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1
Gross, Wolfgang L., and Julia U. Holle. Clinical features of ANCA-associated vasculitis. Oxford University Press, 2013. http://dx.doi.org/10.1093/med/9780199642489.003.0131.
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The primary ANCA-associated vasculitides are granulomatosis with polyangiitis (Wegener's, GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome, CSS). They predominantly affect small (and medium-sized) vessels and share a variable association with ANCA (anti-neutrophil cytoplasm antibody) directed against neutrophil proteinase 3 (PR3, mainly in GPA) and myeloperoxidase (MPO, mainly in MPA and CSS). Crescentic necrotizing glomerulonephritis and alveolar haemorrhage due to pulmonary capillaritis represent classical (vasculitic) organ manifestations of the ANCA-associated vasculitides (AAV). MPA occurs as a 'pure' small (to medium-size) vessel vasculitis, whereas GPA and CSS are characterized by additional distinct clinical and pathological features. In GPA, granulomatous lesions of the upper and/or lower respiratory tract are a hallmark of the disease. Granulomatous lesions may be large in appearance and occur as space-consuming, infiltrating, and destructive inflammatory masses. GPA is believed to follow a stagewise course with an initial localized form, restricted granulomatous lesions of the upper and/or lower respiratory tract without clinical signs of vasculitis, and a consecutive generalization to systemic vasculitis which may be either non-organ-threatening (early systemic) or organ- and life- threatening (generalized GPA). Rarely, patients arrest in the localized stage and do not progress to systemic disease. In EGPA asthma, hypereosinophilia and eosinophilic organ infiltration (e.g. eosinophilic myocarditis) are typical features of the disease apart from vasculitis. Similarly to GPA, EGPA follows a stagewise course: asthma and eosinophilia may precede full-blown disease for several months or years. Recent cohort studies suggest different phenotypes in EGPA (predominantly vasculitic and MPO-ANCA-positive and predominantly with eosinophilic organ infiltration, usually ANCA-negative). This chapter focuses on the clinical features of the primary AAV and their outcome.
2
Bjartell, Anders, and David Ulmert. Clinical features, assessment, and imaging of prostate cancer. Edited by James W. F. Catto. Oxford University Press, 2017. http://dx.doi.org/10.1093/med/9780199659579.003.0063.
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In contemporary practice, most patients with prostate cancer are diagnosed following a prostate-specific antigen (PSA) test and are asymptomatic at the time of diagnosis. Although serum PSA has a low specificity for prostate cancer, it can be used to single out patients with advanced disease. While most men do not have a palpable tumour at digital rectal examination (DRE), those with palpable or an elevated PSA test require transrectal ultrasonography-guided prostate biopsy in order to make a diagnosis of cancer. Tumours are staged clinically as localized, locally advanced, or metastatic. The urologist and the patient need the correct staging information for decision-making. A combination of several parameters (PSA value, Gleason grade and tumour extent on biopsy, and DRE findings) can be used in a variety of tools to predict the extent of the disease and treatment outcomes.
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Patel, Nilay, David Cranston, and Mark Sullivan. The aetiology, epidemiology, clinical features, and investigation of kidney cancer. Edited by James W. F. Catto. Oxford University Press, 2017. http://dx.doi.org/10.1093/med/9780199659579.003.0083.
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Over 270,000 patients worldwide are diagnosed with renal cancer every year. It is the most lethal of all urological malignancies, with 33–44% of patients dying as a result of the disease. The past three decades has seen the incidence of renal cancer increasing by approximately 2% per year. This increased incidence has predominantly been within localized tumours, detected incidentally due to the increased use of cross-sectional imaging in medical practice. Despite an increase in the number of patients undergoing surgery for renal cancer, mortality rates have continued to rise. There is some evidence to suggest this may be a consequence of the overdiagnosis and overtreatment of small renal masses. At present, there is no justification for national screening programmes for renal cancer.
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Misbah, Siraj. Suspected anaphylaxis. Edited by Patrick Davey and David Sprigings. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780199568741.003.0075.
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A type I IgE-mediated systemic allergic reaction is characterized by a constellation of symptoms which are due to widespread histamine release and which comprise acute-onset urticaria, angioedema, bronchospasm, and hypotension. While a mild reaction may be limited to localized urticaria and/or angioedema, a full-blown allergic reaction associated with systemic features is best described as anaphylaxis. The term ‘anaphylactoid’, previously used to denote non-IgE-mediated systemic allergic reactions, is no longer recommended for use.
