i.e. not compared with the general population) was expressed as risk ratios (RR) for binary variables and weighted mean differences (WMD) for continuous variables. Evidence relating to cancer screening practices in the IIMs were synthesized via narrative review. RESULTS: Sixty-nine studies were included in the meta-analysis. DM subtype (RR 2.21), older age (WMD 11.19), male sex (RR 1.53), dysphagia (RR 2.09), cutaneous ulceration (RR 2.73) and anti-transcriptional intermediary factor-1 gamma positivity (RR 4.66) were identified as being associated with significantly increased risk of cancer. PM (RR 0.49) and clinically amyopathic DM (RR 0.44) subtypes, Raynaud's phenomenon (RR 0.61), interstitial lung disease (RR 0.49), very high serum creatine kinase (WMD -1189.96) or lactate dehydrogenase (WMD -336.52) levels, and anti-Jo1 (RR 0.45) or anti-EJ (RR 0.17) positivity were identified as being associated with significantly reduced risk of cancer. Nine studies relating to IIM-specific cancer screening were included. CT scanning of the thorax, abdomen and pelvis appeared to be effective in identifying underlying asymptomatic cancers. CONCLUSION: Cancer risk factors should be evaluated in patients with IIM for risk stratification. Screening evidence is limited but CT scanning could be useful. Prospective studies and consensus guidelines are needed to establish cancer screening strategies in IIM patients.


Subject(s)
Guidelines as Topic , Myositis/complications , Neoplasms/diagnosis , Adenosine Triphosphatases/immunology , Age Factors , Antibodies, Antinuclear/blood , Creatine Kinase/blood , DNA-Binding Proteins/immunology , Deglutition Disorders/complications , Dermatomyositis/complications , Dermatomyositis/diagnosis , Dermatomyositis/etiology , Female , Humans , L-Lactate Dehydrogenase/blood , Lung Diseases, Interstitial/complications , Male , Myositis/blood , Neoplasms/etiology , Publication Bias , Raynaud Disease/complications , Risk , Sex Factors , Skin Ulcer/complications , Tomography, X-Ray Computed , Transcription Factors/immunology
14.
Lupus ; 30(4): 608-614, 2021 Apr.
Article in English | MEDLINE | ID: mdl-33470159

ABSTRACT

OBJECTIVE: To characterize the ultrasound findings of the nail plate and nail bed in systemic lupus erythematosus (SLE) and its association with nail dystrophy. METHODS: Thirty-two SLE patients, 36 patients with osteoarthritis (OA) and 20 healthy individuals were studied. High-frequency linear ultrasound was performed in nails of the second to fifth fingers in all participants. Disease activity (SLEDAI-2K index), accrued organ damage (SLICC/ACR index), autoantibody profile, and Raynaud's phenomenon were also assessed in SLE patients. RESULTS: Nail bed thickness in SLE patients was higher than in healthy individuals (1.25 ± 0.31 mm vs 1.17 ± 0.29 mm; P = 0.01) but lower than in OA (1.39 ± 0.37 mm; P < 0.001), while nail plate thickness was similar among groups. Nail dystrophy was found more frequently in SLE and OA than in healthy individuals. SLE patients with nail dystrophy were older than their counterparts with no dystrophy (39.4 ± 10.4 years vs 27.8 ± 5.6 years; P = 0.004), although nail dystrophy showed no association with SLICC/ACR, SLEDAI-2K, nail bed vascularity, or autoantibodies. CONCLUSIONS: Nail bed in SLE patients is thicker than in healthy individuals but thinner than in OA patients. Nail dystrophy in SLE is associated with advanced age, but not with accrued organ damage, disease activity, Raynaud's phenomenon, or DIP synovitis assessed by ultrasound.


