European Journal of Molecular & Clinical Medicine

The Antiphospholipid syndrome (APS) is an autoimmune condition characterized by recurrent arterialor venous thrombosis and/or pregnancy morbidity in the presenceof persistently positive circulating antiphospholipid antibodies. Present study was carried out to study incidence of Antiphospholipid antibody syndrome and to study clinico-pathologic correlation of APS with special reference to Lupus anticoagulant.
Material & Methods: A prospective analysis was performed in 50 patients who were suspected to have Antiphospholipid syndrome as per the diagnostic criteria. Patients with history of thrombosis, recurrent miscarriages, or k/c/o SLE who were suspected of having Antiphospholipid syndrome were included in the study.
Results: Out of 50 patients which were included in our study, (24%) were males & (76%) were females. This showed that, there was a female predominance with F: M ratio of 3.16:1. The leading presentation was bad obstetric history (multiple miscarriages, etc.) in (56%) referred cases followed by Deep Vein Thrombosis in (10%) & Portal & splenic vein thrombosis in (10%) cases. (48%) cases showed prolonged aPTT while (52%) were normal aPTT. (32%) were DRVVT positive while (68%) cases were DRVVT negative. Thrombosis was leading presentation in (11.75%) dRVVT positive cases followed by (31.25%) of Bad obstetric history (12.5%) cases were BOH + DVT. Out Of 16 APS patients, (87.50%) cases had Primary APS & (12.50%) cases had Secondary APS.
Conclusion: we conclude that any obstetric / thrombotic event should be investigated further by aPTT / dRVVT testing to reduce morbidities associated with the complication

Background: To compare efficacy of high versus low–medium prednisone doses for the treatment of systemic lupus erythematosus patients.
Materials and Methods: Seventy- four adults patients diagnosed with systemic lupus erythematosus of either gender was divided equally (37) into 2 groups. Group I received prednisone doses ≤30 mg/day and group II received prednisone doses >30 mg/day. Activity was measured using the SLEDAI-2K score. Damage accrual was calculated using the SLICC damage index (SDI).

Results: Group I comprised of 20 male and 17 female and group B had 18 male and 19 female (Table I). Age at diagnosis was 36.7 years in group I and 34.6 years in group II, Anti-Ro was seen in 34% and 38%, Anti-La in 24% and 18%, Anti-SM in 18% and 20%, Anti-RNP in 9% and 14%, Anti-DNA in 65% and 50% and Antiphospholipid antibodies in 24% and 32%. SLEDAI at baseline was 9.84 and 9.81 and SDI at baselined was 0.14 and 0.14 in group I and II respectively. The difference was significant (P< 0.05). Main organ systems affected were skin in 4 and 3, CNS in 2 and 1, vasculitis in 1 and 2, articular in 17 and 22, serosal in 5 and 6, kidney in 3 and 4 and haematological in 2 and 4 in group I and II respectively. The difference was significant (P< 0.05). The mean maximum prednisone 1st year was 14.2 and 64.1, average prednisone 1st year was 5 and 24, average prednisone years 1–2 was 3.6 and 18.5, average prednisone years 1–3 was 3.2 and 15.8, average prednisone years 1–4 was 2.7 and 15.1, pulse methyl-prednisolone during the 1st year was 12 and 3, hydroxychloroquine during the 1st year was seen in 37 and 9 and immunosuppressive drugs during the 1st year was seen in 9 and 8 in group I and II respectively. The difference was significant (P< 0.05). 

Background: Rheumatoid arthritis in children and their families can be a
significant health burden. They're linked to the risk of physical impairment, a
lower quality of life, and a lot of direct and indirect expenditures. The goal of this
study is to characterise the clinical spectrum of Juvenile Idiopathic Arthritis
(JIA) in children at Zagazig University hospitals, as well as the frequencies and
various patterns of JIA. Patients and methods: A cross-sectional research
comprised 120 patients with an average age of 16 years. From December 2017 to
December 2019, data on juvenile idiopathic arthritis was gathered during a twoyear
period. Complete blood count, reactive protein, ANA, RF, C3&C4, creatine
phosphokinase, and EMG were all performed. Management and treatment
strategies were implemented, and data on the outcomes was gathered.
Results:Females account for the majority of our rheumatological illness patients.In
our analysis, JIA was the most frequent rheumatological illness, followed by SLE
and lastly HSP.In our study, oligoarticular JIA was the most prevalent subtype of
JIA, followed by polyarticular and then systemic onset type.SLE is the second most
prevalent illness in our research, with the majority of patients being women.The
majority of individuals had cutaneous symptoms and a fever.The most commonly
utilised drugs were corticosteroids, cyclophosphamide, and mycophenolate
mofetil.HSP was the most prevalent kind of vasculitis found in our research.The
majority of patients are females, and those with severe GI symptoms and nephritis
got corticosteroids. Conclusion: The prevalence of rheumatological disorders in
children is underestimated, and there is a lot of overlap in diagnosis. Because
paediatric onset is less apparent than adult start, some patients have a significant
diagnostic lag. Early identification and proper care of these children is critical for
them to have a normal or near-normal life, particularly in patients with
rheumatological illnesses that cause chronic morbidity, such as JIA.