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Glazov, M. M. Spin Systems in Semiconductor Nanostructures. Oxford University Press, 2018. http://dx.doi.org/10.1093/oso/9780198807308.003.0002.
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This chapter is an introduction to a rich variety of effects taking place in the interacting system of electrons and nuclei in semiconductors. It includes also the basics of electronic properties of nanostructures and of spin physics, an overview of fundamental interactions in the electron and nuclear spin systems, the selection rules at optical transitions in semiconductors, spin resonance effect, as well as optical orientation, and dynamical nuclear polarization. In this chapter an analysis of particular features of spin dynamics arising in the structures with localized electrons such as quantum dots, which are studied further in the book, are addressed. The aim of this chapter is to provide basic minimum of information needed to read the remaining chapters.
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Tuschl, Karin, Peter T. Clayton, and Philippa B. Mills. Disorders of Manganese Metabolism. Oxford University Press, 2016. http://dx.doi.org/10.1093/med/9780199972135.003.0045.
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Manganese is an essential trace metal for numerous metalloenzymes. Manganese homeostasis requires tight regulation in vivo and disruption of this balance can lead to manganese overload and subsequent accumulation of manganese in brain, liver, and blood. Mutations in SLC30A10, a cell surface-localized manganese efflux transporter, cause an autosomal recessive hypermanganesemia syndrome with two distinct phenotypes: childhood onset dystonia and adult onset Parkinsonism, associated with chronic liver disease, polycythemia and features of iron depletion. MRI brain appearances are characteristic of Mn deposition with hyperintense basal ganglia on T1-weighted images. Chelation therapy with disodium calcium edetate and iron supplementation effectively lower blood manganese levels, halt liver disease progression and improve neurological symptoms.The inherited form of hypermanganesemia can be distinguished from acquired causes of manganese overload including environmental overexposure and acquired hepatocerebral degeneration in cases of end stage liver disease.
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Sybert, Virginia P. Disorders of Epidermal Appendages. Oxford University Press, 2017. http://dx.doi.org/10.1093/med/9780190276478.003.0003.
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Chapter 3 starts by covering conditions of the hair, including Alopecias (Loose Anagen Hair, Male Pattern Baldness, and Marie Unna Syndrome), Hirsutism (Gingival Fibromatosis and Hypertrichosis, Hypertrichosis Lanuginosa Congenita, Leprechaunism, and Localized Hypertrichosis), and Hair Shaft Abnormalities (including Monilethrix, Pili Annulati, Pili Torti, Pili Trianguli Et Canaliculi, Trichorrhexis Invaginata, Trichorrhexis Nodosa, Woolly Hair, Menkes Disease, Trichodentoosseous Syndrome, Trichorhinophalangeal Syndrome, and Trichothiodystrophy). It then covers conditions of the nails, including Congenital Malalignment of the Great Toenails, Familial Dystrophic Shedding of the Nails, Leukonychia, Twenty-Nail Dystrophy, Nail-Patella Syndrome, Onychotrichodysplasia and Neutropenia, and Pachyonychia Congenita). Conditions of the Sweat Glands (Hidradenitis Suppurativa, Hyperhidrosis, and Multiple Syringomas), Sebaceous Glands (Eruptive Vellus Hair Cysts, Familial Dyskeratotic Comedones, Oral-Facial-Digital Syndrome Type I, and Steatocystoma Multiplex), and Ectodermal Dysplasia Syndromes (AEC Syndrome, Clouston Syndrome, EEC Syndrome, Focal Facial Ectodermal Dysplasia, GAPO Syndrome, Hypohidrotic Ectodermal Dysplasia, and Tooth and Nail Syndrome) are also covered. Each condition is discussed in detail, including dermatologic features, associated anomalies, histopathology, basic defect, treatment, mode of inheritance, prenatal diagnosis, and differential diagnosis.
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O’Neill, Brian P., Jeffrey Allen, Mitchell S. Berger, and Rolf-Dieter Kortmann. Astrocytic tumours: pilocytic astrocytoma, pleomorphic xanthoastrocytoma, and subependymal giant cell astrocytoma. Oxford University Press, 2017. http://dx.doi.org/10.1093/med/9780199651870.003.0002.
