Egyptian Rheumatologist最新文献

{"title":"HLA B27-positive ankylosing spondylitis professional soccer player with a successful return to sports","authors":"Eduard Bezuglov ,&nbsp;Maria Shoshorina ,&nbsp;Mikhail Butovskiy ,&nbsp;Yuri Kuklin ,&nbsp;Kamila Kubacheva ,&nbsp;Vladimir Khaitin ,&nbsp;Ryland Morgans","doi":"10.1016/j.ejr.2023.06.001","DOIUrl":"https://doi.org/10.1016/j.ejr.2023.06.001","url":null,"abstract":"<div><h3>Background</h3><p>Ankylosing spondylitis (AS) is a disease of young adulthood and without adequate treatment it dramatically reduces physical activity.</p></div><div><h3>Aim of the work</h3><p>To present a case with AS that successfully returned to professionally playing sports after treatment.</p></div><div><h3>Case presentation</h3><p>A 19-year-old Eastern European Russian professional male soccer player with AS complained of right ankle joint pain not related to any trauma or injury and gradually intensified. The player's regular medical tests during the preceding years and were unremarkable. He would occasionally complain of discomfort in the ankle joints, sacroiliac area and symphysis but did not limit his regular training activity. He received the standard treatment of sports injuries that included: 14 days of limited physical activity, ibuprofen 200 mg twice/day, cryotherapy and joint taping. Examination revealed mobile joints, regular in shape without any swelling. Erythrocyte sedimentation rate (ESR) was 60 mm/1st hr and C-reactive protein (CRP) 50 mg/dl. AS was diagnosed as he fulfilled the classification criteria. HLA-B27 was positive, left sacroiliitis was present on magnetic resonance imaging (MRI). The Bath AS disease activity index (BASDAI) was 5.9. Treatment also included golimumab 50 mg/month. He improved, returned to regular training and continued playing sports professionally amid supportive therapy. The last BASDAI was 1.5.</p></div><div><h3>Conclusions</h3><p>AS patients with initially high levels of physical activity may restore professionally playing of sports when a proper management plan is provided. Further monitoring is warranted and the creation of a database for athletes with rheumatic diseases is recommended in order to standardize treatment protocols.</p></div>","PeriodicalId":46152,"journal":{"name":"Egyptian Rheumatologist","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49790450","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clinical characteristics and comorbidities in psoriatic arthritis: Experience from a single rheumatology centre in Malaysia","authors":"Wahinuddin Sulaiman ,&nbsp;Lay K. Tan ,&nbsp;Hasnah Mat ,&nbsp;Najjah Tohar ,&nbsp;Abdul Muhaimin Fathi ,&nbsp;Nia Maslia A. Kosenin ,&nbsp;Muhammad N. Naim ,&nbsp;Rafiqah F. Redzuan ,&nbsp;Nur Iffah Ab Rani ,&nbsp;Najiha A. Norhisham ,&nbsp;Ong P. Seung","doi":"10.1016/j.ejr.2023.08.002","DOIUrl":"https://doi.org/10.1016/j.ejr.2023.08.002","url":null,"abstract":"<div><h3>Aim of the work</h3><p>To evaluate the clinical features and associated comorbidities of psoriatic arthritis (PsA) patients from a single rheumatology centre in Malaysia.</p></div><div><h3>Patients and methods</h3><p>247 PsA patients in rheumatology clinic, Raja Permaisuri Bainun Hospital were included. Clinical and laboratory data were retrieved from the medical record.</p></div><div><h3>Results</h3><p>The mean age was 56 ± 13.5 years, 56.1% were female, and 38.9% were Indians. The onset of psoriasis and PsA was significantly earlier among Malays (P &lt; 0.01) whilst Indians had longer disease duration (P &lt; 0.05). The mean duration of progression from psoriasis to PsA was 8.7 years. Alcohol and nail dystrophy were common among Indians (P &lt; 0.05). Plaque psoriasis was the commonest subtype (81%) in nails (pitting, 42.5%; onycholysis, 20.7%), scalp (35.6%) and limbs (32.8%). Pustular psoriasis and onycholysis were common in males (P = 0.05 and 0.002, respectively) whilst scalp psoriasis in females (P &lt; 0.05). Peripheral arthritis was the highest (92.7%) compared to axial (8.1%); 14.6% had both. 38.9% were oligoarthritis and mostly asymmetrical (35.6%). 