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1
Kwa, Vincent Ing Han. Clinical and magnetic resonance observations in cerebral small-vessel disease. [s.n.], 1999.
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2
P, Pullicino, Caplan Louis R, and Hommel Marc, eds. Cerebral small artery disease. Raven Press, 1993.
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3
Gorelick, Philip B., and Leonardo Pantoni. Cerebral Small Vessel Disease. Cambridge University Press, 2014.
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4
Gorelick, Philip B., and Leonardo Pantoni. Cerebral Small Vessel Disease. Cambridge University Press, 2014.
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5
Gorelick, Philip B., and Leonardo Pantoni. Cerebral Small Vessel Disease. Cambridge University Press, 2014.
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8
Gunda, Bence. Multimodal MRI of Cerebral Small Vessel Disease. INTECH Open Access Publisher, 2012.
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9
Charidimou, Andreas, Eric Jouvent, and Susanne J. Van Veluw, eds. Cerebral Small Vessel Diseases: From Vessel Alterations to Cortical Parenchymal Injury. Frontiers Media SA, 2020. http://dx.doi.org/10.3389/978-2-88963-587-0.
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Brown, Christina, and Jamie E. Rubin. Moyamoya Disease. Edited by Kirk Lalwani, Ira Todd Cohen, Ellen Y. Choi, and Vidya T. Raman. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780190685157.003.0032.
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Moyamoya is a rare cerebrovascular disorder characterized by progressive stenosis of the large cerebral arteries. Cerebral ischemia is the most common manifestation in the pediatric population and may present with symptoms of headache, hypertension, blindness, and developmental delay. The gold standard for diagnosis of Moyamoya is digital subtraction angiography but less invasive imaging modalities such as magnetic resonance imaging/angiogram or perfusion magnetic resonance imaging are typically used. Progressive disease is usually treated with surgical revascularization. Indirect revascularization procedures are preferred to direct revascularization in children due to small caliber of vessels, but regardless of the surgical procedure performed, patients often have some degree of long-term neurological impairment after surgery. Maintenance of cerebral perfusion pressure is critical during the perioperative period, and the patient must be monitored closely for signs of cerebral ischemia.
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Chong, Ji Y., and Michael P. Lerario. Small Vessel Disease. Oxford University Press, 2016. http://dx.doi.org/10.1093/med/9780190495541.003.0010.
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Lacunar strokes are strongly associated with hypertension. Long-term blood pressure management is important after lacunar stroke. Antiplatelet therapy should be instituted for secondary stroke prevention.
12
Gonzalez-Quevedo, Alina. Understanding and Treating Small Vessel Disease. Nova Science Publishers, Incorporated, 2020.
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13
Gonzalez-Quevedo, Alina. Understanding and Treating Small Vessel Disease. Nova Science Publishers, Incorporated, 2020.
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14
(Editor), Patrick M. Pullicino, Louis R. Caplan (Editor), and Marc Hommel (Editor), eds. Cerebral Small Artery Disease (Advances in Neurology). Lippincott Williams & Wilkins, 1993.
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15
Irwin, Samuel Terence. Physiological studies on blood flow in the diabetic foot (with special reference to small vessel disease). 1986.
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16
Cui, Zhao, Neil Turner, and Ming-hui Zhao. Antiglomerular basement membrane disease. Edited by Neil Turner. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780199592548.003.0072_update_001.
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Antiglomerular basement membrane (anti-GBM) disease may present as rapidly progressive glomerulonephritis alone, or in the presence of a secondary pulmonary insult (e.g. smoking or other toxicity, or infection) in combination with lung haemorrhage. Rarely it presents as lung disease alone (with haematuria) or as subacute glomerulonephritis. The major differential diagnoses are small vessel vasculitis, which is a more common cause of pulmonary haemorrhage with rapidly progressive glomerulonephritis, and causes of simultaneous pulmonary and renal failure. For most of these, the lung lesion is not pulmonary haemorrhage. The diagnosis often most quickly, most sensitively, specifically and usefully made by renal biopsy, but immunoassays showing a high titre of anti-GBM antibodies in the setting of severe renal disease are also useful. Borderline and even normal anti-GBM titres are not so specific or reliable in some forms of the disease though.