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Pilocytic astrocytoma (PA) (World Health Organization (WHO) grade I). A relatively circumscribed, slow-growing, often cystic astrocytoma occurring in children and young adults, histologically characterized by a biphasic pattern with varying proportions of compacted bipolar cells associated with Rosenthal fibres and loose-textured multipolar cells associated with microcysts and eosinophilic granular bodies. Most PAs are localized, macrocystic, and only marginally infiltrative. However some PAs, such as those arising in the optic pathways, are rarely cystic and may have an extensive infiltrative pattern but within a neuroanatomic pathway. Pleomorphic xanthoastrocytoma (PXA) (WHO grade II). An astrocytic neoplasm with a relatively favourable prognosis, typically encountered in children and young adults, with superficial location in the cerebral hemispheres and involvement of the meninges; characteristic histological features include pleomorphic and lipidized cells expressing glial fibrillary acidic protein and often surrounded by a reticulin network as well as eosinophilic granular bodies. Subependymal giant cell astrocytoma (SEGA) (WHO grade I). A benign, slow-growing tumour typically arising in the wall of the lateral ventricles and composed of large ganglioid astrocytes. It is the most common CNS neoplasm in patients with tuberous sclerosis.
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Sybert, Virginia P. Disorders of the Epidermis. Oxford University Press, 2017. http://dx.doi.org/10.1093/med/9780190276478.003.0002.
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Chapter 2 covers Ichthyoses (Bullous Congenital Ichthyosiform Erythroderma, Harlequin Ichthyosis, Ichthyosis Bullosa of Siemens, Ichthyosis Hystrix, Ichthyosis Vulgaris, Lamellar Exfoliation of the Newborn, Lamellar Ichthyosis/Nonbullous Congenital Ichthyosiform Erythroderma, Netherton Syndrome, Peeling Skin Syndrome, Restrictive Dermopathy, and X-linked Recessive Ichthyosis), Erythrokeratodermas (Erythrokeratodermia Variabilis ET PROGESSIVA, and Pityriasis Rubra Pilaris), Acrokeratoderma (Acrokeratoelastoidosis, Acrokeratosis Verruciformis (HOPF)), Hereditary Palmoplantar Keratodermas (Hereditary Palmoplantar Keratoderma with Deafness, Hereditary Palmoplantar Keratoderma Epidermolytic Hyperkeratosis, Hereditary Palmoplantar Keratoderma Howel-Evans, Hereditary Palmoplantar Keratoderma Olmsted, Hereditary Palmoplantar Keratoderma Punctate, Hereditary Palmoplantar Keratoderma Striata, Hereditary Palmoplantar Keratoderma Unna-Thost, Hereditary Palmoplantar Keratoderma Vohwinkel, Keratolytic Winter Erythema, Mal de Meleda, Papillon-Lefèvre, Scleroatrophic and Keratotic Dermatosis of the Limbs), Porokeratoses (Porokeratosis of Mibelli), Other Disorders of the Epidermis (Absence of Dermatoglyphics, Acanthosis Nigricans, Darier-White Disease, Hereditary Painful Callosities, Keratosis Follicularis Spinulosa Decalvans, Knuckle Pads, Kyrle/Flegel Disease, Ulerythema Ophryogenes), Syndromic Disorders (CHILD Syndrome, Chondrodysplasia Punctata, Ichthyosis with Hypogonadism, KID Syndrome, Neu-Laxova Syndrome, Neutral Lipid Storage Disease with Ichthyosis, Refsum Disease, Richner-Hanhart Syndrome, Sjögren-Larsson Syndrome), Cohesion (Epidermolysis Bullosa, Epidermolysis Bullosa Simplex Dowling-Meara, Epidermolysis Bullosa Simplex Generalized, Epidermolysis Bullosa Simplex Localized, Epidermolysis Bullosa Junctional Generalized, Epidermolysis Bullosa Junctional Generalized Atrophic Benign, Epidermolysis Bullosa Dystrophica Cockayne-Touraine, Epidermolysis Bullosa Dystrophica, Hallopeau-Siemens, Epidermolysis Bullosa Dystrophica Pretibial, Transient Bullous Dermolysis of the Newborn, Hailey-Hailey Disease). Each condition is discussed in detail, including dermatologic features, associated anomalies, histopathology, basic defect, treatment, mode of inheritance, prenatal diagnosis, and differential diagnosis.