24% had enthesitis, 14.6% dactylitis, and 0.8% uveitis. 52.2% had hypertension, followed by dyslipidemia (44.1%), diabetes mellitus (34%), obesity (30%), ischemic heart disease (9.7%), cancer (2.4%), and tuberculosis (0.4%). No significant relationship between the pattern of arthritis and these comorbidities (P &gt; 0.05).</p></div><div><h3>Conclusion</h3><p>PsA was more prevalent in Indians. Malays have younger disease onset. Pustular psoriasis and onycholysis were common in males, while scalp psoriasis in females. Asymmetrical oligoarthritis and plaque psoriasis were the commonest pattern and psoriasis subtype respectively. Comorbidities were not associated with the pattern of arthritis in PsA patients.</p></div>","PeriodicalId":46152,"journal":{"name":"Egyptian Rheumatologist","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49790390","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Tumor necrosis factor-α, its related immunoregulatory long non-coding RNA (THRIL) and micro-RNA 145 as potential biomarkers in lupus nephritis patients","authors":"Salsabeel M. Kahwa ,&nbsp;Olfat G. Shaker ,&nbsp;Basma M. Eissa ,&nbsp;Amany M. Wahb","doi":"10.1016/j.ejr.2023.08.004","DOIUrl":"https://doi.org/10.1016/j.ejr.2023.08.004","url":null,"abstract":"<div><h3>Aim of the work</h3><p>To explore the differential expression of tumor necrosis factor-α (TNF-α), its related immunoregulatory long non-coding RNA (THRIL), and microRNA145 (MIR145) in systemic lupus erythematosus (SLE) patients and their diagnostic utility in lupus nephritis (LN).</p></div><div><h3>Patients and methods</h3><p>The study included 60 SLE patients; 30 with LN, 30 without LN (NN), and 30 matched controls. SLE disease activity index was assessed. Serum TNF-α level was determined by enzyme linked immunosorbent assay. Serum fold change (FC) in expression of THRIL and MIR145 were assayed by real time polymerase chain reaction.</p></div><div><h3>Results</h3><p>The patients mean age was 32.6 ± 6.8 years and were 52 females and 8 males (F:M 6.5:1). Serum TNF-α level was significantly higher in SLE patients (108.6 ± 47.8 pg/ml) compared to control (39.6 ± 3.7 pg/ml) (p &lt; 0.001). THRIL expression was upregulated (8.3 ± 6.9<!--> <!-->vs<em>.</em> <!-->1.02 ± 0.06 FC, p &lt; 0.001) and MIR145 downregulated (0.39 ± 0.36<!--> <!-->vs<em>.</em> <!-->0.92 ± 0.94 FC, p &lt; 0.001) in SLE patients versus controls. THRIL correlated with disease activity (r = 0.27, p = 0.035) and MIR145 with C3 (r = −0.32, p = 0.04) and C4 (r = −0.36, p = 0.016) levels in SLE patients. In LN patients, TNF-α and THRIL were increased while MIR145 downregulated (p &lt; 0.001 each) and proteinuria significantly correlated with TNF-α (r = −0.4,p = 0.028), THRIL (r = 0.48, p = 0.007) and MIR145 (-0.42, p = 0.02). In NN patients, TNF-α and THRIL (p &lt; 0.001 both) were increased while MIR145 downregulated (p = 0.023). TNF-α (cutoff ≥ 122.5 pg/ml, AUC 0.67, p = 0.003), and MIR145 (cutoff ≤ 0.22 FC, AUC 0.71, p = 0.026) discriminated LN from NN. The combination of TNF-α and MIR145 discriminated better than either alone (AUC 0.75, p = 0.002).</p></div><div><h3>Conclusion</h3><p>TNF-α and MIR145 are potential biomarkers of LN in SLE.</p></div>","PeriodicalId":46152,"journal":{"name":"Egyptian Rheumatologist","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49790387","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Serum level of galectin-9 in systemic lupus erythematosus patients with lupus nephritis: Relation to clinical characteristics and disease activity","authors":"Mariam G. Abdelmonem ,&nbsp;Manal A. Mohammed ,&nbsp;Hosam Abdel Twab ,&nbsp;Eman B. Elmarghany","doi":"10.1016/j.ejr.2023.08.001","DOIUrl":"https://doi.org/10.1016/j.ejr.2023.08.001","url":null,"abstract":"<div><h3>Aim of the work</h3><p>To assess galectin-9 (Gal-9) level in the serum of systemic lupus erythematosus (SLE) patients with and without renal involvement and clarify its relation with disease activity.