Neglected tropical rheumatic diseases.

The complexities of dealing with rheumatic diseases in tropical countries are diverse and likely due to limited health care infrastructure, lack of diagnostic and therapeutic facilities, impact of dominant prevailing diseases, and the challenges of differentiating from infectious and non-infectious disease mimics. Several tropical diseases present with musculoskeletal and rheumatic manifestations and often pose a diagnostic dilemma to rheumatologists. The diagnosis is often delayed or the disease is misdiagnosed, leading to poor patient outcomes. Endemic tropical diseases like tuberculosis and leprosy have myriad rheumatic presentations and remain important differentials to consider in patients with rheumatic manifestations. Infection with human immunodeficiency virus is a great masquerade and can mimic manifestations of multiple diseases. The role of viral infections in triggering and perpetuating autoimmunity is well known and chikungunya arthritis is a classic example of the same. This review highlights the rheumatic manifestations of tropical diseases and aims to create awareness among the caregivers. Key Points • It is crucial to be aware and identify infectious diseases presenting with rheumatic manifestations in the tropics. • Presentations akin to classic rheumatic syndromes such as rheumatoid arthritis, spondyloarthritis, systemic lupus erythematosus and vasculitis are common.

Leprosy detection rate in patients under immunosuppression for the treatment of dermatological, rheumatological, and gastroenterological diseases: a systematic review of the literature and meta-analysis.

BACKGROUND: Recently developed immunosuppressive drugs, especially TNF antagonists, may enhance the risk of granulomatous infections, including leprosy. We aimed to evaluate the leprosy detection rate in patients under immunosuppression due to rheumatological, dermatological and gastroenterological diseases. METHODS: We performed a systematic review of the literature by searching the PubMed, EMBASE, LILACS, Web of Science and Scielo databases through 2018. No date or language restrictions were applied. We included all articles that reported the occurrence of leprosy in patients under medication-induced immunosuppression. RESULTS: The search strategy resulted in 15,103 articles; finally, 20 articles were included, with 4 reporting longitudinal designs. The detection rate of leprosy ranged from 0.13 to 116.18 per 100,000 patients/year in the USA and Brazil, respectively. In the meta-analysis, the detection rate of cases of leprosy per 100,000 immunosuppressed patients with rheumatic diseases was 84 (detection rate = 0.00084; 95% CI = 0.0000-0.00266; I2 = 0%, p = 0.55). CONCLUSION: Our analysis showed that leprosy was relatively frequently detected in medication-induced immunosuppressed patients suffering from rheumatological diseases, and further studies are needed. The lack of an active search for leprosy in the included articles precluded more precise conclusions. TRIAL REGISTRATION: This review is registered in PROSPERO with the registry number CRD42018116275 .

Paciente con dermatitis y artritis durante sus embarazos / A patient with dermatitis and arthritis during her two pregnancies

Paciente femenina que presentó durante sus dos embarazos eritema nodoso, úlceras nasales y artritis. Debido a la persistencia del eritema nodoso después del segundo parto, se practicó determinación de ANA y anti-DNA ­ IF, resultando este último positivo, por lo que se plantea el diagnóstico de lupus eritematoso y es referida al Centro Nacional de Enfermedades Reumáticas, (CNER) donde se le practicó biopsia de piel que resultó compatible con lepra: borderline o dimorfa (BB-BL) en estado reaccional tipo II (eritema nodoso leproso: ENL). Se discuten algunos de aspectos de la lepra haciendo especial énfasis en síntomas que presentó la paciente y la exacerbación de los mismos durante los embarazos, posiblemente en relación con algunos cambios inmunológicos adaptativos que son generados para mantener la tolerancia materno fetal(AU)

Female young patient with erythema nodosum, nasal ulcers and arthritis during her two pregnancies. Due to the persistence of the erythema nodosum after the second delivery, ANA and anti-DNA - IF determination were done, the latter being positive, and she was diagnosed with lupus erythematosus and was referred to the National Center for Rheumatic Diseases, where skin biopsy showed to be compatible with leprosy: borderline or dimorphic (BB-BL) in type II reactional state (leprous erythema nodosum: LEN). Some aspects of leprosy are discussed with special emphasis on symptoms that the patient presented and their exacerbation during pregnancies, possibly in relation to some adaptive immunological changes that was generated to maintain maternal fetal tolerance(AU)

Primary Neural Leprosy mimicking rheumatological disorders.