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Cui, Zhao, Neil Turner, and Ming-hui Zhao. Antiglomerular basement membrane disease. Edited by Neil Turner. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780199592548.003.0073_update_001.
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Cyclophosphamide and plasma exchange are the standard of care in rapidly progressive glomerulonephritis or lung haemorrhage caused by antiglomerular basement membrane (anti-GBM) disease, and it is unusual to encounter patients at earlier stages. Steroids are universally used in addition. There is some evidence that plasma exchange may not be a critical part of treatment at an earlier stage. There is no more than anecdotal evidence for other therapies. Slower-onset therapies such as antibodies to B cells are rarely appropriate. If untreated, patients with severe anti-GBM disease will not recover renal function and are at risk of pulmonary haemorrhage. Evidence for the pathogenicity of circulating anti-GBM antibodies provides rationale for removal of circulating antibodies as rapidly as possible, whilst simultaneously inhibiting their synthesis. This was behind the introduction of the combination of plasma exchange with immunosuppressive therapy in mid 1970s, which revolutionized outcomes. Plasmapheresis aims to remove circulating pathogenic antibodies against GBM and possibly other mediators; cyclophosphamide prevents further synthesis of autoantibodies; and steroids act as anti-inflammatory agents to attenuate the glomerular inflammatory response initiated by anti-GBM antibodies. It is clear from experimental models and occasional observations in man that the anti-cell mediated effects of current therapies are important too. Outcomes vary, but in general patient survival is now good, while renal survival remains poor, in many series less than 50% at 1 year. Treatment is toxic and after an early peak in deaths due to pulmonary haemorrhage, secondary infections are the next threat. It may therefore be best not to immunosuppress patients with a very poor renal prognosis who appear to be at low risk of pulmonary haemorrhage. Treatment can usually be curtailed after 3 months without recurrence. ANCA and anti-GBM antibodies occur together in some patients. This is typically an older group which often has features of vasculitis, and the anti-GBM response may often be secondary. Longer treatment as for small vessel vasculitis is usually indicated.
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Hajhosseiny, Reza, Kaivan Khavandi, and David J. Goldsmith. Sudden cardiac death in chronic kidney disease. Edited by David J. Goldsmith. Oxford University Press, 2015. http://dx.doi.org/10.1093/med/9780199592548.003.0108.
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Epidemiological data demonstrate the unique vulnerability of chronic kidney disease (CKD) subjects to cardiovascular disease, the most catastrophic being sudden cardiac death (SCD). In patients with declining kidney function there is a continuum of cardiovascular risk. In those individuals who survive to reach end-stage renal disease (ESRD), the risk of suffering a cardiac event is extremely high. Some of this risk is explained by the common risk factors and traditional cardiovascular events, namely atherosclerotic plaque fissure and rupture, but there is now evidence of a distinct ‘later CKD’ mechanism, notably arrhythmias. This appears particularly true in later stages of CKD and corresponds with the multifaceted range of myocardial and vascular insults operating. The physiological milieu of disordered vessel autoregulation, sequestered vasoprotective agents, loss of conduit and small artery elasticity/compliance, a stiffened and fibrotic myocardium, with calcified and diseased coronary arteries, all within an inflammatory environment, all contribute to arrhythmia generation. The final insult is changes in volume and electrolyte status. Risk stratification tools would be helpful in guiding clinicians to recognize those subjects likely to benefit from specific interventional strategies. These might include the novel, or emerging serum, haemodynamic, or electrocardiographic biomarkers in CKD. Current tools—such as those used for stratifying risk for SCD and determining the need for ICD implantation—are not valid in ESRD patients. Beta blockers appear likely to be generally advisable, blood pressure permitting, for patients with significant cardiomyopathy. Evidence for implantable cardiac defibrillators (ICD) is lacking. There is good reason to think that young dialysis patients at high risk of sudden death may benefit, but the risk/benefit ratio for older patients is less likely to be advantageous. These hypotheses need further investigation.
