Anupam Wakhlu

Arthritis in leprosy

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 Charcots 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.

Leprosy revealed in a rheumatology clinic: A case series

Leprosy classically presents with cutaneous and neural involvement. Rheumatological manifestations are frequent, although often under‐recognized. At times, these may present to a rheumatology clinic prior to the diagnosis of leprosy. Herein, we present our experience with patients referred with various rheumatological disorders who were subsequently diagnosed as having leprosy.

Reliability of radiographic scoring methods in axial psoriatic arthritis.

Important differences exist between axial psoriatic arthritis (AxPsA) and ankylosing spondylitis (AS). The Bath Ankylosing Spondylitis Radiology Index (BASRI), the modified Stoke Ankylosing Spondylitis Spinal Score (mSASSS), and the Radiographic Ankylosing Spondylitis Spinal Score (RASSS) were developed to score AS, and the Psoriatic Arthritis Spondylitis Radiology Index (PASRI) to score AxPsA. We aimed to develop a computerized scoring application and compare the intra‐ and interrater reliability of these scoring systems in AS and AxPsA.

Publishing in black and white: the relevance of listing of scientific journals

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.

Vasculitis and vasculitis-like manifestations in monogenic autoinflammatory syndromes

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.

Undescended testicle: An update on fertility in cryptorchid men.

Cryptorchidism or undescended testis is one of the most common anomalies encountered in paediatric urology and is estimated to affect 1 to 4 per cent of full term and upto 30 per cent of preterm male neonates. The associated problems of sub-fertility or infertility and malignant transformation have been recognized for long. Fertility is impaired after both unilateral and bilateral cryptorchidism. The reported paternity rates in adults are about two-third for unilateral undescended testis and less than one-third for bilateral disease. Over the last five decades, the concepts related to cryptorchidism have changed dramatically as knowledge about its effects has accrued from research conducted worldwide. The recommended age of orchidopexy has fallen progressively from adolescence to less than one year. The realization that the infantile testes are not in a state of ‘suspended animation’ and the recognition of the defect in the androgen dependent transformation of gonocytes into adult dark spermatogonia in cryptorchidism have been recognized as the primary cause of sub-fertility in these patients. This has paved the way for hormone therapy in an attempt to simulate the ‘post-natal gonadotropin surge’ or ‘mini-puberty’. This review summarizes the current knowledge about the various factors affecting the fertility status in cryptorchidism with a particular focus on the derangements in the development and maturation of the germ cells and the role of surgery, hormone therapy and antioxidants in reversing these changes.

Response of Mycobacterium habana vaccine in patients with lepromatous leprosy and their household contacts. A pilot clinical study

Single dose vaccination was carried out with Mycobacterium habana vaccine, 31 lepromatous leprosy cases receiving 1.5 mg (1.5 mg = 6.27 x 10(8) bacilli) and 36 household contacts randomly receiving 1.5, 2.0, 2.5 mg vaccine intradermally. Duration of study was 18 weeks. Vaccination induced lepromin conversion in 100% of lepromatous leprosy cases and lepromin negative household contacts and augmentation of lepromin reactivity in 100% of lepromin positive household contacts, which was stable for the 15 weeks duration of follow-up. The maximum augmentation in lepromin reactivity was obtained with 1.5 mg of vaccine, which is probably the supramaximal dose. Overall, post-vaccination, those without prior BCG vaccination scars showed higher mean values of lepromin augmentation. Local vaccination site changes included induration, ulceration, itching, pain and uncomplicated regional lymphadenopathy, all of which remitted spontaneously by 15 weeks. Systemic side-effects noted were pyrexia, ENL and jaundice, and were seen with no greater frequency than that reported in other vaccine trials. Overall, systemic side-effects were easily controlled and were not accompanied by clinically detectable nerve or ocular damage. The safety profile investigations revealed an increase in the mean values of Hb%, RBC count and PCV in household contacts and of PCV in lepromatous patients, post-vaccination. Alterations in the liver function tests were also observed in patients of lepromatous leprosy. Thus, M. habana vaccine appears to be useful in stimulating specific CMI against M. leprae as evidenced by increased lepromin reactivity.

2016 update of the EULAR recommendations for the management of rheumatoid arthritis: a utopia beyond patients in low/middle income countries?

We read with great interest the recently published recommendations by the European League against Rheumatism (EULAR) on the management of rheumatoid arthritis (RA).1 The EULAR recommendations, although primarily targeted towards European countries, are read and followed across the world including low/middle income nations. Consequently, we were disappointed to note that the updated guidelines recommend the use of biological disease-modifying anti-rheumatic drugs (bDMARDs) or targeted synthetic DMARDs (tsDMARDs) immediately following failure of monotherapy with conventional synthetic DMARDs (csDMARDs) in those patients with poor prognostic factors such as seropositivity for rheumatoid factor (RF) or anticitrullinated peptide antibodies (ACPA), highly active disease or early radiographic joint damage (recommendation number 8).1 This is in contrast to the 2015 guidelines provided by the American College of Rheumatology (ACR) for the management of RA,2 which offer the option of either combining csDMARDs or using bDMARDs or tofacitinib (tsDMARD) following failure of methotrexate monotherapy in RA, irrespective of the presence or absence of such poor prognostic indicators. Early use of bDMARDs in the management of RA poses certain specific problems, as discussed below. Rheumatoid arthritis is one of the …

Sexual dysfunctions and lower urinary tract symptoms in ankylosing spondylitis.

To determine sexual dysfunctions and urinary symptoms in male ankylosing spondylitis (AS) patients and their association with various disease and patient factors.

Cutaneous polyarteritis nodosa presenting with digital gangrene and breast ulcer.

Dear Editor, Lindberg described cutaneous polyarteritis nodosa (CPAN) in 1931 as a benign form of disorder which affects the small and medium-sized arteries confined to the skin, unlike polyarteritis nodosa which is aggressive and often fatal due to multiorgan involvement. The most common clinical manifestations are subcutaneous nodules, livedo reticularis and ulceration of the lower extremity. Arthralgia, myalgia neuropathy and constitutional features may also be present. The etiology is still unknown, although an immune-mediated mechanism has been postulated with several infectious and noninfectious conditions associated with both initiation and relapse of the disease. We present a case of a patient with severe ischemic loss of both lower limbs and breast ulcer as an unusual initial manifestation of CPAN. A 23-year-old woman presented with a history of high-grade fever for 2 days followed by pain and discoloration of both feet which ended in gangrene in 10 days. She also had an ulcer on her right breast which developed over the last 10 days. There was no history of purpura, Raynaud’s phenomenon, recurrent oral ulcers, hair loss or malar rash. She did not have a bad obstetric history and her menstrual cycle was normal. She was not on oral contraceptive pills or any drugs or had any addictions (tobacco, cocaine, ergot), which would have resulted in the ischemic insult. The general physical examination showed normal vitals. Gangrene was present on both feet extending up to the ankle joint with discoloration extending up to the mid-part of the leg (Fig. 1a). Overlying skin was cold with blister formation. The breast ulcer was approximately 4 cm in size (Fig. 1b). Peripheral pulses, both anterior tibial, posterior tibial and dorsalis pedis, were not palpable and the right radial was weak. All other pulsations were present and there was no bruit over the carotid or renal arteries. The laboratory investigations showed hemoglobin 12.6 g/dL, white blood cell count 9600/lL, platelet 228 000/lL, erythrocyte sedimentation rate 50 mm/h and normal C-reactive protein (CRP). Renal and liver functions were normal. Urine analysis was normal. Blister fluid was sterile. Chest radiographs, two-dimensional echocardiography and abdominal ultrasound were