Cumulative pulse methylprednisolone and its relation to disease activity, damage and mortality in systemic lupus erythematosus patients: A post hoc analysis of COMOSLE-EGYPT study.

OBJECTIVE: To investigate the relation between cumulative intravenous methylprednisolone dose and disease activity, damage, and mortality among a group of Egyptian SLE patients. PATIENTS AND METHODS: This is a post hoc analysis of a retrospective multicenter COMOSLE study. Cumulative pulse methylprednisolone dose was abstracted from COMOSLE database. Patients with cumulative pulse dose of ≤ 3.0 g (median dose) were compared to those with cumulative dose of > 3.0 g regarding demographic data, Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and The Systemic Lupus International Collaborating Clinics/ACR Damage Index (SLICC) score as well as treatment received. Additionally, at 1.5, 3, 6, and 9 g of cumulative methylprednisolone, patients were compared regarding SLICC score and risk of mortality. RESULTS: Patients who received > 3 g of methylprednisolone were statistically significantly younger at disease onset, had longer disease duration, higher SLEDAI score at last visit, and higher SLICC score (p = 003, p = 0.002, p = 0.004 and p = < 0.001, respectively). Additionally, with every gram increase in the cumulative methylprednisolone, there was a significant increase in SLICC score by 0.169 (B = 0.169, CI = 0.122-0.216, p-value = < 0.001) and an increased risk of mortality by 13.5% (hazard ratio (HR) = 1.135, CI = 1.091-1.180, p-value = 0.001). The best cutoff value of methylprednisolone dose at which damage may occur, ranged between 2.75 (with sensitivity of 81.4% and specificity of 33.9%) and 3.25 g (with sensitivity of 48.3% and specificity of 71.5%). CONCLUSION: With every gram increase in the cumulative methylprednisolone, there may be increase in damage and mortality, especially in doses exceeding the range of 2.75-3.25 g. Key Points • Treatment of systemic lupus erythematosus should be with the least possible dose of steroids to decrease the risk of damage and mortality. • With every gram increase in the cumulative intravenous methylprednisolone there may be increase in damage and mortality.

High- and low-dose cyclophosphamide in Egyptian lupus nephritis patients: a multicenter retrospective analysis.

BACKGROUND: Lupus nephritis (LN) is a common serious presentation of systemic lupus erythematosus. Cyclophosphamide (CYC) and mycophenolate mofetil (MMF) are listed as the first-line drugs in induction therapy for LN. OBJECTIVE: This study aimed to compare high- and low-dose CYC in a cohort of Egyptian LN patients. PATIENTS AND METHODS: The data of 547 patients with class III/IV active LN who received CYC as induction therapy were retrospectively analyzed. Whereas 399 patients received 6­monthly 0.5-1 g/m2 CYC doses, 148 patients received six biweekly 500 mg CYC doses. Demographic data, laboratory test results, and disease activity index were recorded and compared at presentation and at 6, 12, 18, 24, and 48 months of follow-up. RESULTS: After 48 months, the proportion of patients maintaining normal creatinine levels was higher in the group receiving induction therapy with high-dose CYC (67.9%, 60.4%, p = 0.029), and these patients also had higher proteinuria remission at 36 (26.6%, 14.8%, p = 0.014) and 48 months (24.3%, 12.8%, p = 0.006). Comparison of patient outcomes according to both induction and maintenance therapy showed the best results in patients who received high-dose CYC and continued MMF as maintenance therapy. CONCLUSION: High- and low-dose CYC are comparable in early phases of treatment. However, after a longer duration of follow-up, high-dose CYC was associated with higher remission rates in the current cohort.

COVID-19 outcomes in patients with and without autoimmune rheumatic diseases: A multicenter comparative study.