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Patarroyo, Sully Xiomara Fuentes, and Craig Anderson. Management of ischaemic stroke. Oxford University Press, 2016. http://dx.doi.org/10.1093/med/9780199600830.003.0236.
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Ischaemic stroke is the most common cause of stroke around the world. It is a complex disease with a range of causes, manifestations, outcomes, and treatments. As the therapeutic time window to rescue or ‘protect’ the brain from ischaemic damage is extremely short, effective treatment requires coordinated systems of care, which commence in the prehospital paramedical setting and continue through the emergency department into the critical care environment, neurology ward, rehabilitation, and re-settlement back home. Successful outcomes from ischaemic stroke can be achieved through the effective use of thrombolytic therapy to re-canalize an occluded vessel and re-perfuse the ‘at risk’ area of the brain. Other aspects of management include the prevention of complications of the neurological (cerebral) disability, timely introduction of rehabilitation, realistic goal-setting towards satisfactory recovery, and secondary prevention measures to reduce the high risk of recurrent stroke and other serious vascular events.
20
Tombetti, Enrico, and Justin C. Mason. Pathophysiology of vasculitis. Oxford University Press, 2017. http://dx.doi.org/10.1093/med/9780198755777.003.0017.
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Vasculitis represents a spectrum of disorders that are often divided on the basis of the predominant vessel size affected into large-, medium- and small-vessel vasculitides. This chapter will focus on the pathogenesis of the anti-neutrophil cytoplasmic antibody (ANCA)-associated medium- and small-vessel vasculitides (AAV), and large-vessel vasculitis, Takayasu arteritis, and giant cell arteritis. Underlying pathogenic mechanisms in vasculitis remain to be fully understood. In particular, the initiating event(s) are not known. A combination of infectious or other environmental triggers on a susceptible genetic background is currently favoured. In addition to the vessel size affected, the mechanisms of vascular injury vary. Moreover, extravascular granulomatosis may play an important role in disease manifestations. The innate and adaptive immune systems contribute to its pathogenesis. Although pathogenic antibodies have not been identified in large-vessel vasculitis, ANCA are directly implicated in small- and medium-vessel AAV. Disease manifestations are varied and diverse and may include arterial stenosis or aneurysms, glomerulonephritis and renal failure, gastro-intestinal, pulmonary, cutaneous, and neurological complications, visual disturbance, deafness, and nasal bridge collapse. Life-threatening cardiovascular disease is also seen, with myocarditis, pericarditis, valvular heart disease, thrombosis, systemic and pulmonary arterial hypertension, and accelerated coronary heart disease all reported. Despite this, the prognosis for patients with vasculitis has improved significantly in recent decades. Further understanding of the pathogenesis of vasculitis will lead to the discovery of further therapeutic targets and novel, safer biologic therapies.
21
Markus, Hugh, Anthony Pereira, and Geoffrey Cloud. Ischaemic stroke: common causes. Oxford University Press, 2017. http://dx.doi.org/10.1093/med/9780198737889.003.0008.
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This chapter on common causes of ischaemic stroke reviews the major pathologies underlying ischaemic stroke, namely large-artery disease, cardioembolism, and small-vessel disease. Large-vessel extra- and intracranial atherosclerotic cerebrovascular disease is covered. Cardioembolic aetiologies of stroke including atrial fibrillation and valvular heart disease are discussed. Small-vessel disease causing lacunar stroke and possible heterogonous pathologies underlying this subtype are covered. Dolichoectasia of arteries as a potential cause of stroke and the newer concept of embolic stroke of undetermined source are also discussed.
22
Curry, Nicola, and Raza Alikhan. Normal platelet function. Edited by Patrick Davey and David Sprigings. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780199568741.003.0281.