Subject(s)
Lupus Erythematosus, Systemic/complications , Nail Diseases/etiology , Nails/diagnostic imaging , Ultrasonography/methods , Adult , Age Factors , Autoantibodies/immunology , Female , Healthy Volunteers/statistics & numerical data , Humans , Lupus Erythematosus, Systemic/diagnosis , Lupus Erythematosus, Systemic/epidemiology , Lupus Erythematosus, Systemic/pathology , Male , Middle Aged , Multiple Organ Failure/complications , Multiple Organ Failure/epidemiology , Nail Diseases/pathology , Nails/pathology , Osteoarthritis/epidemiology , Osteoarthritis/pathology , Raynaud Disease/complications , Severity of Illness Index
15.
Lupus ; 30(5): 828-832, 2021 Apr.
Article in English | MEDLINE | ID: mdl-33736541

ABSTRACT

BACKGROUND: Antiphospholipid antibodies (aPL) have been extensively reported in children, but investigations into thrombotic risks associated with aPL positivity in pediatric patients is scarce. Positive aPL are not uncommon in pediatric connective tissue diseases (CTD), but identification and management of these patients is challenging due to lack of validated criteria and a paucity of data. In this study, we identify potential additional risk factors for thrombosis in a unique cohort of pediatric aPL positive carriers. METHODS: Retrospective chart review was performed on 491 pediatric patients with CTD seen in our institution from 2001 to 2019. Patients without persistently moderate to high titer aPL at least 12 weeks apart were excluded. Univariate analysis was performed to evaluate correlation between different risk factors and thrombotic events. RESULTS: Seventy-one aPL positive children with underlying CTD are included in this cohort. The majority (87%) are female and of Hispanic ethnicity (56%). Mean age of the cohort at the diagnosis of connective tissue disease is 12.7 (SD 2.6) years, and mean age of first positive aPL is 13.3 (SD 2.5) years. Average length of follow-up is 4.3 (SD 2.5) years. Four (5.6%) patients experienced arterial thrombosis, and 11 (15.5%) had venous thrombosis. Fifty-seven (80.3%) patients did not have any thromboembolic events. Among traditional risk factors and signs of endothelial injury, only Raynaud's phenomena demonstrated significant association with arterial thrombosis (OR = 8.4, 95%CI 1.13-111, P = 0.039), and hypertension or anti-hypertensive use demonstrated significant association with venous thrombosis (OR = 8.387, 95%CI 1.2 - 94, P = 0.02). CONCLUSION: Data from our cohort suggest that Raynaud's phenomenon is a potential predictor of arterial thrombosis while the presence of hypertension or anti-hypertensive medication use is a potential predictor of venous thrombosis in aPL positive pediatric carriers. Further studies investigating pediatric aPL profiles and risk factors for development of thrombosis are needed to help guide clinicians in caring for these challenging patients.


Subject(s)
Antibodies, Antiphospholipid/blood , Connective Tissue Diseases/immunology , Thrombosis/immunology , Venous Thrombosis/immunology , Adolescent , Antihypertensive Agents/adverse effects , Antihypertensive Agents/therapeutic use , Antiphospholipid Syndrome/complications , Arteries/pathology , Carrier State/blood , Child , Connective Tissue Diseases/complications , Connective Tissue Diseases/diagnosis , Connective Tissue Diseases/pathology , Female , Follow-Up Studies , Humans , Hypertension/complications , Hypertension/diagnosis , Hypertension/drug therapy , Hypertension/epidemiology , Lupus Coagulation Inhibitor/blood , Lupus Erythematosus, Systemic/complications , Male , Raynaud Disease/complications , Raynaud Disease/diagnosis , Raynaud Disease/epidemiology , Retrospective Studies , Risk Factors , Thrombosis/diagnosis , Thrombosis/epidemiology , Venous Thrombosis/epidemiology
16.
Rheumatol Int ; 41(5): 943-949, 2021 May.
Article in English | MEDLINE | ID: mdl-32447423

ABSTRACT

Raynaud's phenomenon is a vasospastic condition affecting hands and feet which may lead to rest pain, ischemic ulcers and gangrene. Botulinum toxin A has been shown to improve peripheral circulation and relieve vasospastic symptoms. Our aim was to assess our treatment outcomes following Botulinum toxin A injections in patients with Raynaud's phenomenon and to explore the importance of toxin concentration and injection sites. Retrospective chart review of patients with primary and secondary Raynaud's syndrome treated with Botulinum toxin A injections and a literature review was conducted. The toxin dose, injection sites, symptom relief, healing of ulcers and complications were assessed. A total of 30 treatment episodes over a 7½ year period were included. All patients had failed medical management. Botulinum toxin A injection was injected primarily in the vicinity of the palmar digital neurovascular bundle. The average total Botulinum toxin A dose injected was 156 U and the concentration was 50 U/ml. All patients reported an improvement in symptoms and healing of digital ulcers. One patient reported a temporary muscle weakness. Six patients had a single treatment episode with long term benefit. Systemic sclerosis patients had an average of 6-month interval between treatment episodes. Higher doses of Botulinum toxin A has been well tolerated with no long term adverse effects. Our study shows that targeted low volume higher concentration Botulinum toxin A injections are effective in treating Raynaud's phenomenon.


