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Renal Vasculitis

Kidney Vasculitis

Renal vasculitis occurs in the setting of autoimmune inflammation of small blood vessels - Wegener granulomatosis, Churg-Strauss syndrome, and microscopic polyangiitis. Rapidly progressive and/or necrotizing glomerulonephritis are the histopathological hallmarks of this condition, with main manifestations being proteinuria and hematuria. The diagnosis requires laboratory workup and biopsy.


Presentation

Renal vasculitis may develop in patients suffering from vasculitis affecting smaller vessels, as is the case in ANCA-associated vasculitides (ANCA means antineutrophil cytoplasmic antibody) [1] [2]. Wegener granulomatosis, microscopic polyangiitis (MPA), and Churg-Strauss syndrome (also known as eosinophilic granulomatosis with polyangiitis) are described as potential causes of renal vasculitis. The clinical presentation of these diseases, although varying in terms of non-renal manifestations, have similar features regarding the kidneys. Hematuria and proteinuria, as well as the presence of white blood cells, casts, and crystals in urine sediment are typical findings [2]. Acute kidney injury (AKI), defined as a rapid decrease in kidney function causing azotemia and electrolyte disbalance, is frequently encountered in patients who develop rapidly progressive glomerulonephritis (RPGN), which is a typical lesion seen in more severe renal injury due to autoimmune mechanisms. In most severe cases, end-stage renal disease (ESRD) due to RPGN may occur in a very short time span, especially in absence of a prompt and accurate diagnosis [2]. Despite the fact that large and medium-sized vessels vasculitic syndromes (such as Takayasu and Kawasaki arteritis, polyarteritis nodosa, and giant cell arteritis) rarely cause glomerulonephritis, these diseases are important causes of renal vasculitis as well [3] [4].

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  • Abstract The associations among human immunodeficiency virus (HIV) infection, focal segmental glomerulosclerosis, and its variant, "collapsing glomerulopathy," often leading to chronic renal failure, are well described.[ncbi.nlm.nih.gov]
Jaundice
  • Abstract Autoimmune pancreatitis (AIP) is an uncommon condition which comprises diffuse or discrete pancreatic enlargement and irregular pancreatic duct strictures of autoimmune origin leading to pain or obstructive jaundice associated with extra-pancreatic[ncbi.nlm.nih.gov]
Hypertension
  • Our results demonstrated that more hypertensive patients and higher BVAS were found in patients with AKI-F than those in other groups (p CONCLUSIONS: The RILFE criteria is a valid measurement of both prognosis and progression in patients with AASV.[ncbi.nlm.nih.gov]
  • The clinical expression of renal vasculitis depends on the size of the affected vessels and includes renovascular hypertension, isolated nonnephrotic proteinuria, interstitial nephritis, and glomerulonephritis, which can be rapidly progressive.[ncbi.nlm.nih.gov]
  • Percutaneous angioplasty in clinical management of renovascular hypertension: initial and long-term results. ‏[books.google.com]
  • Current Opinion in Nephrology and Hypertension: March 1993 Clinical nephrology: PDF Only Abstract During most of the 20th century, the medical literature on systemic vasculitis has been dominated by “splitters,” who sought to characterize the precise[journals.lww.com]
  • The prevalence of hypertension in cases of Takayasu’s arteritis was lower in our dataset than in other studies ( 2 , 9 ).[tidsskriftet.no]