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The platelet is a small (2–4 µm in diameter), discoid, anucleate cell that circulates in the blood. In health, it plays a vital role in haemostasis, and in disease it contributes to disorders of bleeding and thrombosis. Platelets are produced from the surface of megakaryocytes in the bone marrow, under tight homeostatic control regulated by the cytokine thrombopoietin. Platelets have a lifespan of approximately 7–10 days, and usually circulate in the blood stream in a quiescent state. Intact, undamaged vessel walls help to maintain platelets in this inactive state by releasing nitric oxide, which acts both to dilate the vessel wall and to inhibit platelet adhesion, activation, and aggregation. After trauma to the blood vessel wall, platelets are activated and, acting in concert with the endothelium and coagulation factors, form a stable clot. This chapter addresses platelet structure and function, and the response of platelets to vessel injury.
23
Sudhir, Rajini. Pulmonary vasculitis. Edited by Patrick Davey and David Sprigings. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780199568741.003.0140.
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Pulmonary vasculitis comprises a heterogeneous group of disorders characterized by an inflammatory process damaging the vessel wall, leading to ischaemia and tissue necrosis. Wegener’s granulomatosis, Churg–Strauss syndrome, and microscopic polyangiitis are primary, small-vessel, necrotizing vasculitides linked by an overlapping clinicopathological picture and are referred to collectively as ANCA-associated systemic vasculitis. The European Vasculitis Study Group proposed a clinical staging system based on disease activity, to guide treatment.
24
Salama, Alan D. The patient with vasculitis. Edited by Giuseppe Remuzzi. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780199592548.003.0159_update_001.
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Primary systemic vasculitis frequently leads to renal involvement and is responsible for significant numbers of patients progressing to end-stage renal disease. Frequently this is due to small vessel vasculitis, in association with antineutrophil cytoplasm antibody, which requires prompt recognition and timely therapeutic intervention to optimize renal and patient outcomes. Other organ systems are often affected. Relapses occur in about 50%.Less commonly medium or larger vessel vasculitis may involve the kidneys and through ischaemia lead to impaired renal function and renovascular hypertension, as in Takayasu’s or Kawasaki disease, and polyarteritis nodosa (PAN).
25
Ferro, José M., and Ana Catarina Fonseca. Secondary prevention. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780198722366.003.0015.
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There are no specific guidelines regarding secondary stroke prevention in young adult stroke patients. Recommendations for secondary prevention are mainly extrapolated from data obtained from older individuals, because young adults were excluded or under-represented in most secondary stroke prevention clinical trials. Secondary stroke prevention includes (a) screening and control of vascular risk factors, that is, hypertension, diabetes mellitus, hyperlipidaemia, atrial fibrillation, hormonal contraception, infections, trauma, physical inactivity, obesity, poor nutrition, smoking, alcohol, and illicit drug use; and (b) identification and treatment of specific causes of ischaemic stroke, that is, cardioembolism, large vessel extra- and intracranial atherosclerotic disease, small vessel disease, dissection, antiphospholipid syndrome, moyamoya disease, sickle cell disease, and some rare diseases. There is then an opportunity for lifelong prevention of vascular events after stroke in a young adult.
26
Alchi, Bassam, and David Jayne. The patient with antiphospholipid syndrome with or without lupus. Edited by Giuseppe Remuzzi. Oxford University Press, 2015. http://dx.doi.org/10.1093/med/9780199592548.003.0164.