Subject(s)
Botulinum Toxins, Type A/administration & dosage , Raynaud Disease/drug therapy , Adult , Aged , Botulinum Toxins, Type A/pharmacology , Female , Humans , Injections/methods , Male , Middle Aged , Raynaud Disease/complications , Retrospective Studies , Scleroderma, Systemic/complications
17.
Dermatol Surg ; 47(5): 675-677, 2021 05 01.
Article in English | MEDLINE | ID: mdl-33625134

ABSTRACT

BACKGROUND: Cryolipolysis is an effective means of noninvasive fat reduction. There are numerous relative contraindications to cryolipolysis including scars, hernias, and cold-related disorders such as Raynaud disease, because cryolipolysis has a theoretical risk of exacerbating these conditions. OBJECTIVE: To examine predictors of negative outcomes of cryolipolysis, especially as it pertains to safety of cryolipolysis in patients with Raynaud disease. MATERIALS AND METHODS: A retrospective review of patients who received consultation for cryolipolysis was conducted and analyzed. RESULTS: Patients with Raynaud disease did not experience any exacerbations of their underlying condition after cryolipolysis. Side effects in all patients, regardless of medical history, were mild, temporary, and not associated with any predisposing factors. CONCLUSION: Cryolipolysis seems to be safe in patients with mild-moderate Raynaud disease.


Subject(s)
Cosmetic Techniques/adverse effects , Cryotherapy/adverse effects , Raynaud Disease/complications , Adipose Tissue , Adolescent , Adult , Aged , Aged, 80 and over , Contraindications, Procedure , Disease Progression , Female , Humans , Male , Middle Aged , Retrospective Studies , Treatment Failure , Young Adult
18.
Georgian Med News ; (309): 59-64, 2020 Dec.
Article in English | MEDLINE | ID: mdl-33526731

ABSTRACT

The most common comorbidities in patients with rheumatic diseases include cardiovascular diseases (CVD), liver and biliary tract infection, lung diseases, amyloidosis, fractures of different localizations, malignant neoplasms, metabolic disorders and diabetes mellitus (DM). The aim of study was to investigate the clinical course of DM and rheumatoid arthritis (RS) in patients with RA using laboratory and instrumental research methods. There were examined 85 patients with RA who were treated in the rheumatology department of Ivano-Frankivsk Central City Clinical Hospital. The patients' age ranged from 40 to 70 years. Endothelial dysfunction (ED) signs were observed in 76 (89.4%) patients. ED was diagnosed in all patients with RA, co-existent RS and DM. In the patients with RA and those with RA and co-existent RS, impaired EDVBA was detected. In the patients of Group II, the indicator of EDVBA (6.5±0.2%) was significantly lower as compared to the patients of Group I (8.8±0.3%) (р<0.05). The levels of both CRP and TNF-α, serving as non-specific inflammatory markers, were significantly higher (29.37±3.56 mg/l, p<0.01) in the patients with RS as compared to the patients with RA only (23.89±1.77 mg/l). A detailed study of the pathophysiological and immunological features of the clinical course of secondary RS will allow us to optimize its treatment schemes in patients with RA, reduce clinical and laboratory manifestation of RA and improve quality of life in such patients, especially those with a comorbidity.


Subject(s)
Arthritis, Rheumatoid , Cardiovascular Diseases , Diabetes Mellitus , Raynaud Disease , Adult , Aged , Arthritis, Rheumatoid/complications , Arthritis, Rheumatoid/epidemiology , Comorbidity , Diabetes Mellitus/epidemiology , Humans , Middle Aged , Quality of Life , Raynaud Disease/complications
19.
Lupus ; 28(4): 560-564, 2019 Apr.
Article in English | MEDLINE | ID: mdl-30791856
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