Background: - more specific, sensitive, and in the same time non-invasive indicators for the early prediction of juvenile SLE are required.
Aim: - to evaluate serum fractalkine (CX3CL1) as a diagnostic marker, predictor of nephritis, and indicator of disease activity in pediatric SLE.
Methods: - study included 41 children newly diagnosed with SLE, age ranged from 5 to 18 years, 24 (58.5%) were presented lupus nephritis “diagnosed by renal biopsy”. A total of 20 healthy age and sex matched children were included as controls. Serum Fractalkine levels were measured using human CX3CL1 ELISA.
Results: - Serum Fractalkine were significantly higher in SLE patients (median = 1323, range 591- 16547 IU/ml) than healthy controls (median = 950, range 591- 1583 u/ml) p=0.001. Serum Fkn can be a significant diagnostic marker for juvenile lupus indicated by the are under the ROC curve, AUC = 0.81 (95% confidence interval [CI], 0.70-0.92, P<0.001), at the level 1213 IU/ml serum Fkn detected SLE with sensitivity 0.78 (95% CI, 0.62 - 0.89), specificity 0.91 (95% CI, 0.71 – 0.99), positive predictive value 0.94 (95% CI, 0.80 – 0.98), negative predictive value 0.67 (95% CI, 0.55- 0.80), and accuracy 0.82 (95% CI, 0.71 – 0.91). Serum levels of Fkn showed no statistically significant differences when compared in patients with lupus nephritis against patients without nephritis, and was not correlated with disease activity.
Conclusions: - Serum level of Fractalkine can be a diagnostic marker for childhood onset SLE either with or without lupus nephritis, with no significant correlation with activity status or the stage of LN.

GENERALIZED DLE: Patients with generalized discoid lupus erythematosus are at a higher risk of progressing to systemic disease than those with a localized variant. Pregnancy is one of the factors that can trigger lupus erythematosus. There are only one case report published in online search that presented generalized discoid lupus erythematosus in pregnancy. We report a case of 24 year old female patient with 31-32 weeks of gestational age complained blackreddish patches accompanied by fine scales, slightly itchy and a little painful on the forehead,
cheeks, bridge of the nose, chin, ears, neck, chest, stomach, back, upper and lower extremities since ± 2 months before. ANA profile with positive result. The results of histopathologicaland dermoscopyexamination suitable with discoid lupus erythematosus. Results of the internal medicine department revealed a diagnosis of systemic lupus erythematosus. CONCLUSION:Management of DLE aims to improve general condition of patient, control the lesions, prevent of the development of further lesions; fetal growth abnormalities; preterm birth; neonatal lupus and fetal death.

Background: Lupus nephritis (LN) is a serious manifestation of systemic lupus erythematosus. Induction immunosuppression can include high dose cyclophosphamide (NIH protocol), low dose cyclophosphamide (Euro-lupus protocol) or mycophenolate mofetil (MMF). We aimed to assess the efficacy and safety of the 3 induction regimens in
Egyptian patients with LN.

Evans syndrome is a rare autoimmune disorder with an unknown etiology. In this paper, we report the case of a 32-year-old Saudi woman living with Evans syndrome for more than 8 years (post-splenectomy) who was also diagnosed with systemic lupus erythematosus (SLE). She was admitted to our Hospital with severe headache and confusion due to cerebral venous thrombosis. The major hematologic manifestations of SLE were pancytopenia and the antiphospholipid syndrome, which are indicators of disease activity when all other possible causes are excluded. The patient was treated with anticoagulation and immunosuppressive therapy and subsequently showed significant improvements in thrombosis, thrombocytopenia, and anemia. This case report provides an overview of the association between Evan syndrome and SLE.