BACKGROUND AND OBJECTIVE: People with rheumatic diseases are particularly concerned with the coronavirus disease 2019 (COVID-19) pandemic. Our work aimed to study the impact of pre-existing autoimmune rheumatic disease (AIRD) and its immunosuppressive drugs on COVID-19 severity and outcome. PATIENTS AND METHODS: This is a multicenter case-control study performed between September 2020 and February 2021 on 130 adults with COVID-19, including 66 patients with AIRD and 64 without AIRD, who served as a control group. RESULTS: Regarding COVID-19 clinical manifestations; diarrhea, fatigue, and headache were found with significantly higher frequency in the AIRD group while a higher frequency of cough was found in the control group. Comparing COVID-19 complications, only septic shock was significantly higher in the AIRD group (P = 0.013). Both groups were treated with similar COVID-19 drugs except for tocilizumab and anticoagulants, which were statistically significantly more frequently used in the control group (P < 0.001 for both). No statistically significant difference was found between the groups in the outcome or severity of COVID-19. There was no impact of previous immunosuppressive drugs before COVID-19 on the severity of the disease except for a longer duration of recovery in patients on steroids (P < 0.001). Patients with hypertension had severe COVID-19 compared with those without (odds ratio 2.8, 95% confidence interval 1.2-6.9; P = 0.020). CONCLUSION: AIRD may not affect COVID-19 severity and outcome. Similarly, immunosuppressive medications had no effect; except that patients on systemic steroids had longer duration for recovery. Comorbid conditions, such as hypertension, may be associated with more severe COVID-19 disease course.

Comorbidities among Egyptian systemic lupus erythematosus: The COMOSLE-EGYPT study.

OBJECTIVE: To study the prevalence and impact of comorbidities among a cohort of patients with systemic lupus erythematosus (SLE). METHODS: This study is retrospective, multicenter including 902 Egyptian patients with SLE. Medical records were reviewed for demographic data, clinical characteristics, routine laboratory findings, immunological profile, and medications. Moreover, SLE Disease Activity Index (SLEDAI), and the Systemic Lupus International Collaborating Clinics/American College Rheumatology Damage Index scores were calculated. RESULTS: Comorbidities were found in 75.5% of the studied group with hypertension and dyslipidemia as the most frequent comorbidities (43.1% and 40.1%, respectively), followed by sicca features, avascular necrosis, diabetes, osteoporosis and renal failure (11.5%,9%, 9%,8.9%, and 7.1%, respectively). Multivariate regression model showed statistically significant relation between the presence of comorbid condition and each of age (P = 0.006), disease duration (P = 0.041), SLEDAI at onset (P < 0.001), cyclophosphamide intake (P = 0.001), and cumulative pulse intravenous methylprednisone (P < 0.001). Also, when adjusted to age and sex, those with multiple comorbid conditions had 18.5 increased odds of mortality compared to those without comorbidities (odds ratio (OR), 95% confidence interval (CI) = 18.5 (6.65-51.69)]. CONCLUSION: Patients with SLE suffer from several comorbidities, with an increasing risk with age, longer disease duration, higher SLEDAI at onset, cyclophosphamide intake and cumulative pulse intravenous methylprednisone. Risk of mortality is exponentiated with multiple comorbidities.

Disease characteristics in patients with juvenile- and adult-onset systemic lupus erythematosus: A multi-center comparative study.

