Leprosy, a chronic granulomatous infection caused by Mycobacterium leprae, classically presents with cutaneous and neurological manifestations. Musculoskeletal involvement though third most common is underdiagnosed and underreported. It may manifest in the form of Charcot's arthropathy, acute symmetrical polyarthritis or swollen hands and feet syndrome during lepra reactions, insidious-onset chronic symmetrical polyarthritis mimicking RA or as isolated tenosynovitis or tenosynovitis associated with arthritis or neuropathy. At times, articular involvement may be the sole presenting manifestation even without cutaneous lesions. Other rheumatological manifestations occasionally reported are enthesitis, sacroiliitis, cryoglobulinaemic vasculitis and DM. With increasing travel of population between tropical and temperate zones, it is likely that rheumatology clinics in countries free of leprosy may come across cases of leprosy with rheumatological manifestations. Delay in diagnosis and management may be detrimental and may result in deformities and loss of function. Not only this, but recent reports of leprosy being diagnosed in native white populations following anti-TNF-α therapy should alert rheumatologists across the globe to be more familiar with this disease. This review is aimed at presenting a comprehensive clinical scenario of various rheumatological manifestations of leprosy to sensitize rheumatologists and physicians across the continents.

Takayasu arteritis (TA) is a challenging large vessel vasculitis to treat. Distinguishing disease activity from vascular damage is difficult, often relying on clinician judgement aided by composite clinical disease activity indices with angiographic evidence of vessel wall thickening or vessel wall hypermetabolism demonstrable on positron emission tomography computerized tomography (PET CT). Glucocorticoids form the mainstay of remission induction. While other conventional disease modifying anti-rheumatic drugs (cDMARDs) or biologic DMARDs (bDMARDs) are commonly used, evidence supporting their usefulness is sparse and generally of low quality. The only two randomized controlled trials (RCT) of a DMARD in TA failed to show efficacy of abatacept in reducing relapses of TA, however, tocilizumab showed a trend towards reduction in time to relapses. Of the cDMARDs, methotrexate, azathioprine, mycophenolate mofetil (MMF), leflunomide and cyclophosphamide have shown clinical efficacy in case series, with some evidence that methotrexate, azathioprine and MMF might retard angiographic progression. Among bDMARDs, anti-tumor necrosis factor alpha agents and tocilizumab may be useful in patients refractory to cDMARDs with retardation of angiographic progression, based on evidence derived from mostly retrospective case series, whereas the role of rituximab and ustekinumab needs further elucidation. Revascularization, either surgical or endovascular, is the treatment of choice to relieve critical, symptomatic stenoses and are best undertaken during inactive disease. Emerging evidence suggests that patients with TA also have increased cardiovascular risk and this requires appropriate management. Large studies involving multiple centers are the need of the hour to appropriately evaluate utility of currently available immunosuppressive therapy in TA.

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Scientific publishing, including in the field of Rheumatology, is evolving rapidly. Predatory journals are one of the major threats to contemporary publishing, especially to eager and naïve authors. In this narrative review, we discuss mechanisms that authors can employ to white list genuine scientific journals and blacklist "predatory" ones. Inclusion of a journal in reputed indices such as Medline (but not just Pubmed), Web of Science, Scopus or Embase raises the likelihood that the journal is genuine, more so if it is included in the current Journal Citation Reports. Other commercially available whitelists also exist, so also whitelists published by regulatory authorities in some countries. A commercially available blacklist has emerged since the very useful Beall's blacklist became defunct. In the absence of access to a whitelist or blacklist, certain characteristics such as repeated email solicitations for articles with an extremely narrow deadline from unknown sources, lack of inclusion in reputed indices, journals not published or endorsed by national or international society and scarcity of currently published articles should render authors suspicious of the genuine nature of a journal. National societies should work together to generate subject-specific (including Rheumatology specific) whitelists that can be available free of cost to authors from all over the world.

Rheumatological presentations of leprosy may mimic RA, spondyloarthropathy or vasculitis. Pure neuritic variety and spontaneous type 2 lepra reaction pose unique diagnostic challenges. Increased awareness may avoid delay in diagnosis.