Workup

To make the diagnosis of vasculitis, it is necessary to obtain a thorough patient history and conduct a detailed physical examination. If hematuria is noted, parameters of kidney function - creatinine, blood urea nitrogen (BUN), and serum electrolytes should be evaluated [5]. Furthermore, determination of proteinuria, as well as the presence of red and white blood cells, casts, and crystals can be achieved by simple urine testing and are valid indicators of glomerular damage [5]. Detection of anti-neutrophil cytoplasmic antibodies (both perinuclear and cytoplasmic, or p-ANCA and c-ANCA, respectively) in serum may be of a great help in making the diagnosis of small vessel vasculitis, but large titers are observed in all cases of ANCA-associated vasculitides, necessitating additional procedures to confirm the underlying disease [5]. Ultrasonography, computed tomography (CT), and magnetic resonance imaging (MRI) can be used as adjunctive procedures, but the use of a contrast needs to be avoided, as further damage to the kidneys may occur [5]. Fluorodeoxyglucose-positron emission tomography/computed tomography (FDG-PET/CT) is an important imaging study if solid clinical suspicion of vasculitis exists, mainly because it shows which organs are affected by vasculitis [2]. To confirm the diagnosis, the kidney biopsy is the gold standard [2]. Segmental fibrinoid necrosis, an absence of immune deposits in the glomeruli (as seen in pauci-immune glomerulonephritis), and the presence of glomerular crescents are frequently present in ANCA-associated glomerulonephritis [2] [3].

Creatinine Decreased
  • Mean values of serum anti-MPO, disease activity markers and serum creatinine decreased when these values were compared from pre-therapy to the time of switching to MMF, and then to the end of the study anti-MPO: 204 A /- 144 U, 54 A /- 85 U and 12 A [ncbi.nlm.nih.gov]

Treatment

  • CONCLUSION: Considering the balance between suppression of disease activity and adverse effects of treatment, mizoribine may be useful for preemptive treatment for patients with ANCA-associated renal vasculitis at high risk for relapse.[ncbi.nlm.nih.gov]
  • Rituximab-based maintenance treatment is superior to standard treatment with azathioprine. The use of more targeted treatment will hopefully be translated into a better long-term outcome of AAV patients. Copyright 2013 S. Karger AG, Basel.[ncbi.nlm.nih.gov]
  • Older patients respond less well to treatment and have a poorer prognosis.[ncbi.nlm.nih.gov]
  • […] for maintenance treatment.[ncbi.nlm.nih.gov]
  • However, it is unknown whether disease presentation and response to treatment differs from younger patients. We aimed to examine the presentation, response to treatment and outcome of patients over 65 yr of age compared with a younger cohort.[ncbi.nlm.nih.gov]

Prognosis

  • Few studies have been published focusing on AASV patients with poor prognosis.[ncbi.nlm.nih.gov]
  • CONCLUSIONS: The manifestations and prognosis of renal vasculitis range widely. Renal involvement greatly influences prognosis and dictates the need for early and prompt immunosuppressive therapy.[ncbi.nlm.nih.gov]
  • This study further highlights the importance of renal function on prognosis and the need for less toxic treatment regimens.[ncbi.nlm.nih.gov]
  • Moreover, anaemia severity was significantly associated with the degree of renal dysfunction and life prognosis.[ncbi.nlm.nih.gov]
  • These measures should improve the prognosis of patients with PRV.[ncbi.nlm.nih.gov]

Etiology

  • According to previous reports from all over the world, the etiology, prevalence, and prognosis of RPGN including ANCA-associated renal vasculitis varies among races and periods.[ncbi.nlm.nih.gov]
  • Over the past three decades, tremendous progress has been made through investigative efforts to understand etiologic, pathogenetic and clinical underpinnings of these disorders.[uncch.pure.elsevier.com]
  • Etiologies and outcome of acute renal insufficiency in older adults: a renal biopsy study of 259 cases. Am J Kidney Dis 2000; 35: 433-447. 6. Pascual J, Liaño F, Ortuño J. The elderly patient with acute renal failure.[uninet.edu]
  • Etiology Pulmonary-renal syndrome is less commonly a manifestation of immunoglobulin A (IgA)-mediated disorders, such as IgA nephropathy or IgA–associated vasculitis , and of immune complex–mediated renal disease, such as essential mixed cryoglobulinemia[merckmanuals.com]
  • Other testing for alternative etiologies such as infections or other diagnoses should be driven by clinical suspicion.[rheumatologyadvisor.com]