Objectives: This study aims to compare disease characteristics in patients with juvenile-onset systemic lupus erythematosus (JSLE) and adult-onset systemic lupus erythematosus (ASLE). Patients and methods: Between June 2010 and March 2020, a total of 186 patients with JSLE (23 males, 163 females; median age: 25 years; range, 20 to 30.3 years) and 236 patients with ASLE (23 males, 213 females; median age: 35 years; range, 29 to 40 years) were retrospectively analyzed. Clinical and laboratory data, treatment received, Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and Systemic Lupus International Collaborating Clinics (SLICC)/ACR Damage Index (SDI) scores, comorbidities and deaths were compared between the groups. Results: The JSLE patients showed statistically significant higher constitutional manifestations, cardiac manifestations, serositis, nephritis, end-stage renal disease, neurological manifestations, gastrointestinal manifestations, secondary vasculitis, Raynaud's, livedo-reticularis, dry mouth, dry eye, ocular manifestations, avascular necrosis, hematological manifestations, and hypocomplementemia (p<0.001, p=0.016, p=0.005, p=0.001, p=0.04, p<0.001, p<0.001, p<0.001, p=0.002, p=0.043, p=0.004, p=0.03, p<0.001, p=0.01, p<0.001, and p=0.001, respectively). Median SLEDAI scores were statistically significant higher in the JSLE group, both at onset (p<0.001) and in the final follow-up visit (p<0.001). Median SLICC scores were also higher in the JSLE group (p<0.001). Mycophenolate mofetil and intravenous pulse steroids were more frequently used in the juvenile group (p<0.001 and p=0.03, respectively). Hypertension, dyslipidemia, and avascular necrosis were found to be statistically significantly higher in the JSLE group (p<0.001, p=0.006, and p=0.01, respectively). The mortality rate was statistically significantly higher in the JSLE group than the ASLE group (p<0.001). Conclusion: The JSLE patients showed more serious manifestations, higher disease activity, higher damage index, and mortality rate compared to ASLE patients. These results suggest the need of a regular follow-up and close surveillance of JSLE patients.

What is peripheral spondyloarthritis? Identifying proportion, phenotype and burden in post hoc analysis of the ASAS-PerSpA study.

BACKGROUND: Little is known about the prevalence, phenotype, and burden of peripheral spondyloarthritis (pSpA). The objective of the study is to compare the phenotype and burden of disease of pure pSpA to that of pure psoriatic arthritis (PsA), pure axial SpA (axSpA), and combined forms of SpA. METHODS: This is a post hoc analysis of 4,185 patients from the cross-sectional ASAS-Peripheral involvement in SpA (PerSpA) study. Patients were approached in 2 ways: the first approach was based on the rheumatologist's diagnosis (diagnostic approach) and the second one was based on the fulfillment of ASAS or CASPAR classification criteria (classification criteria approach). Demographics, disease phenotype, and burden were compared among pure pSpA, PsA, axSpA, and the combined forms. FINDINGS: The proportion of pSpA was 31.5% of SpA using the classification criteria approach and 10.3% using the diagnostic approach. pSpA was pure (i.e. without axSpA or PsA) in 16.8% of pSpA using the criteria, and in 62.3% using the diagnostic approach. Using classification criteria and diagnostic approach, respectively, pure pSpA patients had a high prevalence of peripheral joint disease (86 and 96%), synovitis (76 and 91%), and enthesitis (57 and 55%), a positive HLA-B27 in 65 and 59%, a high C-Reactive Protein level in 51% and inflammatory back pain in 52 and 42%. However, compared to pure PsA and pure axSpA, they had a significantly higher disease burden, but lower use of biologics using both approaches. INTERPRETATION: The proportion of pSpA varies when using the classification criteria or the diagnostic approach. pSpA occurred in a pure form less frequently than PsA and axSpA and had intermediate features but a higher disease burden. FUNDING: The PerSpA main study has been conducted under the umbrella of ASAS thanks to unrestricted grants from PFIZER, LILLY, ABBVIE, NOVARTIS, UCB, JANSSEN, MERCK.

Root joint involvement in spondyloarthritis: a post hoc analysis from the international ASAS-PerSpA study.

