Giant cell arteritis: insights from a monocentric retrospective cohort study.

Giant cell arteritis (GCA), more common in Northern European populations, has limited data in Arabcountries. Our study reports GCA's clinical manifestations in Jordan and reviews published research on GCA across Arab nations. In this retrospective analysis, GCA patients diagnosed from January 2007 to March 2019 at a Jordanian academic medical center were included through referrals for temporal artery biopsy (TAB). A comprehensive search in PubMed, Scopus, and the DOAJ (Directory of Open Access Journals) databases was conducted to identify all relevant English-language manuscripts from Arab countries on GCA without time limitations. Among 59 diagnosed GCA patients, 41 (69.5%) were clinically diagnosed with a negative TAB, and 19 (30.5%) had a positive result. Females comprised 74.6% (n = 44) with 1:3 male-female ratio. The mean age at diagnosis was 67.3 (± 9.5) years, with most presenting within two weeks (n = 40, 67.8%). Headache was reported by 54 patients (91.5%). Elevated ESR occurred in 51 patients (78%), with a mean of 81 ± 32.2 mm/hr. All received glucocorticoids for 13.1 ± 10 months. Azathioprine, Methotrexate, and Tocilizumab usage was 15.3% (n = 9), 8.5% (n = 5), and 3.4% (n = 2), respectively. Remission was observed in 57.6% (n=34), and 40.7% (n = 24) had a chronic clinical course on treatment. Males had higher biopsy-based diagnoses (p = .008), and biopsy-diagnosed patients were older (p = .043). The literature search yielded only 20 manuscripts originating in the Arab world. The predominant study types included case reports and retrospective analyses, with only one case series and onecase-control study.

Is the patient-perceived impact of psoriatic arthritis a global concept? An international study in 13 Arab countries (TACTIC study).

The Psoriatic Arthritis Impact of Disease (PsAID-12) questionnaire, a recommended measure of patient-reported impact for psoriatic arthritis (PsA), was initially developed in Europe and may lack universal validity. Recognizing the need for a culturally appropriate tool for Arab patients, this study aimed to TranslAte, CulTurally adapt, and validate the PsAID in ArabIC (TACTIC). The PsAID-12 was translated into Arabic using a rigorous process of double translation, back-translation, and cognitive debriefing. The Arabic version was then validated through a study conducted in 13 Arab countries in 2022. Participants were consecutive literate adult patients diagnosed with PsA and fulfilling the CASPAR criteria. Collected data included PsAID-12, disease activity, and legacy patient-reported outcomes. Psychometric properties, such as internal consistency, construct validity, and test-retest reliability, were examined. Factors associated with high PsAID-12 total scores (> 4) were explored using multivariable binary logistic regression. A culturally adapted Arabic PsAID-12 questionnaire was achieved with minor rephrasing. The validation study included 554 patients from 13 countries (mean age 45 years, 59% females), with a mean PsAID score of 3.86 (SD 2.33). The Arabic PsAID-12 demonstrated excellent internal consistency (Cronbach's α = 0.95), and correlations with other measures ranged from 0.63 to 0.78. Test-retest reliability (N = 138 patients) was substantial (intraclass correlation coefficient, ICC 0.90 [0.86-0.93]; Cohen's kappa 0.80). Factors associated with a high PsAID score were disability (odds ratio, OR 3.15 [2.03-4.89]), depression (OR 1.56 [1.35-1.81]), widespread pain (OR 1.31 [1.12-1.53]), and disease activity (OR 1.29 [1.13-1.47]). Pain and fatigue were identified as the most impactful PsAID-12 domains for PsA patients. The Arabic PsAID is a valid and reliable measure that reflects the priorities of patients with PsA. PsAID scores correlated with disease activity and legacy outcome measures, as expected, indicating PsAID is a consistent measure of PsA impact across cultures. These findings highlight the potential of the Arabic PsAID in improving the care provided to Arabic-speaking patients worldwide.

Pancytopenia with aplastic anemia in systemic lupus erythematosus: case series and literature review.

