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   Table of Contents - Current issue
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November 2017
Volume 12 | Issue 6 (Supplement)
Page Nos. 141-231

Online since Thursday, November 23, 2017

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PREFACE  

Scleroderma and related disorders p. 141
Anupam Wakhlu, Durga Prasanna Misra, Vikas Agarwal
DOI:10.4103/0973-3698.219079  
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REVIEW ARTICLES Top

The pathogenesis of scleroderma p. 142
Latika Gupta, Sakir Ahmed, Abhishek Zanwar
DOI:10.4103/0973-3698.219083  
Systemic sclerosis (SSc) results from the complex interplay between the immune system, vasculature and tissue-repair mechanisms. Endothelial injury is the prime event; environmental triggers in the susceptible individual trigger the pathologic process, which translates into fibrosis. The outcome of SSc is not as bleak as it looked a couple of decades ago. With a greater understanding of older pathways, as well as elucidation of newer ones, the potential targets to block and even reverse fibrosis bring around a revolution in the way we look at this once dreaded disease.
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Biomarkers in scleroderma: Current status p. 149
Latika Gupta, Sanat Phatak, Sukesh Edavalath
DOI:10.4103/0973-3698.219087  
Scleroderma is an autoimmune disease characterized by indolent obliterative vasculopathy and widespread fibrosis. The two main morphological manifestations of the disease overlap and may make it difficult to separate activity from damage. Many patients, especially those with the limited subset of the disease, have an indolent course without clear-cut inflammatory manifestations. There is a felt need for validated biomarkers, which can differentiate activity from damage, and yet be sensitive to change with therapy. Multiplex arrays of biomarkers have ushered an era of targeted or personalized medicine based on phenotypic characteristics in an individual.
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Gastro-intestinal involvement in systemic sclerosis p. 156
Saurabh Kedia, Anuj Chhaparia, Pramod Garg
DOI:10.4103/0973-3698.219088  
The gastrointestinal (GI) tract can be involved in up to 90% of patients with systemic sclerosis (SSc) and is the leading cause of morbidity and third most common cause of mortality in these patients. The GI involvement can occur in the absence of cutaneous manifestations in 10% of patients. Vasculopathy, cellular and humoral immunity, and diffuse fibrosis are the principal pathogenetic mechanisms in SSc and begin with autoantibody-mediated neuronal damage followed by muscular damage and fibrosis. This leads to progressive dysmotility of the entire GI tract from mouth to anus and is responsible for the clinical manifestations including gastroesophageal reflux disease and dysphagia due to esophageal involvement, gastroparesis, small intestinal bacterial overgrowth and chronic intestinal pseudo-obstruction, and constipation due to colonic and fecal incontinence due to anorectal involvement. The clinical features resulting from the involvement of these organs often overlap and multiple areas may be involved simultaneously. The treatment remains mostly symptomatic because effective disease-modifying therapies are lacking. These patients are at a risk of malnutrition and nutritional screening, and thus rehabilitation is very important. Refractory cases require nutritional support in the form of enteral nutrition and/or home parenteral nutrition. Future research is needed in the pathogenesis, development of biomarkers for early identification of GI involvement at the asymptomatic stage, and targeted disease-modifying therapies, which can alter/halt the disease progression.
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Small intestinal bacterial overgrowth in patients with systemic sclerosis p. 167
Saara Rawn, Natalia Pitman, Karen Beattie, Ava Bazzaz, Maggie Larche
DOI:10.4103/0973-3698.219077  
Small intestinal bacterial overgrowth (SIBO) is common in patients with systemic sclerosis (SSc) yet often goes underrecognized in clinical practice. In patients with SSc, untreated SIBO may result in marked morbidity and possible mortality. The pathogenesis of SIBO is multifactorial and relates to immune dysregulation, vasculopathy, and dysmotility. This article reviews various diagnostic approaches and therapeutic options for SIBO. Treatment modalities mainly include prokinetics, probiotics, and antibiotics.
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Mimics of scleroderma p. 171
Kaveri K Nalianda, Mithun M Rathen, Soumya Jagadeesan, D Padmanabha Shenoy
DOI:10.4103/0973-3698.219086  
Systemic sclerosis is a rare autoimmune connective tissue disorder characterised typically by tightening and tethering of skin. However, several other disorders are also characterised by hardening and thickening of skin. These mimics can be potentially confused with systemic sclerosis, leading to a misdiagnosis. This review describes the aetiopathogenesis, clinical features and treatment of Morphea (localised scleroderma), Scleredema, Scleromyxoedema, Eosinophilic fasciitis, Nephrogenic Systemic Fibrosis, Diabetic Cheiroarthropathy, chronic GVHD, POEMS syndrome and drug induced scleroderma like illness. A careful and thorough clinical assessment is essential in order to differentiate these mimics from each other and from systemic sclerosis, establish the diagnosis, and initiate appropriate treatment.