OBJECTIVES: The primary objective was to compare the clinical characteristics of SpA patients with and without root joint disease (RJD+ and RJD-). The secondary objectives were to compare the prevalence of RJD across various SpA subtypes and in different world regions, and to compare the SpA axial severity and SpA burden between RJD+ and RJD-. METHODS: This is a post hoc analysis of the Assessment of Spondyloarthritis International Society PerSpA study (PERipheral involvement in SpondyloArthritis), which included 4465 patients with SpA [axial (axSpA), peripheral (pSpA), PsA, IBD, reactive and juvenile] according to the rheumatologist's diagnosis. RJD was defined as the 'ever' presence of hip or shoulder involvement related to SpA, according to the rheumatologist. Patient characteristics were compared between RJD+ and RJD-. Multivariable stepwise binary logistic regression analyses were conducted to identify factors associated with 'RJD', 'hip' and 'shoulder' involvement. RESULTS: RJD was significantly associated with the SpA main diagnosis (highest in pSpA), a higher prevalence of HLA-B27 positivity, enthesitis, tender and swollen joints, CRP, conventional synthetic DMARDs, loss of lumbar lordosis and occiput-wall distance >0. RJD was more prevalent in Asia, and occurred in 1503 patients (33.7%), with more hip (24.2%) than shoulder (13.2%) involvement. Hip involvement had a distinct phenotype, similar to axSpA (including younger age at onset, HLA-B27 positivity), whereas shoulder involvement was associated with features of pSpA (including older age at onset). CONCLUSION: RJD+ SpA patients had a distinctive clinical phenotype compared with RJD-. Hip involvement, based on the rheumatologist's diagnosis, was more prevalent than shoulder involvement and was clinically distinct.

Vasculitis damage index in Behçet's disease.

BACKGROUND: Vasculitis damage index (VDI) is a validated damage index for systemic vasculitis, and as Behçet's disease is considered one of systemic vascular disease we aimed to study the relationship of the vasculitis damage index to clinical manifestations and comorbidity in patients with Behçet's disease (BD) to determine if VDI could be used to assess damage in patients with BD. METHODS: A total of 109 patients with BD were recruited from the Rheumatology Department (outpatient and inpatient clinic), Cairo University Hospitals. All patients were subjected to full history taking, clinical examination, and routine laboratory investigations. Disease activity was assessed by the BD current activity form, and the VDI was calculated in all patients. The relationship of the VDI to the disease clinical manifestations was studied. Mann-Whitney and Kruskal Wallis tests were used to estimate differences in quantitative variables. Spearman correlation test was used to test for correlation between quantitative variables. RESULTS: In the current study, the VDI ranged from 1 to 10, with a mean of 3.5 ± 1.8. It was significantly associated with total thrombosis (P = 0.022); total neurological manifestations (P = 0.000), especially stroke and cranial nerve affection; uveitis (P = 0.005); avascular necrosis (AVN) (P = 0.015); osteoporosis (P = 0.01); impaired vision (P < 0.0001); cataract (P < 0.0001); and diabetes (P = 0.012). Generally, immunosuppressive treatment was significantly associated with VDI (P = 0.039), especially cyclophosphamide (P < 0.0001), biological agent (P = 0.008), chlorambucil (P = 0.003), and anticoagulant (P = 0.02). VDI was also significantly correlated with age (P = 0.033), disease duration (P = 0.029), and duration of eye involvement (P = 0.003). CONCLUSION: VDI is significantly associated with most disease parameters of BD, except for parameters such as mucocutaneous manifestations and uncomplicated venous thrombosis; however, further studies may be needed to establish BD-specific damage index.

Does smoking affect level of seropositivity in RA? A post-HOC global and inter-country analysis of COMORA cohort.

