European Journal of Rheumatology (Eur J Rheumatol) is an international, open access peer reviewed journal committed to promoting the highest standards of scientific exchange and education. The journal is published quarterly on January, April, July and October. The aim of the European Journal of Rheumatology is to cover various aspects of rheumatology for its readers, encompassing the spectrum of diseases with arthritis, musculoskeletal conditions, autoinflammatory diseases, connective tissue disorders, osteoporosis, translational research, the latest therapies and treatment programs. European Journal of Rheumatology publishes original articles, invited reviews, case based reviews, letters to the editor and images in rheumatology. The publication language of the journal is English.

Clinical Trial Registry of India: CTRI/2017/03/00824.

Despite being a common procedure, percutaneous renal biopsy (PRB) carries the potential for complications. The British Association of Paediatric Nephrologist (BAPN) has published standards for pediatric PRB. As Indian data are scarce, we conducted a prospective audit of 100 consecutive pediatric renal biopsies (60% males) under real-time ultrasound guidance. Nephrotic syndrome was the most common indication for PRB (68%) with minimal change disease (30%) and focal segmental glomerulosclerosis (25%) being the most common histopathological lesions. Gross hematuria was observed in six cases. Major complications was noted in one case, who needed longer hospital stay. The result of the audit demonstrated achievability of BAPN standards. In addition, we also show the usefulness of 16 gauge biopsy needle over 18 gauge biopsy needles (median number of glomeruli 25, range 3–90 vs 13, range 6–46, P = 0.001) without any increase in complications. Being a single center study, we do hope that our results will encourage a wider survey on the current state of pediatric PRB.

Hemophagocytic lymphohistiocytosis (HLH) is an inflammatory condition that may run a rapid fatal course and calls for prompt diagnosis. Early intervention with steroids and other immunosuppressive drugs can contain the disease process and favours positive outcome. Ferritin ≥500 ng/ml is a HLH diagnostic criterion. We evaluated the diagnostic potential of admission ferritin, in children with HLH. Pediatric patients of a referral teaching hospital from Feb 2010-Oct 2013 having been investigated for ferritin on admission were included. HLH was confirmed when patients had clinical features and met 5/8 diagnostic criteria of the revised 2004 HLH guidelines. Ferritin was estimated on Cobas e411 by electrochemiluminiscence, with a measuring range of 0.5-2000 ng/ml. Dilutions were made when linearity exceeded and absolute values were reported. 905 on-admission ferritin investigations were reviewed out of which 346 values ≥500 ng/ml. Hyperferritinemia was seen in HLH/MAS (macrophage activation syndrome) [HLH group, median age 4 year 4 month, 59% male] and in systemic lupus erythematosus, sepsis, juvenile idiopathic arthritis, impending HLH, haemolytic anemias and malignancy [non-HLH group, median age 4 year 6 month, 60% male]. Of 346, 72 cases of hyperferritinemia were diagnosed with secondary HLH while one patient had primary HLH. 13/73 patients expired. The median ferritin level of the HLH group was significantly higher [6556 (2402-11,734) ng/ml] compared to non-HLH group [median 1175 (943-2000) ng/ml] ( < 0.0001). Receiver operator characteristics curve analysis revealed optimal admission ferritin of 3120 ng/ml as the cut-off with sensitivity of 70% and specificity of 88.9% for HLH diagnosis, exceeding the currently prescribed cut-off of 500 ng/ml. Hyperferritinemia below 3120 ng/ml has higher negative predictive value to rule out secondary HLH on admission in the study population of children predominantly diagnosed with infection associated HLH than the prescribed cut-off as per the 2004 guidelines. This may prove to be beneficial to alert physicians for prompt intervention which considerably decreases mortality in this often fatal condition.

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