10
Hickey, Sam, and Naomi Hossain, eds. The Politics of Education in Developing Countries. Oxford University Press, 2019. http://dx.doi.org/10.1093/oso/9780198835684.001.0001.
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This book examines the politics of the learning crisis in the global South, where learning outcomes have stagnated or worsened, despite progress towards Universal Primary Education since the 1990s. Comparative analysis of education reform in Bangladesh, Cambodia, Ghana, Rwanda, South Africa, and Uganda highlights systemic failure on the frontline of education service delivery, driven by deeper crises of policymaking and implementation: few governments try to raise educational standards with any conviction, and education bureaucracies are unable to deliver even those learning reforms that get through the policy process. Introductory chapters develop a theoretical framework within which to examine the critical features of the politics of education. Case study chapters demonstrate that political settlements, or the balance of power between contending social groups, shape the extent to which elites commit to adopting and implementing reforms aimed at improving learning outcomes, and the nature this influence takes. Informal politics and power relations can generate incentives that undermine rather than support elite commitment to development, politicizing the provision of education. Tracing reform processes from their policy origins down to the frontline, it seems that successful schools emerged as localized solutions to specific solutions, often against the grain of dysfunctional sectoral arrangements and the national-level political settlement, but with local political backing. The book concludes with discussion of the need for more politically attuned approaches that focus on building coalitions for change and supporting ‘best-fit’ types of problem-solving fixes, rather than calling for systemic change.
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Hangan, Horia, and Ahsan Kareem, eds. The Oxford Handbook of Non-Synoptic Wind Storms. Oxford University Press, 2020. http://dx.doi.org/10.1093/oxfordhb/9780190670252.001.0001.
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This handbook is currently in development, with individual articles publishing online in advance of print publication. At this time, we cannot add information about unpublished articles in this handbook, however the table of contents will continue to grow as additional articles pass through the review process and are added to the site. Please note that the online publication date for this handbook is the date that the first article in the title was published online. For more information, please read the site FAQs. Wind storms impact human lives, their built as well as natural habitat. During the last century, society’s vulnerability to wind storms has been reduced by enhanced knowledge of their impact and by controlling exposure through better design. However, only two of the wind systems have so far been considered in the design of buildings and structures, i.e., synoptic winds resulting from macroscale weather systems spanning thousands of kilometers, e.g., extratropical storms, and mesoscale tropical storms spanning hundreds of kilometers and traveling fast, e.g., hurricanes/typhoons/cyclones. During the last two decades, enough evidence has surfaced to support that a third type of very localized wind storms, the non-synoptic winds, are the most damaging in some regions of the world. Thus far there are no design provisions established for the codification of these wind storms. Their characterization in terms of climatology, wind field and intensity, frequency and occurrence, as well as their impact on the built environment, is slowly developing. This handbook presents the state-of-the-art of knowledge related to all these features including their risk, insurance issues, and economics. The research in this area is on the one hand more arduous given the reduced scale, the three-dimensionality, and nonstationary aspects of these non-synoptic winds while, at the same time, its understanding and modeling are being aided by the emergence of novel modeling and simulation techniques which are addressed in this handbook. This will serve as a guiding resource for those interested in learning about and contributing to the advancement of the field.
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Keshav, Satish, and Alexandra Kent. Diagnosis in suspected gastrointestinal disease. Edited by Patrick Davey and David Sprigings. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780199568741.003.0194.
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The most frequent presenting complaints suggesting gastrointestinal disease are diarrhoea, constipation, nausea, vomiting, anorexia, and abdominal pain, which can localize to any of the quadrants of the abdomen observed from the front. Loss of weight is a feature of some gastrointestinal diseases, and general symptoms such as fever, malaise, and arthralgia may also occur. According to the National Institute for Clinical Excellence (NICE), alarm symptoms include unintentional weight loss, dysphagia, chronic gastrointestinal bleeding, iron deficiency anaemia, an abdominal mass, and/or persistent vomiting.
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Youngblood, Mark W., and Hal Blumenfeld. Biological Basis of Primary Generalized Epilepsies—Pathophysiology. Oxford University Press, 2017. http://dx.doi.org/10.1093/med/9780199937837.003.0037.