To study the association of smoking status and the level of seropositivity in RA patients from COMORA Cohort. A post hoc analysis of COMORA database included 3439 RA patients was performed. Current smokers or recently quitted (< 3 years) were initially compared to those who never smoked or stopped > 3 years (Group I vs. II) regarding their seropositivity status (high positive, low positive and negative) for Rheumatoid Factor (RF) or Anti-citrullinated antibodies (ACPA). A further comparison was made between current smokers (Group III) and never smoked patients (Group IV). Analysis was also done on the individual country level for the 17 countries included in the COMORA study. Out of 3439 RA patients, 705 (20.5%) were smokers (group I), and 2734 (79.5%) were non-smokers (group II). Significantly more patients in group I, 442 (62.7%), had high levels of seropositivity than those in group II, 1556 (56.9%), [P = 0.006, OR 1.27 (95% CI, 1.07-1.5)]. More current smoker patients (group III-286 out of 456 "62.7%") had high levels of seropositivity than never smoked patients (group IV-1236 out of 2191 "56.4%"), with significant difference [P = 0.013, OR 1.3 (95% CI, 1.06-1.6)]. In 11 countries, higher proportions of patients with high level of seropositivity in group I was found, with statistical significance in four countries. Smoking was associated with higher level of seropositivity in patients with RA in this post hoc analysis, both on a global level and in certain individual countries. As smoking is a modifiable risk factor, studying the effects of quitting smoking on level of seropositivity and other disease parameters is warranted.

Lupus-related vasculitis in a cohort of systemic lupus erythematosus patients.

Objectives: This study aims to examine the frequency and clinical association of lupus-related vasculitis in patients with systemic lupus erythematosus (SLE). Patients and methods: We retrospectively analyzed medical records of a total of 565 SLE patients (42 males, 523 females; mean age: 32.7±9.5 years; range, 13 to 63 years) between January 2017 and February 2020. Demographic, clinical data, and laboratory data and treatment modalities applied were recorded. Lupus-related vasculitis and its different types were documented, and the patients with vasculitis were compared with those without vasculitis. Results: The mean disease duration was 8.9±6.3 years. Vasculitis associated with lupus was found in 191 (33.45%) patients. Cutaneous vasculitis was found in 59.2%, visceral vasculitis in 34.0%, and both in 6.8% of total vasculitis patients. The patients with vasculitis had a longer disease duration (p=0.01), were more likely to have juvenile onset (p=0.002), livedo reticularis (p<0.001), Raynaud's phenomenon (RP) (p<0.001), digital gangrene (p<0.001), thrombosis (p=0.003), and cranial neuropathy (p=0.004). The patients with vasculitis showed a higher prevalence of hypercholesterolemia (p=0.045), diabetes mellitus (p=0.026), higher Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) at disease onset (p<0.001), and Systemic Lupus International Collaborating Clinics (SLICC) Damage Index (p=0.003) scores. They had more prevalent hematological manifestations (p<0.001), hypocomplementemia (p=0.007), received a higher cumulative dose of intravenous methylprednisolone (p<0.001), and had also more frequent cyclophosphamide (p=0.016) and azathioprine intake (p<0.001). In the logistic regression analysis, SLE vasculitis was independently associated with juvenile disease onset, livedo reticularis, RP, hematological manifestations, and higher scores of SLEDAI at disease onset (p<0.05). Conclusion: Juvenile disease onset, livedo reticularis, RP, hematological manifestations, and higher SLEDAI scores at disease onset may be associated with the development of vasculitis in SLE patients.

Vasculitis damage index in Behçet's disease

Abstract Background: Vasculitis damage index (VDI) is a validated damage index for systemic vasculitis, and as Behçet's disease is considered one of systemic vascular disease we aimed to study the relationship of the vasculitis damage index to clinical manifestations and comorbidity in patients with Behçet's disease (BD) to determine if VDI could be used to assess damage in patients with BD. Methods: A total of 109 patients with BD were recruited from the Rheumatology Department (outpatient and inpatient clinic), Cairo University Hospitals. All patients were subjected to full history taking, clinical examination, and routine laboratory investigations. Disease activity was assessed by the BD current activity form, and the VDI was calculated in all patients. The relationship of the VDI to the disease clinical manifestations was studied. Mann-Whitney and Kruskal Wallis tests were used to estimate differences in quantitative variables. Spearman correlation test was used to test for correlation between quantitative variables. Results: In the current study, the VDI ranged from 1 to 10, with a mean of 3.5 ± 1.8. It was significantly associated with total thrombosis (P = 0.022); total neurological manifestations (P = 0.000), especially stroke and cranial nerve affection; uveitis (P = 0.005); avascular necrosis (AVN) (P = 0.015); osteoporosis (P = 0.01); impaired vision (P < 0.0001); cataract (P < 0.0001); and diabetes (P = 0.012). Generally, immunosuppressive treatment was significantly associated with VDI (P = 0.039), especially cyclophosphamide (P < 0.0001), biological agent (P = 0.008), chlorambucil (P = 0.003), and anticoagulant (P = 0.02). VDI was also significantly correlated with age (P = 0.033), disease duration (P = 0.029), and duration of eye involvement (P = 0.003). Conclusion: VDI is significantly associated with most disease parameters of BD, except for parameters such as mucocutaneous manifestations and uncomplicated venous thrombosis; however, further studies may be needed to establish BD-specific damage index.