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Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by recurrent arterial or venous thrombosis and/or pregnancy loss, accompanied by laboratory evidence of antiphospholipid antibodies (aPL), namely anticardiolipin antibodies (aCL), lupus anticoagulant (LA), and antibodies directed against beta-2 glycoprotein 1 (β2GP1). APS may occur as a ‘primary’ form, ‘antiphospholipid syndrome,’ without any known systemic disease or may occur in the context of systemic lupus erythematosus (SLE), ‘SLE-related APS’. APS may affect any organ system and displays a broad spectrum of thrombotic manifestations, ranging from isolated lower extremity deep vein thrombosis to the ‘thrombotic storm’ observed in catastrophic antiphospholipid syndrome. Less frequently, patients present with non-thrombotic manifestations (e.g. thrombocytopaenia, livedo reticularis, pulmonary hypertension, valvular heart disease, chorea, and recurrent fetal loss).The kidney is a major target organ in both primary and SLE-related APS. Renal involvement is typically caused by thrombosis occurring at any location within the renal vasculature, leading to diverse effects, depending on the size, type, and site of vessel involved. The renal manifestations of APS include renal artery stenosis and/or renovascular hypertension, renal infarction, APS nephropathy (APSN), renal vein thrombosis, allograft vasculopathy and vascular thrombosis, and thrombosis of dialysis access.Typical vascular lesions of APSN may be acute, the so-called thrombotic microangiopathy, and/or chronic, such as arteriosclerosis, fibrous intimal hyperplasia, tubular thyroidization, and focal cortical atrophy. The spectrum of renal lesions includes non-thrombotic conditions, such as glomerulonephritis. Furthermore, renal manifestations of APS may coexist with other pathologies, especially proliferative lupus nephritis.Early diagnosis of APS requires a high degree of clinical suspicion. The diagnosis requires one clinical (vascular thrombosis or pregnancy morbidity) and at least one laboratory (LA, aCL, and/or anti-β2GP1) criterion, positive on repeated testing.The aetiology of APS is not known. Although aPL are diagnostic of, and pathogenic in, APS, a ‘second hit’ (usually an inflammatory event) may trigger thrombosis in APS. The pathogenesis of the thrombotic tendency in APS remains to be elucidated, but may involve a combination of autoantibody-mediated dysregulation of coagulation, platelet activation, and endothelial injury.Treatment of APS remains centred on anticoagulation; however, it has also included the use of corticosteroids and other immunosuppressive therapy. The prognosis of patients with primary APS is variable and unpredictable. The presence of APS increases morbidity (renal and cerebral) and mortality of SLE patients.
27
Fromm, Annette. Vascular aetiology. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780198722366.003.0004.
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Vascular aetiology of young ischaemic stroke covers a broad spectrum of causes. It includes the risk factor-mediated causes considered more common among the elderly on one hand, and a large number of rather rare disorders and conditions typical for younger ages on the other hand. This chapter is focused on atherosclerotic aetiology and comorbidity, small vessel disease and arterial dissection, which account for a majority of young ischaemic strokes worldwide, are treatable, and need to be considered as overall or contributing causes early during investigation. Specific and rare causes of young ischaemic stroke will be presented elsewhere.
28
Jayne, David. The patient with vasculitis. Edited by Giuseppe Remuzzi. Oxford University Press, 2015. http://dx.doi.org/10.1093/med/9780199592548.003.0157.
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The definition of vasculitis syndromes relies on clinical, serological, and pathological descriptions. A number of primary syndromes are recognized, of presumed autoimmune aetiology, but vasculitis may also occur as a secondary disease process. The heterogeneity of clinical presentation, low specificity of many clinical features, and mimics of other diseases complicate vasculitis diagnosis. The kidney is the most common severe manifestation for small vessel vasculitis syndromes and the severity of renal involvement predicts end-stage renal failure risk and death. Suspicion of vasculitis is key to early diagnosis and improving outcomes of vasculitis patients.The current understanding of the classification of vasculitis syndromes is presented in this chapter along with descriptions of clinical presentations, and approaches to diagnosis.
29
Woywodt, Alexander, and Diana Chiu. Drug-induced and toxic glomerulopathies. Edited by Neil Turner. Oxford University Press, 2015. http://dx.doi.org/10.1093/med/9780199592548.003.0082.