Leprosy is a chronic infectious disease caused by Mycobacterium leprae, which affects mainly the skin and peripherical nerves. Brasil has not yet achieved its goal of elimination of the number of cases of this disease, ranking second in terms of absolute numbers worldwide, with India occupying the first position. Primary Neural Leprosy is considered to be a challenge in diagnosis, since it affects the peripherical nerve system with the absence of skin lesions, thus mimicking rheumatological disorders, like in the case presented. A male, 31, with no previous comorbidities, five years ago, started feeling severe pain in the left ankle as well as morning hand pain and stiffness. After many years of being submitted to intense rheumatological disease investigation, they all proved to be negative. Upon physical examination, the patient presented no skin lesions, symmetric polyarthritis in metacarpophalangeal joints and thickness of the left sural nerve. Lab exams showed no alterations and bacilloscopy was negative. Ultrasonography was used to investigate the thickness of the left sural nerve. Biopsy showed a minimal amount of perineural lymphocytes and positive AFB testing. Based on the electroneuromyography, the conclusion was multiple mononeuropathy, and multibacillary polychemotherapy was started. Leprosy remains a public health problem in Brasil. Due to the high prevalence of the disease, our medical colleagues must be alert and trained to recognize this clinical presentation of leprosy. Correct referral to Reference Centers accelerates research, contributing to an accurate diagnosis, classification, and treatment, thus preventing irreversible sequelae with severe functional disability.

Rheumatology science and practice in India.

The practice of rheumatology in a country like India presents its own unique challenges, including the need to manage patients in a cost-constrained setting, where the lack of uniform government funding for healthcare merits the need to optimize the use of cheaper medicines, as well as devise innovative strategies to minimize the use of costlier drugs such as biologic disease-modifying agents. Use of immunosuppressive agents is also associated with increased risks of infectious complications, such as the reactivation of tuberculosis. In this narrative review, we provide a flavor of such challenges unique to Rheumatology practice in India, and review the published literature on the management of common rheumatic diseases from India. In addition, we critically review existing guidelines for the management of rheumatic diseases from this part of the world. We also discuss infectious etiologies of rheumatic complaints, such as leprosy, tuberculosis, and Chikungunya arthritis, which are often encountered here, and pose a diagnostic as well as therapeutic challenge for clinicians. There remains a need to identify and test more cost-effective strategies for Indian patients with rheumatic diseases, as well as the requirement for more government participation to enhance scant facilities for the treatment of such diseases as well as foster the development of healthcare services such as specialist nurses, occupational therapists and physiotherapists to enable better management of these conditions.

Guidelines for management of rheumatic diseases in developing countries from basics to real-world situation: relevance, need, and processes for development.

Guidelines or recommendations help to provide uniform standards in medical practice. The development of guidelines requires adherence to pre-defined norms prescribed by different international organizations such as the European League against Rheumatism (EULAR). We searched Pubmed and LILACS to identify published papers in five major rheumatic diseases (rheumatoid arthritis, systemic lupus erythematosus, spondyloarthropathies, osteoarthritis, and scleroderma) from different countries based on their economic prosperity and could find a lack of published literature from most economically weaker regions. Similarly, published guidelines in these rheumatic diseases were sparse from Asia and Africa, which are economically developed to a lesser extent than other regions of the world. Considering differing economic realities driving patient care in different regions of the world, unique challenges in certain geographic areas such as musculoskeletal manifestations of infectious diseases like leprosy and tuberculosis, as well as distinct risk of malignancies and other comorbid conditions, National Rheumatology societies should work towards developing more guidelines for rheumatic diseases from regions such as Asia and Africa, while following strictly the prescribed norms for the same. With a paucity of guidelines for such regions currently, an alternative (although less preferable) suggestion would be that major international societies, whose guidelines are widely read and followed the world over, should consider inputs from experts from diverse regions of the world while developing these guidelines.