Inter-observer agreement of standard joint count examination and disease global assessment in a cohort of Egyptian Rheumatoid Arthritis patients / Acuerdo entre observadores del examen estándar de recuentos articulares y la escala visual analógica en una cohorte de pacientes con artritis reumatoide egipcia

OBJECTIVES: To assess the inter-observer agreement of standard joint count between experienced Rheumatology professor (Prof) and young Rheumatology fellow (candidate), and to compare disease global assessment between professor, young candidate and patients. METHODS: This study included one hundred rheumatoid arthritis patients. For all patients independent clinical evaluation was done by two rheumatologists (professor and candidate) for detection of tenderness in 28 joints and swelling in 26 joints. The study also involved global assessment of disease activity by the provider (Prof and candidate) (EGA) as well as by the patient (PGA). The EGA was determined without previous knowledge of the patient's laboratory test results. RESULTS: A highly significant accordance (correlation) between professor and candidate was found in both the number of tender joints (p < 0.001) (r=0.946), and the number of swollen joints (p < 0.001) (r=0.797). Regarding swollen joints, the highest agreement was in right knee (0.929), while poor agreement was found in the right 5th MCP (0.049). Regarding tender joints, the highest analogy was in the right elbow (0.899), in contrast to the left 3rd PIP (0.462) which showed the least congruence. Agreement study using kappa measurement for disease global assessment showed: moderate agreement (between professor and candidate) (0.405), fair agreement between (professor and patient) (0.213), fair agreement between (candidate and patient) (0.367). CONCLUSION: Inter-observer reliability was better for TJCs than SJCs. Regarding SJCs agreement was better in large joints such as the knees compared to the small joints such as the MCPs. Disease global assessment may show discrepancy between patients and physicians

OBJETIVOS: Evaluar el acuerdo interobservador del recuento de articulaciones estándar entre el profesor experimentado y el candidato joven, y comparar la evaluación global de la enfermedad entre el profesor, el candidato joven y los pacientes. MÉTODOS: Este estudio incluyó a 100 pacientes con artritis reumatoide. Para todos los pacientes, la evaluación clínica independiente fue realizada por 2 reumatólogos (profesor y candidato) para la detección de la sensibilidad en 28 articulaciones y la hinchazón en 26 articulaciones. También en este estudio evaluaciones globales de la actividad de la enfermedad por el proveedor (prof y candidato) Se realizó el paciente (PGA) para cada paciente. El EGA se determinó sin conocimiento previo de los resultados de la prueba de laboratorio del paciente. RESULTADOS: Se encontró una concordancia altamente significativa (correlación) entre el profesor y el candidato tanto en el número de articulaciones sensibles (p <0.001) (r = 0.946), y el número de articulaciones inflamadas (p <0.001) (r = 0.797). En cuanto a las articulaciones inflamadas, el mayor acuerdo fue en la rodilla derecha (0,929), mientras que el acuerdo pobre fue encontrado en el 5to MCP derecho (0.049). En cuanto a las articulaciones sensibles, la mayor analogía fue en el codo derecho (0,899), encontraste con el tercer PIP izquierdo (0.462) que mostró la menor congruencia. El estudio del acuerdo utilizando la medición kappa para la evaluación global de la enfermedad mostró: Acuerdo moderado (entre profesor y candidato) (0,405), Acuerdo justo entre (profesor y paciente) (0.213), Acuerdo justo entre (candidato y paciente) (0,367). CONCLUSIÓN: La con fiabilidad entre observadores fue mejor para TJCs que SJCs. Con respecto a los SJC, el acuerdo fue mejor en las articulaciones grandes, como las rodillas en comparación con las articulaciones pequeñas, como los MCP. La evaluación global de la enfermedad puede mostrar discrepancias entre los pacientes y los médicos

