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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].

Collapse
  • 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]
  • Rheumatology (Oxford) 40 : 831 – 832, 2001 Major GA, Moore PG: Profound circulatory collapse due to azathioprine. J R Soc Med 78 : 1052 – 1053, 1985 Cunningham T, Barraclough D, Muirden K: Azathioprine-induced shock.[cjasn.asnjournals.org]
Pathologist
  • Biopsies were assessed by two independent pathologists, blinded to patient data. Clinical data were collected retrospectively. RESULTS: Patients were followed-up for a median of 189 months from diagnosis.[ncbi.nlm.nih.gov]
  • […] groups with similar renal insufficiency, older patients were more likely to progress to end-stage renal failure ( P .039), survival was worse ( P .016), and death occurred earlier. 7 HISTOPATHOLOGIC CLASSIFICATION An international working group of renal pathologists[mdedge.com]
Jaundice
  • 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 manifestations[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]
  • 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]
Withdrawn
  • Immunosuppressive therapy was subsequently withdrawn. At the last follow-up visit, 12 months after the second MSC infusion, the patient remained in clinical remission without any therapy.[ncbi.nlm.nih.gov]
  • Relapse usually occurs when immunosuppressive treatment is decreased or withdrawn. Relapse may occur on dialysis and less frequently in transplanted patients. If vasculitis relapses, treatment should be restarted.[uninet.edu]
  • Maintenance therapy continued for 12 months then withdrawn over 3 months. Trimethoprim-sulfamethoxazole 1600/320 daily recommended for WG patients for additional 2 years.[bmcnephrol.biomedcentral.com]
Oliguria
  • A 73-year-old man was admitted to the hospital for malaise, weight loss, and oliguria.[ncbi.nlm.nih.gov]
  • In conclusion, elderly patients with AAV have more acute renal failure, more associated renal disease, worse creatinine levels, more oliguria and less extrarenal clinical manifestations than younger patients at the time of presentation of vasculitis.[uninet.edu]
  • Renal manifestations at TOD included: hematuria (92%), proteinuria (84%), hypertension (29%), oliguria (20%), and nephrotic syndrome (14%). 29 patients (27%) required dialysis at TOD.[acrabstracts.org]
Lethargy
  • Abstract We report the case of a 62-year-old woman with rheumatoid arthritis treated with adalimumab, an anti-tumor necrosis factor alpha drug, who presented with 4 weeks of lethargy, upper respiratory tract symptoms, a vasculitic skin rash, and rapidly[ncbi.nlm.nih.gov]
  • CASE REPORT A 7-year-old girl in good health presented with confusion, lethargy, and right-sided weakness for 2 hours on the day of admission.[pediatrics.aappublications.org]

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].

Microalbuminuria
  • Typical findings of diabetic nephropathy are early hyperfiltration followed by microalbuminuria and overt proteinuria, resulting in a progressive decrease in glomerular filtration rate.[ncbi.nlm.nih.gov]
  • Eight patients had either a normal protein–creatinine ratio or microalbuminuria, 17 had proteinuria and 11 had nephrotic proteinuria. Estimated glomerular filtration rate.[tidsskriftet.no]
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]
Candida
  • 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]

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]

Prognosis

  • Few studies have been published focusing on AASV patients with poor prognosis.[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]
  • 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]
  • Other testing for alternative etiologies such as infections or other diagnoses should be driven by clinical suspicion.[rheumatologyadvisor.com]
  • Often biopsy of the gastrointestinal tract will be required to establish the vasculitic etiology of gastrointestinal ulcers from those caused by other processes including glucocorticoid therapy.[renalandurologynews.com]
  • Although the etiology is not always known, the incidence of vasculitis is increasing, and the diagnosis and management of patients may be challenging because of its relative infrequency, changing nomenclature, and variability of clinical expression.[aafp.org]

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, Matthew[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 vasculitides. Curr Opin Rheumatol 2003 ; 15 : 11 –16 3 Hoffman GS, Kerr GS, Leavitt RY et al. Wegener granulomatosis: an analysis of 158 patients.[academic.oup.com]
Sex distribution
Age distribution

Pathophysiology

  • 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]
  • 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: 2019-07-11 21:42