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Scleroderma mimicker – Eosinophilic fasciitis p. 180
Debanjali Sinha, Alakendu Ghosh
DOI:10.4103/0973-3698.219081  
Eosinophilic fasciitis is an uncommon connective tissue disorder characterized by thickening of the deep fascia and overlying skin and subcutaneous tissue. It may mimic scleroderma and other scleroderma-like conditions. It may be a manifestation of paraneoplastic disorders or may be associated with hematological disorders including lymphomas. Definitive diagnosis is made on histological examination of a deep skin biopsy revealing thickened deep fascia and infiltration by lymphocytes and eosinophils. Enhancement of deep fascia on Gadolinium contrast-enhanced magnetic resonance imaging may be used as a substitute for skin biopsy. Ultrasound imaging is an evolving imaging tool for diagnosing it. Glucocorticoids with or without immunosuppressive agents remains the mainstay of therapy with good response, generally. A younger age of onset, morphea like lesions and dermal fibrosclerosis is more likely to be associated with the refractory disease. Early diagnosis and appropriate treatment may result in better outcomes in terms of morbidity and quality of life of the patients.
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Epidemic dropsy: A mimic of scleroderma? p. 185
Anupam Wakhlu, Rasmi Ranjan Sahoo
DOI:10.4103/0973-3698.219075  
Systemic sclerosis (SSc) is an autoimmune connective tissue disease involving the skin and internal organs and characterized pathologically by microvascular damage and increased deposition of connective tissue. Skin changes seen in SSc include edema, inflammation, induration, thickening, and progressive skin fibrosis. Histologically, skin fibrosis, accumulation of compact collagen in the dermis, effacement of rete pegs, infiltration by CD4+ T cells, and skin atrophy are observed. The “toxic oil syndrome” reported from Spain caused an outbreak of a scleroderma-like illness and was caused by ingestion of contaminated rapeseed cooking oil. Epidemic dropsy is caused by ingestion of mustard oil contaminated with the oil of Argemone mexicana. The major alkaloids in Argemone oil are sanguinarine and dihydrosanguinarine. These alkaloids produce widespread capillary dilatation, increased capillary permeability, and endothelial proliferation, akin to the toxic oil syndrome. Cutaneous manifestations include erythematous and tender bilaterally symmetrical pitting edema usually involving lower limbs, skin thickening and tethering, pigmentation, and presence of telangiectasias. The dermatopathology observed in epidemic dropsy includes atrophy and flattening of rete pegs, hypertrophy of and deposition of collagen, vascular dilatation and proliferation, and subcutaneous inflammation and fibrosis. Epidemic dropsy usually presents with subacute multisystem involvement, which may mimic a connective tissue disease. Skin involvement in epidemic dropsy may closely mimic cutaneous manifestations in SSc, both clinically and histologically. Thus, the clinician needs to be aware that epidemic dropsy with cutaneous involvement, especially if encountered sporadically, may be mistakenly diagnosed as scleroderma.
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Fibroblastic rheumatism p. 189
Jyoti Ranjan Parida
DOI:10.4103/0973-3698.219082  
Fibroblastic rheumatism (FR) is a rare dermoarthopathy reported from different parts of the world since 1980. Although the exact cause is unknown, few reports implicate infection may be a triggering event. Patients usually present with multiple skin nodules and polyarthropathy with progressive skin contractures. Laboratory parameters including acute phase reactants are usually normal. The confirmatory diagnosis is based on histopathologic study of skin nodules, which demonstrate fibroblastic proliferation, thickened collagen fibers, dermal fibrosis, and decreased number of elastic fibers. Immunoreactivity for b-catenin, smooth muscle actin, and the monoclonal antibody HHF35 show myofibroblastic differentiation. Treatments with oral prednisolone and other disease-modifying drugs such as methotrexate, infliximab, and interferon have been tried with variable success. In general, skin lesions respond more aptly than joint symptoms indicating that skin fibroblast is more amenable to treatment than synovial fibroblasts. Awareness regarding this orphan disease among clinicians and pathologists will help in more reporting of such cases and finding out optimal treatment regimen.
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Newer insights into the management of interstitial lung disease in systemic sclerosis p. 194
Robert L Mango, Jay H Ryu, Ashima Makol
DOI:10.4103/0973-3698.219084  
Interstitial lung disease (ILD) is a debilitating complication of systemic sclerosis (SSc) and now the leading cause of death in SSc patients, largely from progressive respiratory failure or advanced pulmonary hypertension. Despite significant advances in our understanding of the epidemiology and pathogenesis of SSc-ILD, there are significant uncertainties in the approach to managing these patients given the heterogeneity of presentation, substantial variability in progression, and presence of comorbid cardiopulmonary conditions, particularly pulmonary hypertension and esophageal dilation with recurrent aspiration pneumonitis that portend poor prognosis. Early detection of progressive lung involvement based on worsening pulmonary physiology and quantification of fibrosing alveolitis severity on high-resolution computed tomography is critical as response to immunomodulatory agents is usually best when initiated earlier in the disease course. A selected group of patients may benefit from early referral for hematopoietic stem cell transplantation or lung/heart–lung transplant. The last decade has seen a significant advance in evidence-based approaches to treatment of SSc-ILD with immune suppressants, and there are several ongoing treatment trials with recent advances in understanding of the role of pro-inflammatory and profibrotic cytokines in SSc-ILD. The efficacy of antifibrotic agents in idiopathic pulmonary fibrosis has also provided another promising avenue for utilization in these patients. In this review, we will provide an up-to-date review of the treatment options for SSc-ILD, the ongoing studies moving this field forward, emerging treatments for SSc-ILD, and propose a management algorithm for SSc-ILD, based on the available evidence in the literature and our experience.