</p></div><div><h3>Patients and methods</h3><p>50 SLE patients; 25 with lupus nephritis (LN) and 25 without as well as 25 controls were studied. Systemic Lupus International Collaborating Clinics (SLICC) renal activity score and SLE disease activity index 2000 (SLEDAI-2 K) were determined. Serum Gal-9 was measured in all participants.</p></div><div><h3>Results</h3><p>Gal-9 level was significantly elevated in SLE patients with (16.7; 11.6–33.7 ng/ml) and without (15.9; 11.8–25 ng/ml) compared to controls (3.9; 2.8–5.4 ng/ml) (p &lt; 0.001) but was comparable between the patients groups (p = 0.83). In LN patients, serum Gal-9 and SLICC renal activity score significantly correlated (r = 0.48, p = 0.016). Serum Gal-9 significantly correlated with SLEDAI-2 K in patients with (r = 0.71, p &lt; 0.001) and without (r = 0.95, p &lt; 0.001) LN, with anti-double stranded deoxyribonucleic acid (anti-ds-DNA) titers (with r = 0.57, p &lt; 0.001 and without r = 0.79, p &lt; 0.001) and inversely with C3 (with r = -0.44, p = 0.027 and without r = -0.63, p &lt; 0.001) and C4 (with r = -0.47, p = 0.018 and without r = -0.43, p = 0.03). Gal-9 had an area under the curve (AUC) of 0.96 to distinguish SLE cases from control. However, AUC between LN group and non-nephritic SLE was 0.48. On regression, SLEDAI-2 K was the only significant factor associated with serum Gal-9 (p &lt; 0.001).</p></div><div><h3>Conclusion</h3><p>In SLE patients, significantly raised Gal-9 levels and relation with disease activity were detected indicating its clinical relevance as biomarker of disease activity and its potential value in the disease diagnosis. Its value in discriminating LN from non-nephritic SLE is limited.</p></div>","PeriodicalId":46152,"journal":{"name":"Egyptian Rheumatologist","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49790388","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Hospitalization and outcome of systemic lupus erythematosus patients admitted to the Rheumatology ward of Kasr Al-Ainy University Hospital","authors":"Hanan A. Kotb,&nbsp;Shaimaa Khalad,&nbsp;Abdelkawy Moghazy","doi":"10.1016/j.ejr.2023.03.007","DOIUrl":"https://doi.org/10.1016/j.ejr.2023.03.007","url":null,"abstract":"<div><h3>Aim of the work</h3><p>To determine the frequency, causes and disease features affecting outcome of hospitalized systemic lupus erythematosus (SLE) patients, length of hospital stay and the risk factors.</p></div><div><h3>Patients and methods</h3><p>132 SLE patients accounting for 167 hospitalizations were recruited from the Rheumatology outpatient clinic, Faculty of Medicine, Cairo University Hospitals during 2019. SLE disease activity index (SLEDAI) and systemic<!--> <!-->lupus international collaborating clinics damage index (SLICC-DI) were assessed. Data concerning admission was recorded.</p></div><div><h3>Results</h3><p>The mean age of the patients was 31.4 ± 10.7 years and disease duration of 91.5 ± 84.3 months. Their mean SLEDAI was 8.7 ± 7.2 and SLICC-DI was 0.87 ± 1.2. The frequency of hospitalization was 167/1456 follow up visits (11.5%): 127 single admissions and 40 recurrent admissions. 137 hospital admissions were females and 30 males (F:M 4.6:1).The most common causes of hospitalization were disease activity in 115/167 hospital admissions (68.9%), infection in 39/167 (23.4%), comorbidities in 33/167 (19.8%). Outcome of hospitalization was improvement in 154/167 (92.2%), morbidity in 7/167 (4.2%), and mortality in 5/167 (3%). Readmission occurred in 40/167 (24%). Higher SLEDAI and gastrointestinal involvement were risk factors for longer hospital stay (p = 0.001 and p = 0.003 respectively). Morbidity was related to higher SLEDAI and SLICC-DI while mortality was related to prolonged azathioprine intake and cardiovascular involvement.</p></div><div><h3>Conclusion</h3><p>Disease flare and infection were the most common causes of hospitalization among SLE patients. Higher disease activity and damage as well as cardiovascular involvement were the factors associated with worse outcome. Higher SLEDAI and gastrointestinal involvement were risk factors for longer hospital stay.