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The primary generalized epilepsies include a heterogeneous group of seizures including absence, myoclonic, and generalized tonic-clonic seizures that are not strictly localized on EEG and not secondary to another disorder. The seizures are often associated with a loss of consciousness and may present with motor manifestations, including convulsions and arrest of respiration. Generalized spike-and-wave discharges on electroencephalogram are a uniting feature, and this pattern of activity is a direct manifestation of the underlying mechanism of these disorders. A review of important underlying circuitry will set the stage to discuss the pathological and genetic basis of these disorders, and the chapter will conclude with a review of current and potential therapeutics.
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Quack, Joachim Friedrich. On the Regionalization of Roman-Period Egyptian Hands. Oxford University Press, 2018. http://dx.doi.org/10.1093/oso/9780198768104.003.0008.
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In earlier periods of Egyptian history, cursive writing tends to display a certain degree of uniformity all over the country, and it is difficult to localize a hieratic text just on the basis of its writing style. Beginning in the Ptolemaic period and even more so in Roman imperial times, indigenous Egyptian scripts tend to become regionalized to such a degree that, for relatively well-known places, the attribution of an unprovenanced item simply on the basis of the individual hand can become a viable option. Even places of comparatively limited distance can develop seriously different features in orthography as well as preferred sign forms. The most likely explanation is that there was no super-regional centre setting standards to be emulated all over the country. Thus, teaching Egyptian writing was purely a local tradition taking place in the temple schools, and local habits could grow freely.
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Kindler, David T., Marcela Morales, and Paul Stacey. Education For All: Ten years of open education luminaries from around the world - In celebration of Open Education Global’s 10th Anniversary of Open Education Awards for Excellence. buch & netz, 2021. http://dx.doi.org/10.36862/oeg-415.
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Education for all is a bold, audacious statement. But that is the very goal of open education. Can you imagine a world where access to education materials is free? Where teachers and learners have the right to reuse, revise, remix, localize and translate those materials? Where copies of textbooks and course materials can be retained without cost? Can you imagine a world where teachers and learners co-create education together? A world where learners engage in assignments that generate global public goods benefiting everyone? You may say this isn’t possible, but open educators around the world have been doing this for years. Building on the work of luminaries such as those featured in this book, open education has grown into a global movement transforming education. Each year, Open Education Global opens up nominations for awards to the entire global open education community. As part of the 10th anniversary of these awards, OEGlobal is publishing this Education For All book, collecting all ten years of award winners into a single volume. This book is a celebration of their achievements.
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D'Arcens, Louise. World Medievalism. Oxford University Press, 2021. http://dx.doi.org/10.1093/oso/9780198825944.001.0001.
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World Medievalism: The Middle Ages in Modern Textual Culture explores the ways in which a range of modern textual cultures have continued to engage creatively with the medieval past in order to come to terms with the global present. Building its argument through four case studies—from the Middle East, France, Southeast Asia, and Indigenous Australia–it shows that to understand medievalism as a cultural idiom with global reach, we need to develop a more nuanced grasp of the different ways ‘the Middle Ages’ have come to signify beyond Europe as well as within a Europe that has been transformed by multiculturalism and the global economy. The book’s case studies are explored within a conceptual framework in which medievalism itself is formulated as ‘world-disclosing’—a transhistorical encounter that enables the modern subject to apprehend the past ‘world’ opened up in medieval and medievalist texts and objects. The book analyses the cultural and material conditions under which its texts are produced, disseminated, and received and examines literature alongside films, television programs, newspapers and journals, political tracts, as well as such material and artefactual texts as photographs, paintings, statues, buildings, rock art, and fossils. While the case studies feature distinctive localized forms of medievalism, taken together they reveal how imperial and global legacies have ensured that the medieval period continues to be perceived as a commonly held past that can be retrieved, reclaimed, or revived in response to the accelerated changes and uncertainties of global modernity.
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MacLean, Allan B. Vulval pain. Oxford University Press, 2017. http://dx.doi.org/10.1093/med/9780198749547.003.0009.
