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Systemic sclerosis (SSc) (scleroderma) is an uncommon multisystem connective tissue disease with high unmet need and mortality. There has been an improvement in overall outcome and survival over the past three decades, but it still has the highest mortality among any of the autoimmune rheumatic diseases. Progress in its management has come through more organized assessment and treatment together with the emergence of therapies that can target specific complications of the disease such as renal crisis and pulmonary arterial hypertension. In addition, there is a growing understanding of pathogenesis that allows more targeted approaches to therapy to be explored in clinical trials. In this review, several aspects of SSc management including the more targeted therapies including strategies to block specific pathways or mediators have been discussed.

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Background: In this study the clinical outcomes of different biologics agents in patients with rheumatoid arthritis (RA) has been appraised. Methods: Nineteen RA patients with DMARD failure were administered etanercept (n = 7) or infliximab (n = 12), and 17 RA patients with TNFi failure were administered rituximab (n = 13), abatacept (n = 2), or tocilizumab (n = 2) as per 2013 EULAR guidelines. Baseline demographic details, disease duration, rheumatoid factor, and anticyclic citrullinated peptide antibody were obtained. To monitor disease activity, disease activity score 28-erythrocyte sedimentation rate (DAS28-ESR) score was obtained at baseline, and after 3 months and 6 months of therapy initiation. Results: All the groups were comparable in the baseline. Over 6 months of treatment, the reduction in disease activity, as evidenced by reduction in the mean DAS28-ESR scores was statistically significant for all patients when considered together, as well as when individual biologics were considered separately (P < 0.05 in all cases). However, there was no statistically significant difference in the magnitude of reduction in the mean DAS28-ESR scores between patients who received etanercept and infliximab in DMARD failure RA patients (P = 0.877), or between patients who received rituximab, abatacept, and tocilizumab in TNFi failure patients (P = 0.455). Conclusions: Different biologic agents showed similar efficacy in patients with RA.

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A 61-year-old female presented with 6 months of polyarthralgia associated with constitutional symptoms. These included weight loss, night sweats, lethargy and worsening mobility and activities of daily living. There was no significant medical history. On examination, she had synovitis of multiple joints. Investigations for rheumatoid factor and anti-cyclic citrullinated peptide antibody were negative. There was an acute phase response in the form of raised erythrocyte sedimentation rate and C-reactive protein. Contrast-enhanced computed tomography showed pancreatic and right ovarian cystic lesions, which turned out to be clinically insignificant. Positron emission tomography-computed tomography demonstrated fluorodeoxyglucose avid lesion in the right hemi-thyroid. Ultrasound of thyroid gland showed a 13 mm hyporeflective, irregular, subcapsular nodule in the upper lobe with some microcalcification. Fine needle aspiration cytology was diagnostic of papillary carcinoma, confirmed on total thyroidectomy. Arthritis completely resolved within 8 weeks postoperatively. We report the first case of paraneoplastic carcinomatous polyarthritis in association with a papillary thyroid carcinoma as evidenced by a resolution of joint manifestations and laboratory markers of inflammation posttotal thyroidectomy.

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Background: Kawasaki disease (KD) is an important cause of acquired heart disease with significant morbidity if not diagnosed and managed appropriately. It is usually under-diagnosed due to lack of knowledge regarding this entity among pediatricians and dermatologists. In this retrospective, tertiary care teaching institute based study clinical profile and outcome of KD in children from Himalayan region of north India was appraised. Methods: Clinical details of all the cases diagnosed as KD over 3 years from January 2011 to December 2013 were collected from inpatient files and outpatient follow-up clinical records. Results: Twelve children were diagnosed to have KD out of which seven had classical KD and five had incomplete KD. Mean age at presentation was 2.45 years and male to female ratio was 1.4:1. All children were treated with intravenous immunoglobulin and aspirin. Two out of 12 children had residual coronary artery disease at 6 weeks, 3 and 6 months of follow-up echocardiography. Conclusions: KD remains an important differential diagnosis for fever, rash, and lymphadenopathy in children <5 years.

