The association of several adverse prognostic factors with the development of poor renal outcome has been confirmed in this study.

Behçet’s disease (BD) is a systemic vasculitis of unknown etiology. It is characterized by recurrent oral and genital ulcers, uveitis, and a number of systemic manifestations. Although the disease is recognized worldwide, its prevalence is highly variable. A detailed review and analysis of the worldwide published reports on BD showed that not only the prevalence of the disease but also its cardiac complications differ according to the geographic distribution of the studied population. With the exception of France, Greece, Spain, and Italy, very few reports and studies regarding BD have been published from the western countries. Cardiovascular complications are an important cause of poor outcome in patients with BD. Over the past few years, many case reports and studies have been published, providing more facts about these complications. For example, peculiar echocardiographic findings in patients with aortic valve regurgitation and intracardiac thrombi secondary to BD were recently described. The role of these findings in the initial diagnosis of the disease, however, remains to be evaluated. On the other hand, some reports present contradictory results, especially concerning the left ventricular diastolic function, pathogenesis of coronary artery disease, and proper management of the cardiac complications in BD. Importantly, management of these complications is based mainly on the discretion of the treating physician due to the absence of large controlled studies and clear guidelines. This approach sometimes creates inconsistent data and allows controversies to persist. The review presented here will discuss some of the facts and controversies related to cardiac complications in BD.

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Objectives ANCA-associated vasculitis (AAV) can affect all age groups. We aimed to show that differences in disease presentation and 6 month outcome between younger- and older-onset patients are still incompletely understood. Methods We included patients enrolled in the Diagnostic and Classification Criteria for Primary Systemic Vasculitis (DCVAS) study between October 2010 and January 2017 with a diagnosis of AAV. We divided the population according to age at diagnosis: <65 years or ≥65 years. We adjusted associations for the type of AAV and the type of ANCA (anti-MPO, anti-PR3 or negative). Results A total of 1338 patients with AAV were included: 66% had disease onset at <65 years of age [female 50%; mean age 48.4 years (s.d. 12.6)] and 34% had disease onset at ≥65 years [female 54%; mean age 73.6 years (s.d. 6)]. ANCA (MPO) positivity was more frequent in the older group (48% vs 27%; P = 0.001). Younger patients had higher rates of musculoskeletal, cutaneous and ENT manifestations compared with older patients. Systemic, neurologic,cardiovascular involvement and worsening renal function were more frequent in the older-onset group. Damage accrual, measured with the Vasculitis Damage Index (VDI), was significantly higher in older patients, 12% of whom had a 6 month VDI ≥5, compared with 7% of younger patients (P = 0.01). Older age was an independent risk factor for early death within 6 months from diagnosis [hazard ratio 2.06 (95% CI 1.07, 3.97); P = 0.03]. Conclusion Within 6 months of diagnosis of AAV, patients >65 years of age display a different pattern of organ involvement and an increased risk of significant damage and mortality compared with younger patients.

Objective The objective of this paper is to investigate the utility of serum procalcitonin (PCT) and C-reactive protein (CRP) as markers of infection in systemic lupus erythematosus (SLE) patients. Patients and methods Sixty-nine SLE patients with symptoms and signs of infection proved by culture and/or a favorable response to antibiotics and 69 SLE patients without infection were included. Serum PCT and plasma high-sensitivity CRP were assessed by an enzyme-linked immunosorbent assay. Results SLE patients with infection had a significantly higher level of CRP than those without infection ((median (IQR) 104.5 (25.5-100.9) and 10.3 (5.4-23.1) mg/l, respectively), p<0.001). Conclusion Serum PCT could not differentiate SLE patients with or without bacterial infection in this study, while the utility of CRP as a marker of infection has been confirmed.

Prognosis of systemic sclerosis largely depends on involvement of internal organs. The aim was to evaluate renal impairment in patients with systemic sclerosis by measuring the Glomerular filteration rate (GFR) and then calculating the GFR using the Cockgroft and Gault formula and the Modification of Diet in Renal Disease Equation (MDRD) formula. Thirty one scleroderma patients were recruited from the Rheumatology and Rehabilitation Department, Cairo University Hospitals, mean age 43.25 +/- 11.28 years, 31 healthy controls were included. Disease severity was done using Medsger score. GFR was measured using classical Gates method TC99mDTPA. The modified Cockcroft and Gault formula and equation 7 from the MDRD were used for calculation of GFR. All patients had within normal serum creatinine levels. A normal GFR (>89ml/min) was found in 45.1%. Gates method showed reduced GFR was reported in 54.9%. Stage II chronic kidney disease (60-89 ml/min) found 32.3%, and stage III (30-59 ml/min) in 22.6%. The formulae used showed reduction of GFR in 35.29% of those affected by the Cockcroft-Gault and in 41.17% of those affected using the MDRD. No correlation to patients' age, disease duration, or severity. A positive correlation was also reported between the presence of renal involvement and pulmonary vascular involvement p = 0.04. Gates method showed reduction of the GFR in 54.9% of the systemic sclerosis patients. The formulae used were not as precise as the measured GFR in diagnosing all cases with subclinical renal involvement. Patients with systemic sclerosis should be screened for renal involvement irrespective of disease severity or duration.

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Objective The objective of this study was to describe the clinical and immunological pattern and disease outcome in Egyptian systemic lupus erythematosus patients. Patients and methods The medical records of 770 systemic lupus erythematosus patients who were followed from 2002-2015 at Kasr Alainy Hospital, Cairo University, were retrospectively reviewed. Results There were 707 (91.8%) females. The mean age at disease onset was 22.1 ± 8.6 and the disease duration was 6.1 ± 4.5 years. The main clinical manifestations were mucocutaneous (90.8% with oral ulcers affecting 52.5%), arthritis (80.3%), nephritis (67.8%), hematologic involvement (64.9%), serositis (55.2%) and neuropsychiatric manifestations (44.3%). The frequencies of antinuclear antibodies were 94.3%, anti-dsDNA 74.8%, anti-Smith 11%, anticardiolipin antibodies 29.5% and lupus anticoagulant 19.8%. Infections, predominantly bacterial, affected 337 (43.8%) patients. Thirty-three (4.3%) patients died. The main causes of death were sepsis and disease activity. The five- and 10-year survival rates for the total cohort were 97.4% and 96.3%, respectively, and were 96% and 92%, respectively for those with nephritis ( p = 0.008). Autoimmune hemolytic anemia, thrombocytopenia, elevated serum creatinine, a higher damage index, infections, a higher glucocorticoid dose and cyclophosphamide use ≥ six months were associated with an increased risk of mortality with odds ratios of 3.69, p < 0.01; 4.12, p < 0.001; 1.54, p < 0.001; 1.43, p < 0.001; 5.08, p < 0.001; 5.04, p < 0.001 and 2.25, p = 0.03, respectively. Conclusion Compared to other cohorts, a relatively lower mean age at systemic lupus erythematosus onset and higher frequencies of oral ulcers, serositis and nephritis were found.