Inter-observer agreement of standard joint count examination and disease global assessment in a cohort of Egyptian Rheumatoid Arthritis patients.

OBJECTIVES: To assess the inter-observer agreement of standard joint count between experienced Rheumatology professor (Prof) and young Rheumatology fellow (candidate), and to compare disease global assessment between professor, young candidate and patients. METHODS: This study included one hundred rheumatoid arthritis patients. For all patients independent clinical evaluation was done by two rheumatologists (professor and candidate) for detection of tenderness in 28 joints and swelling in 26 joints. The study also involved global assessment of disease activity by the provider (Prof and candidate) (EGA) as well as by the patient (PGA). The EGA was determined without previous knowledge of the patient's laboratory test results. RESULTS: A highly significant accordance (correlation) between professor and candidate was found in both the number of tender joints (p<0.001) (r=0.946), and the number of swollen joints (p<0.001) (r=0.797). Regarding swollen joints, the highest agreement was in right knee (0.929), while poor agreement was found in the right 5th MCP (0.049). Regarding tender joints, the highest analogy was in the right elbow (0.899), in contrast to the left 3rd PIP (0.462) which showed the least congruence. Agreement study using kappa measurement for disease global assessment showed: moderate agreement (between professor and candidate) (0.405), fair agreement between (professor and patient) (0.213), fair agreement between (candidate and patient) (0.367). CONCLUSION: Inter-observer reliability was better for TJCs than SJCs. Regarding SJCs agreement was better in large joints such as the knees compared to the small joints such as the MCPs. Disease global assessment may show discrepancy between patients and physicians.

Growth Differentiation Factor-15 (GDF-15) Level and Relation to Clinical Manifestations in Egyptian Systemic Sclerosis patients: Preliminary Data.

AIM OF THE WORK: This study aims to assess Growth differentiation factor-15 (GDF-15) level in Scleroderma patients and its relation to disease manifestations. PATIENTS AND METHODS: This study included 55 scleroderma patients and 40 age and sex matched healthy volunteers. All patients were subjected to full history taking, thorough clinical examination, and laboratory investigations. GDF-15 serum levels were analyzed in patients and controls using human GDF-15 immunoassay Quantikine ELISA kit. RESULTS: The GDF-15 serum level was significantly higher in Systemic sclerosis (SSc) patients in comparison to healthy control individuals, p-value = 0.004. In addition, the GDF-15 serum levels increased in a significant way in patients with diffuse SSc than those with limited SSc, p = 0.026. Also, we had discovered a significant positive correlation between serum GDF-15 levels and the modified Rodnan score of the SSc patients, r = 0.442, p = 0.001 and a significant association was found between high GDF-15 level and SSc patients with interstitial pulmonary fibrosis (IPF) as compared to healthy controls (p = 0.002). However, no significant difference was found between SSc patients without IPF and healthy subjects regarding GDF-15 level (p = 0.106). CONCLUSION: GDF-15 serum levels were elevated in patients with SSc and correlated with the extent of skin fibrosis, and it was found to be higher in SSc patients with IPF. Such results may suggest a pivotal role of GDF-15 in fibrotic changes in SSc, and GDF-15 could be a treatment target in SSc patients in future.