Aplastic anemia (AA) is a rare, potentially catastrophic hematopoiesis failure manifested by pancytopenia and bone marrow aplasia. AA occurrence in Systemic Lupus Erythematosus (SLE) patients is extremely rare. The diagnosis may be delayed due to other possible pancytopenia etiologies. Confirmation of peripheral cytopenias diagnosis necessitates a bone marrow aspiration. The management of AA is challenging, and the literature reported using glucocorticoids, danazol, plasmapheresis, cyclophosphamide, intravenous immunoglobulin, and cyclosporine. We report two cases of SLE patients who presented with pancytopenia, with bone marrow biopsy confirmed AA. One case was treated with cyclophosphamide but unfortunately succumbed to Acute Respiratory Distress Syndrome (ARDS), while the other case was managed with rituximab with a good response. Interestingly, both patients were on azathioprine before the diagnosis of AA. A comprehensive search for reported cases of AA in PubMed, Scopus, and the Directory of Open Access Journals databases was performed to enhance the understanding of the diagnostic and management challenges associated with AA in SLE, facilitating ongoing exploration and research in this field. The decision to do a BM aspiration and biopsy is recommended for SLE patients with an abrupt decline in blood counts and previously stable blood counts.

Pseudoxanthoma elasticum veiled as vasculitis: shedding light on an uncommon disorder and an in-depth review of the literature.

Pseudoxanthoma Elasticum (PXE) is a rare genetic disorder caused by an autosomal recessive mutation in the ABCC6 gene. It manifests with distinctive clinical symptoms impacting the skin, eyes, and cardiovascular system, along with an elevated risk of cardiovascular diseases. We present a case of a 34-year-old male patient who was initially referred to the rheumatology clinic for evaluation due to suspected large vessel vasculitis. The patient's primary complaint was severe hemifacial pain radiating to the neck and upper limb. Radiological imaging studies unveiled substantial vascular narrowing and collateral vessel formation, prompting further investigation to exclude systemic vasculitis. Intriguingly, the patient also exhibited cutaneous manifestations, which were later confirmed via skin biopsy as consistent with PXE. An ophthalmological examination further revealed the presence of the classic PXE findings of angioid streaks. Given the rarity of PXE and its multifaceted clinical presentation, it can be particularly challenging to diagnose and manage. As such, cases like the one presented here may necessitate a referral to a rheumatologist for evaluation of potential systemic involvement. To provide a comprehensive perspective on PXE, we conducted a systematic review of case reports published in the past decade in English, collected from PubMed, Scopus, and the Directory of Open Access databases. The analysis of these cases will be discussed to shed light on the diversity of PXE's clinical features and the diagnostic and management dilemmas it poses and to facilitate ongoing exploration and research into this intricate condition, ultimately leading to improved care for individuals affected by PXE.

Idiopathic granulomatous mastitis: clinical, histopathological, and radiological characteristics and management approaches.

Idiopathic Granulomatous Mastitis (IGM) is an infrequent, benign breast disease that primarily affects women during their childbearing years and can be mistaken for breast cancer. This study aimed to review the clinical, radiological, and histopathological findings of patients with IGM in addition to management and outcome. Retrospective cross-sectional study of biopsy-confirmed IGM at an academic medical center and a private hospital in Amman, Jordan. Fifty-four patients were included, with a mean age of 37.0 ± 9.04 years, mostly presenting with a breast lump (n = 52, 96.3%) and breast pain (n = 45 patients, 84.9%). Approximately half of the patients (51.9%) were parous, and 50% had breastfed for an average duration of 30.37 ± 22.38 months. Most of the patients had either solitary or multiple abscesses on breast ultrasound. Histopathological analysis (n = 35) showed mostly either moderate inflammation (n = 16, 45.7%) or severe inflammation (n = 14, 40%). Two-thirds of the patients underwent surgical interventions at the time of diagnosis, mostly incision and drainage (n = 16, 29%) or surgical excision (n = 7, 13%), and no mastectomies were performed. The most common medical treatment included a combination of antibiotics, corticosteroids, and methotrexate (n = 21, 38.8%). After follow-up, 31 patients remained in remission, 3 experienced relapses, and 3 had a chronic course. The use of corticosteroids was significantly associated with remission (p = 0.035). The presentation and demographics of IGM patients in Jordan were consistent with the existing literature. Prospective research is needed to explore different treatment options and disease outcomes.

Clinical and technical determinants of positive temporal artery biopsy: a retrospective cohort study.