Acro-osteolysis.

Acro-osteolysis is an osteolysis of the distal phalanges of the hands and feet and can affect the terminal tuft or the shaft of the distal phalanx (transverse or band acro-osteolysis). It is often associated with distal digital ischemia, digital calcinosis, or severe sensory neuropathy. Acro-osteolysis has been associated with a heterogeneous group of disorders, including occupational activities, infections, rheumatic disorders (systemic sclerosis, psoriatic arthritis), endocrinopathies, genetic disorders, and lysosomal storage disorders. Plain radiography is the gold standard for the detection of acro-osteolysis.

Leprosy Masquerading as Systemic Rheumatic Diseases.

Rheumatologic manifestations not only are encountered in leprosy but also can be the first and even the sole presenting manifestation. The hallmark of leprosy is skin and peripheral nerve affection; however, it can affect a wide range of other organs, with the joints being the commonest. We have searched PubMed with the key words leprosy, arthritis, vasculitis, rheumatic diseases, and autoantibodies in a proper combination. Relevant studies were retrieved from scanning of their abstracts. The relevant references provided in these articles were also selected and reviewed. We summarize the clinical and laboratory manifestations that make leprosy masquerade as a systemic rheumatic disease. Moreover, we report 4 Egyptian patients who presented with rheumatologic manifestations, namely, arthritis and vasculitis that turned out to be leprosy related.

Leprae reaction resembling rheumatologic disease as presenting feature of leprosy.

Leprosy is a chronic granulomatous infection caused by Mycobacterium leprae with predominant involvement of skin and nerves. We present a 70-year-old man with leprosy whose initial presentation resembled rheumatologic disease, due to leprae reaction. He presented with an 8-week history of worsening neuropathic pain in the right forearm, associated with necrotic skin lesions on his fingers that had ulcerated. Physical examination revealed two tender necrotic ulcers at the tip of the right middle finger and the dorsal aspect of the left middle finger. The patient had right wrist tenosynovitis and right elbow bursitis. Apart from raised inflammatory markers, the investigations for infection, connective tissue disease, vasculitis, thromboembolic disease and malignancy were negative. During the fourth week of hospitalization, we noticed a 2-cm hypoesthetic indurated plaque on the right inner arm. Further examination revealed thickened bilateral ulnar, radial and popliteal nerves. A slit skin smear was negative. Two skin biopsies and a biopsy of the olecranon bursa revealed granulomatous inflammation. He was diagnosed with paucibacillary leprosy with neuritis. He responded well to multidrug therapy and prednisolone; his symptoms resolved over a few weeks. This case illustrates the challenges in diagnosing a case of leprosy with atypical presentation in a non-endemic country.

Profile of Rheumatological Manifestations in Leprosy in a Tertiary Care Hospital from Himachal Pradesh.

The rheumatological manifestations of leprosy occur singly or in varying combinations, particularly during lepra reactions. Despite being third most common, these remain under diagnosed and under reported. This study has been carried out to study the spectrum of rheumatological manifestations in leprosy patients. One hundred consecutive patients of leprosy presenting during January to December 2013 were studied for rheumatological manifestations. Complete hemogram, serum biochemistry, urinalysis, rheumatoid factor, ASO titer, C-reactive protein, ANA, and x-rays for hands, feet, chest and involved joints were performed. These 100 (M:F 66:34) patients aged between 16-80 years had indeterminate (2 patients), TT (4 patients), BT (26 patients), BB (2 patients), and LL leprosy (32 patients). 27 patients had rheumatological manifestations; arthritis involving large or small joints in 23 patients being the commonest. 7 of 24 patients in type-1 lepra reaction had enthesitis in 3 patients and oligoarthritis in 4 patients. Rheumatoid arthritis-like polyarthritis was noted in 19 patients with type-2 reaction. Tenosynovitis, dactylitis, bony changes were also noted. Except for one case, these features were present in patients having lepra reactions. Rheumatoid factor in 14, ANA in 15, C-reactive protein in 45 cases was positive. ASO was positive 34 cases. Symmetrical polyarthritis involving small joints of hands and feet, oligoarthritis, enthesitis and dactylitis are common in leprosy particularly with borderline leprosy, type-2 lepra reaction especially in the presence of positive RAfactor.