Matrix Metalloproteinase-9 rs17576 Gene Polymorphism and Behçet's Disease: Is There an Association?

BACKGROUND: Clinical studies have reported a significant association between matrix metalloproteinases (MMP), particularly (MMP-9), and inflammatory diseases including Behçet's disease (BD). PURPOSE: To study the relationship between MMP-9 rs17576 gene polymorphism and the development of BD, and its relation to disease activity among Egyptian patients. METHODS: A total of 100 BD patients and 100 healthy control volunteers were genotyped for MMP-9 rs17576 polymorphism with polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP), followed by the confirmation of our results in random subgroups using direct DNA sequencing technique. RESULTS: The frequency of the GG genotype and G allele was significantly higher in BD patients as compared to the normal controls (p = 0.011, OR 8.61; p = 0.03, OR 1.65, respectively). There was no significant association between the MMP-9 rs17576 polymorphism and the clinical outcomes of BD. CONCLUSION: our study suggests a significant association of the MMP-9 rs17576 A/G polymorphism with increased risk of BD development in Egyptian patients.

Association of VDR ApaI and TaqI Gene Polymorphisms with the Risk of Scleroderma and Behçet's Disease.

Vitamin D deficiency and vitamin D receptor (VDR) gene polymorphisms have been reported in autoimmune diseases. However, their role in Behçet's disease (BD) and scleroderma remains inconclusive. Our aim was to evaluate vitamin D receptor (ApaI, TaqI) gene polymorphisms in relation to Behçet's disease and scleroderma in Egyptians. The study was conducted on 54 patients with BD, 30 scleroderma patients, and 60 healthy control subjects. VDR (ApaI, TaqI) gene polymorphisms were investigated using polymerase chain reaction-restriction fragment length polymorphism technique. The "a" allele of ApaI (A/a) polymorphism was significantly associated with increased BD risk (OR = 2.09, 95% CI = 1.18-3.71, p = 0.011), while the TaqI "tt" genotype was significantly lower in BD patients as compared to control subjects (OR = 0.35, 95% CI = 0.13-0.9, p = 0.026). Carriage of "aT" VDR haplotype was significantly associated with higher BD risk (OR = 2.28, 95% = 1.14-4.56, p = 0.022). In conclusion, our findings suggest that VDR gene polymorphisms have a significant association with BD in Egyptian patients.

Comparison of comorbidities of the Egyptian rheumatoid arthritis patients to the global cohort of the COMORA study: a post-hoc analysis.

The aims of this study are to present the results of Egyptian RA patients included in COMORA cohort and compare it to general COMORA cohort, concerning prevalence of comorbidities, and level of application of recommendations related to detection/prevention of comorbidities. Three-hundred eight Egyptian RA patients included in the cross-sectional, observational, multi-center, international study "COMORA", were compared to the total number of 3612 RA patients. The CRF of COMORA was used in all patients. CRF collects demographic and disease characteristics, comorbidities, risk factors, and compliance with recommendations regarding management of comorbidities. Data were analyzed according to COMORA protocol. Egyptian RA patients were significantly younger, had more active disease, and were more functionally disabled. They showed more frequent use of NSAIDs, methotrexate and steroids and significantly lower use of bDMARDs when compared to non-Egyptians. Egyptian patients had the highest ever HCV prevalence, while depression, hypertension, smoking and dyslipidemia were less prevalent in Egyptians. Prevalence of malignancy risk factors was highly deficient among Egyptians; primarily due to lack of screening. Further, following recommendations for monitoring comorbidities is significantly deficient among Egyptian patients. Egyptian patients had more active disease and more functional impairment than the rest of the COMORA cohort; with lower use of bDMARDs, that is possibly related to the economic situation. Also, there is a clear gap in screening and monitoring comorbidities. Awareness among Egyptian healthcare providers (and possibly similar third-world countries) to detect and manage RA-related comorbidities is required.