Temporal artery biopsy (TAB) is one of the diagnostic tools to confirm the diagnosis of giant cell arteritis (GCA). We aim to evaluate the clinical and technical determinants of a positive biopsy. Demographics, clinical, technical, and laboratory data of all TAB's performed between 2007 and 2019 at a single academic medical center. 107 biopsies performed for 103 patients were included; 72.9% were female, and 27.1% were male. The mean age at the time of biopsy was 67.1 ± 9.3 years. One biopsy was excluded for lack of arterial tissue content. Of the remaining 106, 19.6% were positive. The length of the biopsy and the number of arterial cross-sections were not significantly associated with its result. A positive biopsy was seen more in patients with low albumin (p = 0.010) and hypothyroidism (p = 0.017) but less in those with prior glucocorticoids treatment (p = 0.028). Predictors of a positive biopsy included male gender [OR 4.029, 95% CI (1.330-12.209), p = 0.014]; elevated ESR [OR 3.998, 95% CI (1.908-6.787), p = 0.023]; polymyalgia rheumatica (PMR) symptoms [OR 5.121, 95% CI (2.094-9.872), p = 0.001]; and advancing in age (6.5% per every additional year), [OR 1.065, 95% CI (1.005-1.130), p = 0.033]. 53.7% of the patients were eventually diagnosed with GCA; 39.2% of them were based on positive biopsy. In conclusion, old age, male gender, elevated ESR, and PMR symptoms increase the odds of positive TAB. Technical factors, such as biopsy length and the number of cross-sections, did not influence eventual biopsy results, highlighting the pivotal role of the clinical presentation of the patients in selecting patients for TAB.

Validation of the cultural adaptation of the Kujala score in Arabic.

BACKGROUND: Patellofemoral pain is a common condition. The Kujala score is a well-established scoring system to assess anterior knee pain and has been translated into many languages including Arabic. The purpose of this cross-sectional study is to culturally adapt the Arabic version of the Kujala score and determine its validity. METHODS: The Kujala score is composed of 13 multiple-choice questions. We modified two questions in the score; running and squatting, and were replaced with questions related to walking on different terrain and prostration, each with the same number of answer choices as the original questions so as not to affect the final score. These modifications were written in Arabic by the same group who translated and validated the original score into Arabic. The original and modified Kujala scores was printed and given to patients complaining of patellofemoral pain to be filled during their visit to the orthopedic outpatient clinics. Final scores for the original and modified questionnaires were calculated. Data was analyzed using SPSS statistics version 21.0 measuring Cronbach's alpha, intraclass correlation coefficient, and Pearson correlation. RESULTS: Ninety-four patients were included in the study, 28 (29.8%) men and 66 (70.2%) women. The mean age for the included patients was 43.67 (± 14.46) years. The mean score for the modified initial questionnaire was 63.91 (± 16.32), and the mean score for the modified re-test questionnaire was 66.52 (± 17.50). There was a statistically significant difference between the mean scores (p = 0.041), with a mean difference of 1.97 (95% CI 0.08 to 3.856). We found a significant strong correlation between the score before and after changing the questions with a p value of < 0.001. CONCLUSIONS: The culturally modified Arabic Kujala questionnaire is shown to be a valid, well-designed tool and an appropriate method of measuring patellofemoral pain.

Renal dysfunction among rheumatoid arthritis patients: A retrospective cohort study.

BACKGROUND: Rheumatoid arthritis (RA) is a common rheumatological disease which can involve a variety of different renal manifestations. This may be explained by disease effect itself or by medications used for treatment that may lead to renal dysfunction and its complications.We aimed to identify the prevalence and factors that played a role in renal dysfunction among RA Jordanian patients. METHOD: 285 patients with RA visiting outpatient clinic between March 2016 and March 2017 were included in a retrospective study design. Age, gender, comorbidities, duration of the disease, medications and laboratory results were gathered and scoring of RA activity was done. RESULTS: Data gathered from the 285 patients showed a female predominance with 88.4% female and 11.6% male. The average disease duration was 6.7 years. Age, DM, HTN, and serum CRP were associated with worse renal function on univariate analysis. 44 patients (18.8%) presented with microscopic hematuria, 16 (6.9%) with proteinuria and only 5 (2.1%) patients presented with both microscopic hematuria and proteinuria. Patients with eGFR <60 ml/min had longer disease duration with a mean of 11 years (±7.7) in comparison to 6.4 years (±6.1) for those with eGFR>90 ml/min (P = 0.001). CONCLUSION: Renal dysfunction is not common in RA Jordanian population and has variable presentations. Age and the duration of illness play a major role in the progression of CKD if present. Future prospective studies evaluating renal biopsies in RA patients are needed.