[Analysis of 29 cases of scleritis. Experience of a Rheumato-Ophthalmology Service]. / Análise de 29 casos de esclerite. Experiência de um serviço de Reumato-Oftalmologia.

UNLABELLED: Scleritis are entities that may have rheumatic diseases, infections or tumors as etiologic factors or may be a disease restricted only to the eye. PURPOSE: To share a three year experience of a rheumato-ophthalmologic ambulatory in the diagnosis and treatment of patients with scleritis. METHODS: This was a study of 29 cases. All of them were analyzed by the Ophthalmology and Rheumatology Services. RESULTS: From the 29 cases, an underlying disease was found in 55.17%. Rheumatic diseases were present in 34.4%; infectious in 20.6%. Idiophatic forms of scleritis did not differ from the rheumatic ones although a higher number of necrotizing cases were seen in the later group (with no statistical significance). Scleritis due to rheumatic diseases was treated more frequently with oral corticosteroids and immunosuppressive drugs. CONCLUSIONS: Patients with scleritis have a high rate of infectious and rheumatic diseases. Cooperation of rheumatologists and ophthalmologists was useful for the etiologic identification of patients with scleritis.

The difficulty in diagnosis and treatment of leprosy.

Leprosy is still an important and debilitating disease with a broad clinical spectrum. However, this disease occurs most often endemically, and as an imported disease it can also still be recognized in the nonendemic industrialized world.

Análise de 29 casos de esclerite. Experiência de um serviço de Reumato-Oftalmologia / Analysis of 29 cases of scleritis. Experience of a Rheumato-Ophthalmology Service

Esclerites são doenças que podem ter como causa uma doença reumatológica, infecciosa ou tumoral ou, ainda, ser uma entidade restrita apenas ao olho. Objetivo: Relatar a experiência de dois anos de um ambulatório conjunto de Reumato-Oftalmologia no diagnóstico e acompanhamento de pacientes com esclerite por três anos. Métodos: Esta é uma análise de 29 casos de esclerite. Todos os casos foram avaliados pelo serviço de Oftalmologia e pelo de Reumatologia. Resultados: Dos 29 casos identificados, foi possível identificar uma doença subjacente em 55,17 por cento dos casos. Observamos doenças reumáticas em 34,4 por cento e infecciosas 20,6 por cento dos casos. As esclerites idiopáticas não se distinguiram das reumáticas quanto às características clínicas embora um maior número de casos com a forma necrosante tenha sido visto nas reumáticas (sem significância estatística). Esclerites com doença reumática subjacente foram tratadas mais vezes com corticóides orais e imunossupressores. Conclusões: A cooperação entre reumatologistas e oftalmologistas se mostrou útil na identificação etiológica de pacientes com esclerite.

Scleritis are entities that may have rheumatic diseases, infections or tumors as etiologic factors or may be a disease restricted only to the eye. Purpose: To share a three year experience of a rheumato-ophthalmologic ambulatory in the diagnosis and treatment of patients with scleritis. Methods: This was a study of 29 cases. All of them were analyzed by the Ophthalmology and Rheumatology Services. Results: From the 29 cases, an underlying disease was found in 55.17 percent. Rheumatic diseases were present in 34.4 percent; infectious in 20.6 percent. Idiophatic forms of scleritis did not differ from the rheumatic ones although a higher number of necrotizing cases were seen in the later group (with no statistical significance). Scleritis due to rheumatic diseases was treated more frequently with oral corticosteroids and immunosuppressive drugs. Conclusions: Patients with scleritis have a high rate of infectious and rheumatic diseases. Cooperation of rheumatologists and ophthalmologists was useful for the etiologic identification of patients with scleritis.