</p></div>","PeriodicalId":46152,"journal":{"name":"Egyptian Rheumatologist","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49819192","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Management of knee osteoarthritis using percutaneous high tibial osteotomy for correction of genu varum deformity in adolescents and young adults","authors":"Ahmed M. Kaddah ,&nbsp;Wesam G. Alanani ,&nbsp;Mohamed M. Hegazi ,&nbsp;Mohamed T. AbdAlFattah","doi":"10.1016/j.ejr.2023.04.003","DOIUrl":"https://doi.org/10.1016/j.ejr.2023.04.003","url":null,"abstract":"<div><h3>Background</h3><p>Genu varum is not uncommon among young adults and adolescents and may increase the risk of knee osteoarthritis (OA).</p></div><div><h3>Aim of the work</h3><p>To analyse the ability to correct and achieve osseous union of genu varum with high tibial osteotomy (HTO) using a dynamic axial fixator (DAF) and to evaluate knee function and pain.</p></div><div><h3>Patients and methods</h3><p>Fifteen knees in 13 patients with genu varus and medial compartment knee pain underwent open wedge HTO using DAF. Clinical evaluation was done using Modified Lysholm score and new Knee Society score (KSS). Long-standing X-rays were performed.</p></div><div><h3>Results</h3><p>The mean age of the patients was 21.9 ± 2.57 years (18–28 years) and they were 8 males and 5 females. The mean medial proximal tibial angle (MPTA) improved from 78.9 ± 4.35 (69–84) pre-operative to 91.7 ± 2.37 (88–96) post-operative. The mean mechanical tibiofemoral angle (mTFA) improved from 168.5 ± 4.06 (160–176) pre-operative to 180.4 ± 3.11 (176–186) post-operative. The target correction was achieved in 12 knees (80%) and under-correction occurred in three (20%). Significant post-operative improvements in pain and function of daily life were seen. All osteotomies healed before 4 months. The mean Modified Lyshlom and KSS scores improved from 66.4 ± 24.02 and 135.1 ± 43.95 preoperatively to 95.5 ± 9.51 and 236.9 ± 11.38 respectively (p &lt; 0.0001 both). 6/15 (40%) knees showed complications in the form of superficial pin tract infection that all resolved without any sequela.</p></div><div><h3>Conclusion</h3><p>For the treatment of proximal tibia vara, HTO with DAF was safe and effective, allowing for correct post-operative alignment modification with high union rates and trivial sequelae.</p></div>","PeriodicalId":46152,"journal":{"name":"Egyptian Rheumatologist","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49835221","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Comparative assessment of hepatitis C virus-related arthritis and rheumatoid arthritis: Relation to clinical, serologic and ultrasonographic characteristics","authors":"Hala L. Fayed ,&nbsp;Amira A. Shahin ,&nbsp;Abo El-Magd M. El-Bohy ,&nbsp;Sahar S. Younis","doi":"10.1016/j.ejr.2023.03.006","DOIUrl":"https://doi.org/10.1016/j.ejr.2023.03.006","url":null,"abstract":"<div><h3>Background</h3><p>Extrahepatic manifestations of hepatitis C virus (HCV) infection includes HCV-related arthritis (HCV-A) that may mimic rheumatoid arthritis (RA). Musculoskeletal ultrasound (MSUS) can aid in discriminating both conditions.</p></div><div><h3>Aim of the work</h3><p>To study the clinical, serological and imaging (Xray, MSUS) characteristics of HCV-A and compare them to RA.</p></div><div><h3>Patients and methods</h3><p>The study included 30 patients with HCV-A and 30 age- and sex-matched RA patients negative for HCV. Ritchie articular index (RAI), tender joint count (TJC) and swollen joint count (SJC) assessed arthritis. Patient global health assessment (PGHA) and modified health assessment questionnaire (MHAQ) were evaluated. The erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), rheumatoid factor (RF), anti-cyclic citrullinated peptide (anti-CCP) and serum cryoglobulins were measured. Radiologic assessment included short Larsen score (SLS) and MSUS using 7-joint ultrasound score (US7).