Teratomata, by reason of their bizarre nature and relatively rare occurrence, have aroused interest for many centuries. The main sites of occurrence in order of frequency are the gonads, the retroperitoneal region, the anterior mediastinum, presacral and coccygeal regions and the base of the skull (Willis, 1953). Calbet (cited by Riker and Potts, 1948) calculated that a sacro-coccygeal tumour occurred in every 34,582 births; of these a relatively small proportion were teratomata. The first known record of a sacro-coccygeal teratoma was made on a cuneiform tablet in about 2000 B.C. and was stored in the Royal Library of Nineveh (Ballantyne, 1894). Amongst thousands of tablets found there, one dealt with the portents of ' I 1 monstrous births and various deformities found in newly born infants. On this tablet it was stated that ' when a woman gives

Objectives  To correlate renal function with the site of the ectopic orifice in patients with a single ectopic ureter and to evaluate the role of ureteric reimplantation in the preservation of renal function. Patients and methods  Forty‐four patients (41 female, age 1.5 months to 20 years) with a single ectopic ureter have been managed in our institution in the last 21 years. The classical symptom of continuous wetting with intermittent normal micturition was reported in most of the female patients. The investigative evaluation included intravenous urography (IVU), cysto‐urethroscopy, vaginoscopy with retrograde ureteric catheterization, micturating cysto‐urethrography (MCU) and ultrasonography. Diuretic renography was carried out in four patients after it became available in 1992. Renal function was assessed in relation to urinary tract anomalies and with outcome after ureteric re‐implantation. Results  Thirty‐eight patients (two males) had a unilateral ectopic ureter; the ectopic orifice was vaginal in 12, vestibular in 11, urethral in nine, at the bladder neck in two, the seminal vesicle in one and undetermined in three. Twenty‐one patients had renal and/or ureteric abnormalities, with reflux detected on MCU in three ureters. Associated anomalies included hypospadias (two, one female), skeletal anomalies (two), anorectal malformations (three), cryptorchidism (two), and unilateral cystic ovary (one). Two patients had preoperative hypertension. In 15 patients, renal function was considered sufficient to justify ureteric reimplantation, 14 of whom regained continence. One girl had suprapubic leakage from the bladder and died during secondary nephroureterectomy. Another girl had persistent incontinence; she was found to have contralateral duplex ureters with a vestibular ectopic orifice and was cured after upper polar hemi‐nephroureterectomy. IVU and renography carried out in two patients each within 4 weeks of surgery showed a moderate improvement in renal function. Eight patients reported for follow‐up after ureteric reimplantation (mean duration 11 months); none had hypertension or urinary infection. Twenty‐three patients with rudimentary kidneys underwent nephroureterectomy. Histopathological examination of the excised kidneys showed moderate to severe dysplasia with chronic pyelonephritis. Six patients (one male) had bilateral single ectopic ureters, with normal renal function in the five females. Unilateral reimplantation in the boy resolved the symptoms; one girl died before surgery and the other four underwent bilateral ureteric reimplantation, after which one was dry for up to 3 h while the other three were incontinent, one of whom subsequently underwent urinary diversion. Conclusions  There was no clear correlation of renal function with the site of the ectopic ureteric orifice, as most of the patients with a vaginal ectopic ureter had sufficient renal function to justify renal preservation. Ureteric reimplantation preserved renal function, although the improvement after surgery was determined by the deg...

Monogenic autoinflammatory syndromes are a rare group of disorders characterized by periodic episodes of systemic inflammation of endogenous origin. Sometimes, these diseases may present with features akin to vasculitis. We conducted a literature review on such vasculitic manifestations in described monogenic autoinflammatory syndromes utilizing the Online Mendelian Inheritance in Man (OMIM), Medline, and Scopus databases. Our search identified that Familial Mediterranean fever (FMF) can manifest with features of either small, medium, large, or variable-vessel vasculitis. Stimulator of interferon gene (STING)-associated vasculopathy of infancy (SAVI) is an interferonopathy that can mimic the presentation of medium-vessel or small-vessel vasculitis, whereas deficiency of adenosine deaminase 2 (DADA2) is another such mimic of medium-vessel vasculitis, associated in a significant number of patients with features of immunodeficiency. Occasional reports exist of vasculitic manifestations in tumor necrosis factor (TNF) receptor-associated periodic fever syndrome (TRAPS) and chronic infantile neurologic cutaneous and articular disorder (CINCA), whereas mevalonate kinase deficiency can also mimic the presentation of small- or medium-vessel vasculitis. Clinicians should be aware of the possibility of autoinflammatory disease presenting as vasculitis to diagnose and treat the same appropriately.