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Objective: To assess treatment responses and long-term outcomes in a multiethnic lupus cohort with proliferative lupus nephritis (LN) from a single United Kingdom (UK) center. Methods: 86 lupus patients were diagnosed with active proliferative LN between 1995 and 2015 at Barts Health, a large inner city hospital in London, UK. They were grouped by ethnicity into South Asians (Bangladeshi, Indian, Pakistani, and Sri Lankan), blacks (African Blacks and Afro-Caribbeans), and Caucasians. Remission rates were analyzed at 6 and 24 months after induction treatment with cyclophosphamide (CYC) or mycophenolate mofetil (MMF). Prognostic factors for the treatment response were identified by regression analysis. Kaplan-Meier method was applied to assess long-term renal survival and Cox proportional hazards model for risk factors for developing end-stage renal disease. Results: MMF achieved a higher remission rate in blacks compared to CYC (70% vs. 16.7%, P = 0.005) at 6 months, showed a trend in Asians (77.8% vs. 38.9%, P = 0.057), and comparable response in Caucasians (42.9% vs. 55.6%, P = 0.614). Low baseline serum creatinine was the strongest predictor for favorable treatment response (odds ratio 0.98, 95% confidence interval [95% CI]: 0.97-0.99, P = 0.045). Renal survival glomerular filtration rate (GFR >15 ml/min/1.73 m ² ) was 79.8% and 75.6% at 5 and 10 years, lowest in blacks (60.5%) followed by Asians (86.7%) then Caucasians (88.9%) (P = 0.030). Low GFR (GFR <30 ml/min/1.73 m ² ) on presentation was an independent risk factor for poor 10 years renal survival (hazard ratio 32.55, 95% CI: 3.70-286.64, P = 0.002). Conclusions: MMF appears to be at least as effective as CYC as an induction agent in this multiethnic cohort but there were important differences in long-term renal outcomes based on ethnic group and baseline GFR and creatinine.

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We report the case of a 80-year-old woman with a severe mononeuritis multiplex over bilateral lower limb, with no other clinical signs or symptoms of vasculitis. Laboratory test showed elevated erythrocyte sedimentation rate and C reactive protein, low C4 levels, positive rheumatoid factor and polyclonal IgG and IgA cryoglobulins at a high cryocrit. Malignancies disorders and infectious diseases screening was negative. Nerve biopsy revealed signs of vasculitis with inflammatory infiltrate of epineural vessels, axonal degeneration and moderate loss of myelinated fibres. The diagnosis of essential mixed cryoglobulinemia vasculitis was established. She received treatment with plasmapheresis and high doses of steroids with progression of the symptoms to the upper limbs and persistence of positive cryoglobulins, low C4 levels and high acute phase reactants. She required treatment with rituximab and mycophenolate mofetil to control the disease. In conclusion we report an uncommon clinical presentation of a cryoglobulinemia vasculitis successfully treated with two B- cell depleting therapies.

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Objective: To illustrate the role of CD134 and FAS and FAS ligand genes polymorphism as biomarkers for disease activity in Egyptian patients with Lupus Nephritis. Materials and Methods: Twenty-five patients with biopsy-proven LN, 25 patients with SLE with no evidence of nephritis, and fifty patients matched apparently healthy volunteers. Levels of CD134 were measured using flow cytometry. FAS and FASL gene polymorphisms were detected using polymerase chain reaction-restriction fragment length polymorphism. Furthermore, carotid artery intima-media thickness (IMT) measurements were done. Results: LN group had highest level of CD134 compared to other two groups, and also higher among SLE compared to controls with highly significant differences in between. Frequency of AA genotype of FASA-670G polymorphism was significantly higher in LN and SLE patients than in controls. The frequency of A allele was statistically higher in LN and in SLE group than in controls. Furthermore, the frequency of CC genotype of C-844T polymorphism of FASL gene was significantly higher in LN and SLE patients than in healthy controls. The frequency of C allele was statistically higher in LN and in SLE group than in controls. Conclusion: Co-stimulatory molecules on CD4+ T-cells together with FAS, and FASL polymorphisms are associated with disease activity in this preliminary study.

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