</p></div><div><h3>Results</h3><p>The mean age of the HCV-A patients was 45.8 ± 4.7 years; RA was 43.3 ± 5.6 years; F:M was 27:3 in HCV-A patients; 29:1 in RA; HCV-A patients had no subcutaneous nodules, joint deformities or bone erosions. US7 showed that Gray-Scale (GS)/Power Doppler (PD) synovitis and tenosynovitis had higher mean values in RA versus HCV-A patients (p &lt; 0.001; p &lt; 0.001; p = 0.011; p = 0.008 respectively). A significant correlation was found between SJC with GS and PD synovitis in both groups.</p></div><div><h3>Conclusion</h3><p>HCV-A differ from RA features as being non nodular, non-deforming, non-erosive and aid in the predilection of HCV-A diagnosis. MSUS can offer a useful imaging modality elucidating inflammatory components of HCV-A and highlighting the spectrum of the condition.</p></div>","PeriodicalId":46152,"journal":{"name":"Egyptian Rheumatologist","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49819191","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Pulmonary artery pseudoaneurysms (PAPs) in Hughes-Stovin syndrome (HSS) as an emerging concept for a potentially fatal course","authors":"Tubig C. Joy ,&nbsp;Juljani Sherwina ,&nbsp;Ayuyao Fernando ,&nbsp;Ma E Limpin ,&nbsp;Ma Paz Mateo ,&nbsp;Khalid Alhusseiny","doi":"10.1016/j.ejr.2023.03.004","DOIUrl":"https://doi.org/10.1016/j.ejr.2023.03.004","url":null,"abstract":"<div><h3>Introduction</h3><p>Hughes-Stovin syndrome (HSS) is a systemic vasculitis characterized by pulmonary artery aneurysms (PAA) and widespread venous and arterial thrombosis. Fatalities in HSS resulted from unforeseeable fatal suffocating hemoptysis. It is critical to early detect pulmonary involvement to take timely measures against inevitable serious life-threatening complications.</p></div><div><h3>Case presentation</h3><p>A 27 year old Filipino male patient presented to the emergency department with an acute attack of massive hemoptysis. The markers of inflammation were elevated with anemia and a normal coagulation profile. The patient had bilateral lower limb edema with tender calf muscles. Color Doppler ultrasound revealed bilateral deep vein thrombosis. Importantly, an urgent computerized tomography pulmonary angiography (CTPA) revealed bilateral large PAAs which matched the pattern of pseudoaneurysms described by the HSS international study group (HSSISG). There was no history of iridocyclitis, recurrent oral or genital ulcers. Accordingly, the patient was diagnosed with HSS. The patient received intravenous pulse methylprednisolone (1 g/3 days), then oral prednisone (1 mg/kg/d/3 months), and monthly pulse cyclophosphamide (1 g) for three months. The patient remained symptom-free, yet on the third pulse of cyclophosphamide; he patient contracted corona virus disease-2019 (COVID-19) infection and died three weeks later from a fatal episode of massive hemoptysis. The case was discussed and the recent literature was reviewed relative to fatal hemoptysis associated with pulmonary artery pseudoaneurysms (PAPs) pattern in HSS</p></div><div><h3>Conclusion</h3><p>HSS presenting with massive hemoptysis could be lifethreatening and the PAPs are considered a double edged sword. Detailed description of such rare cases is warranted for optimum future management.</p></div>","PeriodicalId":46152,"journal":{"name":"Egyptian Rheumatologist","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49792926","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Juvenile clinically amyopathic dermatomyositis (CADM): Case-based review","authors":"Wahinuddin Sulaiman ,&nbsp;Farisya Mohd Lepatoni ,&nbsp;Jyi Jong Tang ,&nbsp;Nurul Bahiyah Baharudin","doi":"10.1016/j.ejr.2023.03.005","DOIUrl":"https://doi.org/10.1016/j.ejr.2023.03.005","url":null,"abstract":"<div><h3>Background</h3><p>Clinically amyopathic dermatomyositis (CADM) is rare as the classical cutaneous features are present with absence of myopathy and the serum creatinine kinase, electromyographic examination and muscle biopsy are usually normal.