Epidemiology

  • Epidemiologic picture of Kawasaki disease in Beijing from 1995 through 1999. Pediatr Infect Dis J. 2002;21(2):103-7. Mahr A, Guillevin L, Poissonnet M, Aymé S.[revistanefrologia.org]
  • BACKGROUND: The epidemiology of renal vasculitis in different populations is poorly understood.[ncbi.nlm.nih.gov]
  • Clinical and epidemiological research Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis: 2-year results of a randomised trial Rachel B Jones 1 , Shunsuke Furuta 1 , Jan Willem Cohen Tervaert 2 , Thomas Hauser 3 , Raashid Luqmani 4[ard.bmj.com]
  • Textbook of Systemic Vasculitis is an in-depth guide to the epidemiology, classification and genetics of systemic vasculitis as well as imaging, symptoms and management of the disease.[books.google.com]
  • Epidemiology of the vas-culitides. Semin Respir Crit Care Med 2004; 25:455-64. 8. Puéchal X. Antineutrophil cytoplasmic antibody-associated vasculitis. Rev Rhum 2007;74:824-32. 9. Pagnoux C, Mahr A, Guillevin L.[sjkdt.org]
Sex distribution
Age distribution

Pathophysiology

  • We review clinical features, advances in therapy and understanding of pathophysiology. 2010 Elsevier B.V. All rights reserved.[ncbi.nlm.nih.gov]
  • Both the innate and the acquired immune system are involved in the pathophysiology of renal vasculitis.[ncbi.nlm.nih.gov]
  • Hypersensitivity Vasculitis: Background, Pathophysiology, Epidemiology. Patel, M et al. Medscape. Available online at through . Accessed August 2015.[labtestsonline.org]
  • CLASSIFICATION CONFUSING and UNSATISFYING DUE TO 1.OVERLAPPING PRESENTATIONS 2.MIX OF PRIMARY AND SECONDARY FORMS 3.EVOLVING UNDERSTANDING OF PATHOPHYSIOLOGY 4.EVOLVING CONSENSUS REGARDING USE OF EPONYMS 5.VARIED OPINION ON WHETHER CLASSIFCATION SHOULD[slideshare.net]

Prevention

  • The use of cotrimoxazole is likely to be beneficial to prevent relapse of vasculitis. AZA is effective as maintenance therapy once remission has been achieved.[ncbi.nlm.nih.gov]
  • Thus, PRV should be identified at an early stage and the treatment protocol should prevent infectious complications. These measures should improve the prognosis of patients with PRV.[ncbi.nlm.nih.gov]
  • Rituximab therapy was started, but it was discontinued after the third dose to minimize the risk of systemic spread of a severe oral Candida infection and to prevent superinfections that were facilitated by leukopenia.[ncbi.nlm.nih.gov]
  • Their clinical work informs and stimulates the research to define the basis of disease and potential new less toxic therapies that may prevent and treat crescentic glomerulonephritis.[monash.edu]
  • We use preventive approaches to help avoid kidney injury and disease, along with advanced therapies to improve kidney health and function.[umiamihealth.org]

References

Article

  1. Walters G, Willis NS, Craig JC. Interventions for renal vasculitis in adults. Cochrane Database Syst Rev. 2015;9:CD003232.
  2. Rowaiye OO, Kusztal M, Klinger M. The kidneys and ANCA-associated vasculitis: from pathogenesis to diagnosis. Clin Kidney J. 2015;8(3):343-350.
  3. John R, Herzenberg AM. Vasculitis affecting the kidney. Semin Diagn Pathol. 2009;26(2):89-102.
  4. Maritati F, Iannuzzella F, Pavia MP, Pasquali S, Vaglio A. Kidney involvement in medium- and large-vessel vasculitis. J Nephrol. 2016;29(4):495-505.
  5. Porter RS, Kaplan JL. Merck Manual of Diagnosis and Therapy. 19th Edition. Merck Sharp & Dohme Corp. Whitehouse Station, N.J; 2011.

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Last updated: 2018-06-22 08:46