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Vulval pain or pain involving the vulval tissue is discussed in Chapter 9. It becomes chronic when lasting for at least three months. Vulvodynia is a subset of chronic vulval pain, once known causes (infective, inflammatory, neoplastic, neurological, traumatic, iatrogenic and hormone deficiencies) are excluded. It reportedly affects one in six women at some stage of their lives. Uncertain terminology has hampered understanding. Even the latest classification from the International Society for the Study of Vulvovaginal Disease has deficiencies but it allows the discarding of previously used unhelpful terms. Differentiating features between provoked (entry dyspareunia), and unprovoked, localised and generalised, overlap, both in diagnosis and management. Older theories on causation included infection, irritation and inflammation but laboratory-based research has not supported these. Hormonal and neural mechanisms seem more likely to cause the pain, while the interplay of biological, psychological, and social factors has recently gained credence. Publications on successful management demonstrate a powerful placebo effect. The role of specially designated vulval pain clinics, multidisciplinary approaches, and team working is emphasised. General measures in vulval care, such as wearing clothes made of natural fibre, using emollients or carrying out pelvic floor exercises besides reducing stress, can minimise the pain. Topical anaesthetic creams or systemic treatments with antidepressants or anti-epileptics have advocates. Treatment is most effective when careful selection, adequate counselling, and ongoing psychosomatic evaluation address all the interactive factors that initiate, and maintain vulval pain besides modulating patient response. Case scenarios illustrate the complexities of diagnosis and management.
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Kaufmann, Philipp A., and Oliver Gaemperli. Hybrid Cardiac Imaging. Oxford University Press, 2015. http://dx.doi.org/10.1093/med/9780199392094.003.0028.
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Assessment of both coronary anatomy and myocardial perfusion are equally important for the appropriate treatment of patients with stable coronary artery disease. Cardiac hybrid imaging allows integration of coronary anatomy and perfusion in one all-in-one image, thereby avoiding mental integration of findings. In selected subgroups of patients, cardiac hybrid imaging has demonstrated superior diagnostic accuracy compared to single modalities. The combination of coronary anatomy and function provides incremental prognostic information and improves risk stratification of patients with suspected or known CAD. Aside from CT coronary angiography, coronary artery calcium score (CACS) scans obtained from native ECG-triggered CT are used for hybrid imaging. They are used either for attenuation correction, or can be combined with radionuclide information to improve CAD detection and risk stratification. A large number of integrated hybrid scanners are commercially available and offer advantages for cardiac hybrid imaging. However, these devices are not mandatory, and hybrid imaging is perfectly feasible from two separate datasets using appropriate image fusion software. Cardiac magnetic resonance has entered the arena of hybrid imaging and several integrated PET/MRI devices are already commercially available. Its advantages include the lack of ionizing radiation and a high spatial resolution, particularly for soft tissue structures. In research, hybrid imaging moves beyond its conventional borders of perfusion imaging to target specific molecular or biological pathways that underlie cardiac disease, a concept known as molecular imaging. The combination of radionuclide imaging with CT or MRI offers attractive features to co-localize biological signals from radiolabeled targeted compounds with microanatomical structures.
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Denton, Christopher P., and Pia Moinzadeh. Systemic sclerosis. Oxford University Press, 2013. http://dx.doi.org/10.1093/med/9780199642489.003.0121.
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The term 'scleroderma' describes a group of conditions in which the development of thickened, fibrotic skin is a cardinal feature. This includes localized forms of scleroderma (e.g. morphoea) and also systemic forms of the disease that are more correctly termed systemic sclerosis. Systemic sclerosis (SSc) is a multiorgan, autoimmune disease that has a high clinical burden and mortality, due to affecting the skin as well as internal organs. As with other related diseases there is a female predominance and marked clinical diversity. The pathogenesis of SSc is not fully elucidated; it includes endothelial cell injury fibroblast activation and autoimmunity that lead to skin and internal organ manifestations. The majority of cases exhibit characteristic serum autoantibodies. Some of these antibodies are scleroderma-specific reactivities including anti-centromere (ACA), anti-topoisomerase-1 (ATA or Scl 70) or anti-RNA polymerase III antibodies. These anti-nuclear antibody (ANA) patterns are generally mutually exclusive and serve as useful clinical markers of disease subgroups. Additional subsetting of scleroderma cases, based on the extent of skin sclerosis, permits classification into limited and diffuse subsets. Because of the heterogeneity of the disease patients may suffer from different organ manifestations, such as lung fibrosis, hypertensive renal crisis, severe cardiac disease, gastrointestinal involvement, and pulmonary arterial hypertension. Although outcomes have improved recently, systemic sclerosis still has the highest case-specific mortality of any of the autoimmune rheumatic diseases and requires careful and systematic investigation, management and follow-up. Treatment includes symptomatic strategies with attention to each involved organ system; it is still an area where therapeutic progress and better understanding of pathogenesis is increasingly anticipated.