</p></div><div><h3>Aim of the work</h3><p>To report a young girl with juvenile CADM who responded to corticosteroid and mycophenolate mofetil, without progressing to myopathy and other systemic complications over a 3-year period.</p></div><div><h3>Case presentation</h3><p>An 11-year-old Malay girl was afebrile and presented with debilitating polyarthritis and classical cutaneous manifestations of dermatomyositis which was confirmed by histological findings, but without myopathy and systemic involvement. The anti-nuclear antibody (ANA) was positive 1:160, with speckled pattern and anti-p155/140 antibodies (anti-transcriptional intermediary factor 1, anti-TIF1γ antibody) were positive. Skin biopsy revealed typical histological findings consistent with dermatomyositis including vacuolar changes of the basal layer, increased lymphocytic infiltrate and increased mucin deposition in the dermis. She received pulse methylprednisolone (250 mg/day/3 days) followed by oral prednisolone 1 mg/kg/day tapered until her cutaneous lesions showed marked improvement. Hydroxychloroquine (HCQ) (200 mg/day) and oral methotrexate (10 mg/week) were added. Six months after initial treatment, mycophenolate mofetil (MMF) 1 g/day was added in view of the slow improvement of her cutaneous lesions and was discontinued a year later as she demonstrated favourable outcome. Besides the skin lesions, her arthritis also responded well to treatment.</p></div><div><h3>Conclusion</h3><p>Juvenile CADM is rare but with early recognition and treatment, the prognosis is good especially in children as this may reduce the risk of systemic complications and progression to myopathy.</p></div>","PeriodicalId":46152,"journal":{"name":"Egyptian Rheumatologist","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49792927","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"High frequency ultrasound assessment of skin in systemic sclerosis patients","authors":"Mervat Abo Gabal,&nbsp;Mohamed R. Mohamed,&nbsp;Mohamed A. Moawed,&nbsp;Caroline S. Morad","doi":"10.1016/j.ejr.2023.04.005","DOIUrl":"https://doi.org/10.1016/j.ejr.2023.04.005","url":null,"abstract":"<div><h3>Aim of the work</h3><p>To evaluate the role of high frequency ultrasound (HFU) in assessing skin changes in terms of thickness and echogenicity in systemic sclerosis (SSc) patients with early and late changes.</p></div><div><h3>Patients and methods</h3><p>Twenty-three SSc patients were enrolled along with 21 matched controls. Skin thickness was assessed using modified Rodnan skin score (mRSS) and HFU.</p></div><div><h3>Results</h3><p>Patients had mean age of 41.8 ± 9.1 years, 91.3% were females, mean disease duration 6.0 ± 4.6 years, 11 patients had early (&lt;5 years) and 12 late (≥5 years) disease, 8 patients had limited (lcSSc) and 15 diffuse (dcSSc) cutaneous SSc. Antinuclear antibody was positive in 17 (73.9%) and antiscleroderma-17 in 18 (78.3%). Patients had significantly thicker skin between second and third metacarpophalangeal joint (L = 1.52 ± 0.35 mm vs 1.25 ± 0.35 mm; p = 0.017 and T = 1.48 ± 0.34 mm vs 1.26 ± 0.33 mm; p = 0.038 respectively). Patients with early disease had thicker skin than those with late disease. HFU dermal thickness showed no significant difference according to gender, subtypes, presence and absence of clinical manifestations or autoantibody positivity. There was significant higher dermal thickness in patients with reflux (p = 0.009) and was lower in patients with interstitial pulmonary fibrosis (p &lt; 0.05). There was negative correlation between US dermal thickness and disease duration (p &lt; 0.05). mRSS showed no correlation with HFU dermal thickness in all areas.</p></div><div><h3>Conclusion</h3><p>HFU is useful in assessing skin pathologic changes (even subclinical changes) in terms of thickness and echogenicity in SSc patients. Moreover, it could be a potential screening tool in differentiating normal from pathologic skin thickness.</p></div>","PeriodicalId":46152,"journal":{"name":"Egyptian Rheumatologist","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49819193","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}