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Screening and management of pulmonary arterial hypertension in systemic sclerosis p. 204
Vivek Nagaraja, Dinesh Khanna
DOI:10.4103/0973-3698.219085  
Systemic sclerosis-associated pulmonary hypertension (SSc-PH) and pulmonary arterial (PA) hypertension (SSc-PAH) are well-recognized manifestations. SSc-PH is a hemodynamic observation, and it is important to identify underlying etiology. SSc patients commonly have mixed etiology for SSc-PH due to interstitial lung disease, PAH, and left heart disease. SSc-PAH is associated with high morbidity and mortality. Early detection of PAH through routine screening improves survival in patients with SSc. Right heart catheterization is mandatory to diagnose PAH. SSc-PAH patients should be managed by a multidisciplinary team comprising of rheumatologist, pulmonologist, cardiologist, and physiotherapist. Various pharmacotherapy options to treat SSc-PAH are derived from the idiopathic PAH management. Upfront or sequential combination therapy of PAH-specific drugs seems to confer a clinical benefit compared to monotherapy. Cardiopulmonary rehabilitation should be considered as a part of the management plan. Lung transplantation is a consideration in patients who are not responding to pharmacotherapy. Although the long-term prognosis of SSc-PAH has been historically poor, the landscape is gradually changing with early detection and institution of treatment.
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Cardiovascular risk in systemic sclerosis: Micro- and Macro-vascular involvement p. 211
Eleni Pagkopoulou, Marina Poutakidou, Alexandros Garyfallos, George Kitas, Theodoros Dimitroulas
DOI:10.4103/0973-3698.219080  
Systemic sclerosis (SSc) is an autoimmune disease characterized by fibrosis of multiple organs (kidney, heart, lung, gastrointestinal tract, and skin), endothelial damage leading to vascular disease, and autoantibody production. Although the microvascular disease is well-understood, mechanistic insights explaining the presence and extent of macrovascular disease in SSc patients has been a matter of intense debate, especially in the past few years. Patients with systemic sclerosis have an increased risk for atherosclerotic cardiovascular disease (CVD), possibly mediated by inflammatory and fibrotic mechanisms. The excess cardiovascular risk in SSc is suggested by increased arterial stiffness, carotid intima thickening, and reduced flow-mediated dilatation. Given the involvement of the microvasculature, the differentiation between primary and ischemic heart disease is difficult. There is a relative paucity of data regarding clinical and preclinical CVD in SSc. Therefore, large cohort studies are required to clarify whether CVD is predominantly associated with atherosclerosis or microvascular involvement. The aim of this review is to discuss primary and ischemic heart disease and their contribution to CVD in SSc.
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Hematopoietic stem cell transplantation in systemic sclerosis p. 218
KG Chengappa, VS Negi
DOI:10.4103/0973-3698.219078  
Systemic sclerosis (SSc) is a disease associated with a significant morbidity and mortality, with relatively small benefits seen with immunosuppression. Of late, hematopoietic stem cell transplantation has emerged as a promising therapy for SSc with a significant reduction in mortality in the longer term. We discuss the principles behind stem cell transplantation in SSc as well as the recent trials which have shown significant promise with this technique.
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Phosphodiesterase-5 inhibitors: Raynaud's and beyond p. 227
Sanat Phatak, Sajal Ajmani, Vikas Agarwal, Durga Prasanna Misra
DOI:10.4103/0973-3698.219076  
Phosphodiesterases (PDE) are a group of ubiquitously present enzymes involved in regulation of various cellular pathways. PDE5 acts to metabolize cyclic guanosine monophosphate (GMP). The various PDE5 inhibitors available are sildenafil, tadalafil, vardenafil, and mirodenafil. We shall discuss the roles of various PDE5 inhibitors in rheumatic diseases. PDE5 inhibitors prevent degradation of cyclic GMP; hence, they have vasodilatory properties which render them useful in the management of secondary Raynaud's phenomenon. They have also demonstrated efficacy in the healing of digital ulcers in systemic sclerosis and potentially prevent the formation of new digital ulcers. Their vasodilatory property has also been utilized in the management of pulmonary arterial hypertension, wherein their ability to favorably affect hemodynamics of a pressure-overloaded right heart is beneficial. Recent evidences have suggested a potential antifibrotic role of these agents, and studies in idiopathic pulmonary fibrosis and systemic sclerosis-associated interstitial lung disease hold promise for future exploration of these agents for these indications.
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