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Glomerulopathies induced by particular exogenous compounds or molecules include those attributable to toxicity, and those caused by inducing an immune or autoimmune response. Tubules are more commonly the target of toxicity as they absorb and concentrate components of filtrate. Damage to endothelial cells may account for thrombotic microangiopathy in response to calcineurin inhibitors. Endothelial cells are also likely to be the target in drug-induced small vessel vasculitis. Toxicity to podocytes accounts for focal segmental glomerulosclerosis caused by pamidronate and other agents. Chloroquine can cause a remarkable pseudo-storage disorder with inclusions in podocytes that resemble those seen in Fabry disease. The mechanism by which drugs cause minimal change disease, another podocyte disorder, is not known. Membranous nephropathy may be caused by exposure to gold, mercury, and some other drugs; this is antibody mediated and presumably the targets are altered podocyte surface molecules. Inhibitors of the mammalian target of rapamycin (mTOR) cause proteinuria, possibly through effects on vascular endothelial growth factor, inhibitors of which are associated with not only proteinuria (an expected podocyte effect) but also thrombotic microangiopathy (endothelial cell effect). This latter may be through disturbing podocyte-endothelium cross-signaling.
30
Lai, Kar Neng, and Sydney C. W. Tang. Immunoglobulin A nephropathy. Edited by Neil Turner. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780199592548.003.0066_update_001.
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Immunoglobulin A (IgA) nephropathy characteristically causes haematuria and may present as a nephritic illness in older children and young adults. However, it may occur at any age and is commonly asymptomatic, associated first with haematuria alone, later progressing in some patients to hypertension, proteinuria, and progressive loss of glomerular filtration. While this evolution is characteristically slow, over decades, in some it is rapid, leading to early end-stage renal failure. It is common for the disease to present late, as advanced renal disease, or malignant hypertension. It may present with acute kidney injury caused by crescentic disease, but acute kidney injury caused by haematuria may be confused clinically with the same. Henoch–Schönlein purpura is a type of small vessel vasculitis that is most commonly seen in children, but which occurs at all ages, that is associated with IgA deposition. In older children and most adults it merges closely into IgA nephropathy after the acute event. Outcomes in adults are less good. IgA nephropathy is the most common type of glomerulonephritis in most developed countries. The disease is more common in men, and appears to be much less common in black people. The detected incidence is strongly influenced by biopsy policies; the lower your threshold to biopsy patients with haematuria, the more of this condition you discover. There are clear genetic tendencies but the strongest risk seems to come from genes in the human leucocyte antigen complex.
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Turner, Neil. Crescentic (rapidly progressive) glomerulonephritis. Edited by Neil Turner. Oxford University Press, 2015. http://dx.doi.org/10.1093/med/9780199592548.003.0070.
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Crescent formation refers to the appearance of proliferating cells in Bowman’s space in response to severe glomerular inflammation. Any aggressive ‘nephritic’ diseases that cause basement membrane breaks may provoke this. Specific serum proteins appear to be responsible for provoking crescent formation as it is largely abolished by defibrination in animal models. The cells in the crescent are initially mostly hypertrophying and proliferating parietal epithelial cells that normally line Bowman’s capsule. Foci of proliferation of these cells (extracapillary proliferation) are the first steps of crescent formation. Monocytes are frequently seen in established crescents. At this stage recovery of glomerular structure and function is possible in many circumstances. However, if Bowman’s capsule is ruptured, fibroblast ingress followed by fibrosis and glomerulosclerosis are likely. Crescentic nephritis is a histological description, but it fits closely with the clinical picture of rapidly progressive glomerulonephritis (RPGN), in which renal function is lost over days to weeks. The diseases most likely to cause this clinical picture are small vessel vasculitis, anti-GBM disease, lupus nephritis, and post-infectious glomerulonephritis. Any ‘nephritic’ disease may provoke crescent formation, but it is frequently encountered in immunoglobulin A nephropathy/Henoch–Schönlein purpura, and in post-infective glomerulonephritis. Recognizing the clinical picture is important as aggressive immunosuppression can be effective in saving glomerular function in some of the conditions causing it.
32
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.