The Efficacy of Therapeutic Selective Nerve Block in Treating Lumbar Radiculopathy and Avoiding Surgery.

BACKGROUND: Selective nerve root block (SNRB) is a procedure that can be used as a diagnostic or a therapeutic method. SNRB can be used in multiple sites, including cervical and lumbar . Our study aims to investigate the clinical effectiveness of the use of fluoroscopically guided therapeutic selective nerve root block as a non-surgical symptom management of lumbar radiculopathy. PATIENTS AND METHODS: This is a prospective study of therapeutic nerve root block in 76 patients with low back pain and/or sciatica at Jordan University Hospital. Data was collected by independent clinical interviewers, and visual analogue score (VAS) was used to measure pain severity. RESULTS: A total of 76 patients, 25 (32.8%) males and 51 (67.2%) females, underwent SNRB. 69 (90.7%) patients improved immediately after the procedure. Out of the total, 22 (28.9%) patients showed a long-term relief of symptoms and did not experience any recurrence during the three months of follow-up, while 47 (61.8%) experienced a recurrence of pain. In patients experiencing recurrence of symptoms, 35 needed surgery. CONCLUSION: Therapeutic SNRB is an important procedure in the pain management of patients with lumbar radiculopathy caused by lumbar disc prolapse and foraminal stenosis. Our study showed that avoidance of surgery was achieved in up to 54% of patients; pain relief for at least 6 months was achieved in up to 29% of patients after a single SNRB. This makes it a very good second line of management after conservative treatment and a possible method to delay, and sometimes cease, the need for surgery.

Sarcoidosis in Jordan: A Study of the Clinical Phenotype and Disease Outcome.

OBJECTIVES: This study aims to evaluate the clinical phenotypic features of sarcoidosis in a single-center academic hospital in Jordan. PATIENTS AND METHODS: A retrospective file review was performed at an academic medical center in Jordan that included all patients diagnosed with sarcoidosis between January 2000 and December 2018. A total of 150 patients with sarcoidosis (38 males, 112 females; mean age 47.8±11.7 years; range, 17 to 79 years) were evaluated. Clinical data extracted from the files included the sex of the patient, the age at time of diagnosis, diagnosis date, the season during which the diagnosis was established, and smoking history. Biopsy histopathology, spirometry, nerve conduction, echocardiography, and imaging reports including plain radiographs, ultrasonographic, magnetic resonance and computed tomography reports were reviewed. Data including laboratory values, medication usage, clinical outcomes, and morbidity/mortality were collected. Pulmonary function tests including spirometry and lung volumes along with the diffusing capacity for carbon monoxide were reviewed for the presence of restriction, obstruction or reduction in the diffusion capacity of carbon monoxide. Identification of extra-thoracic organ involvement was determined in each patient in accordance with the criteria suggested by the updated World Association of Sarcoidosis and Other Granulomatous Disorders. RESULTS: A total of 77.3% of the patients were diagnosed by biopsy. One case of Lofgren's syndrome was identified. Of the patients, 18.0% had isolated pulmonary sarcoidosis, 75.3% had pulmonary and extra-pulmonary sarcoidosis and 6.7% had isolated extra-pulmonary sarcoidosis while 81.3% had respiratory symptoms, mostly shortness of breath and cough. Extra-thoracic organ involvement mostly involved the musculoskeletal system (33%) followed by the skin (20%). Female patients had more extra-thoracic involvement but the sex difference was only statistically significant for cutaneous involvement. Of the patients, 84% received treatment while 20% had disease remission during the first two years after diagnosis and 70% required treatment beyond two years after diagnosis. CONCLUSION: Various sarcoidosis clinical phenotypes are seen among Jordanian patients. Jordanian females are more affected by the disease and have more extra-thoracic involvement compared to male patients. A large number of the study patients received treatment.