Vasculitis
Philip T. Cagle, Timothy C. Allen, Mary Beth Beasley in Diagnostic Pulmonary Pathology, 2008
MPA is defined as necrotizing vasculitis that involves small vessels and has few or no immune deposits. It is a multisystem disease of middle-aged to older age group, with the vasculitis involving the skin, lungs, and kidney. Other synonyms include systemic necrotizing vasculitis, leukocytoclastic vasculitis, and hypersensitivity vasculitis. Clinical, epidemiologic, and pathologic differences warrant the separation of MPA from polyarteritis nodosa on the basis of absence of small vessel vasculitis in the latter (25). MPA is the most common cause for pulmonary-renal vasculitic syndrome (25). There is often a long prodromal phase with severe constitutional symptoms. Almost all patients develop rapidly progressive pauci-immune necrotizing and crescentic glomerulonephritis. Severe and extensive pulmonary involvement may result in fatal pulmonary hemorrhage. It is associated with P-ANCA antibody in approximately 75% to 80% of patients. Bilateral pulmonary opacities and consolidations may be seen on computed tomograms.
Arteropathies, Microcirculation and Vasculitis
Mary N. Sheppard in Practical Cardiovascular Pathology, 2022
Microscopic polyangiitis (MPA) affects small-sized vessels (i.e. capillaries, venules or arterioles) without granulomas and is a necrotizing small vessel vasculitis with little or absent immune deposits (pauci-immune vasculitis). It does not usually involve larger vessels unlike polyarteritis nodosa. In Western countries, MPA shows a lower prevalence than Wegener's disease, it affects more men than women and usually commences after the age of 50 years. The two organs most typically involved are the kidneys and the lungs. MPA can also involve any other organ especially nerves, the skin, and the musculoskeletal system, heart, eye and the intestines. Its typical clinical manifestations are rapidly progressive glomerulonephritis and alveolar haemorrhage. Capillaritis with fibrinoid necrosis and neutrophilic infiltrate is the most common vascular lesion. MPA is a more appropriate name than microscopic polyarteritis because some patients have no evidence for arterial involvement. The absence or paucity of immunoglobulin localization in vessel walls distinguishes microscopic polyangiitis from immune complex mediated small vessel vasculitis, such as Henoch-Schönlein purpura and cryoglobulinaemic vasculitis. Clinical, epidemiological and pathological differences warrant the separation of microscopic polyangiitis from polyarteritis nodosa on the basis of involvement of capillaries and venules by the former but not the latter. Pauci-immune necrotizing and crescentic glomerulonephritis and haemorrhagic pulmonary capillaritis are common in patients with microscopic polyangiitis.16
Vasculitides
Ayşe Serap Karadağ, Lawrence Charles Parish, Jordan V. Wang in Roxburgh's Common Skin Diseases, 2022
The ELK criteria include ELK manifestations in addition to a positive c-ANCA or typical histopathologic finding qualifies for a diagnosis of GPA. Histologically, pauci-immune necrotizing granulomas are typically visualized in small and medium-sized blood vessels. When purely cutaneous in presentation, the histology may show a neutrophilic infiltrate (Figure 13.10). On immunofluorescence, c-ANCA stains are localized to the cytoplasm whereas multiplied nuclei are non-reactive. Antibodies are directed against PR3.
Clinical spectrum of immunoglobulin A vasculitis in children and determining the best timing of urine examination to predict renal involvement
Published in Postgraduate Medicine, 2022
Fatma Yazılıtaş, Evrim Kargın Çakıcı, Eda Didem Kurt Şükür, Semanur Özdel, Tülin Güngör, Esra Bağlan, Evra Çelikkaya, Deniz Karakaya, Diclehan Orhan, Mehmet Bülbül
Pauci-immune GN, the most common cause of rapidly progressive glomerulonephritis, is known as an antineutrophil cytoplasmic antibody (ANCA) associated vasculitis, may indicate systemic small vessel vasculitides. However, a sub-group of pauci-immune crescentic glomerulonephritis patients continue to appear negative when performing ANCA testing. The patients have severe glomerular lesions and poor renal outcomes despite fewer extra-renal manifestations. Pauci-immune GN is characterized by a lack of significant glomerular immune deposits.9 Similar to C1q nephropathy; the significance of association of pauci-immune GN with IgA vasculitis nephritis is also unclear. There is no data on the importance of IgA vasculitis associated with C1q nephropathy or Pauci-immune GN. Unfortunately, we could not speculate about any underlying pathophysiological role between IgA vasculitis, Pauci-immune GN and C1q nephropathy.
COVID-19: a novel menace for the practice of nephrology and how to manage it with minor devastation?
Published in Renal Failure, 2020
Sena Ulu, Ozkan Gungor, Ebru Gok Oguz, Nuri Baris Hasbal, Didem Turgut, Mustafa Arici
Answer: Based on current evidence, we are not sure. In a series of 701 patients, proteinuria and hematuria were present in 44 and 27% of patients, respectively. 13% of patients presented with GFR < 60 mL/min and 5% with AKI [13]. These presentations all suggested evidence of glomerular injury. In a recent case report, an African American patient with COVID-19 who had a rapid decline in kidney function was reported. Renal biopsy of the patient revealed a collapsing glomerulopathy. In any clinical condition of rapidly progressive GN (RPGN) in COVID-19 infected patients, patients should be evaluated individually in terms of benefits and life-treating side effects. A kidney biopsy is controversial, depends on patients’ clinical conditions. Corticosteroid treatment is skeptical and should be balanced with the worsening of infection control. Plasmapheresis/plasma exchange might be other option instead of high-dose corticosteroids. Again, in a patient with newly diagnosed pauci immune glomerulonephritis and COVID-19, in life-threatening conditions related to vasculitis (vasculitis with lung involvement, lupus nephritis with other organ involvement, etc.), IS treatment should be in mind. Although there is no evident data, IVIG might be used according to infection severity and vasculitis course.
Clinical and pathologic characteristics of pauci-immune anti-myeloperoxidase antibody associated glomerulonephritis with nephrotic range proteinuria
Published in Renal Failure, 2018
Peng-Cheng Xu, Tong Chen, Shan Gao, Shui-Yi Hu, Li Wei, Tie-Kun Yan
No consistent conclusion about the relationship between proteinuria and renal prognosis of AAV has been drawn by previous studies [23–25]. Hauer et al scored the clinical and histological features of 96 patients with renal biopsies and found the proteinuria did not predict the eGFR at 18 months [24], but in that study the patients with immune deposits in renal biopsies were not excluded. Yorioka et al. [25] found that patients with MPO-ANCA GN and did not survive during follow-up had a lower serum albumin and creatinine clearance than those who survived, but there was no difference of urinary protein between two groups. However, only 17 cases were enrolled in this study. Pauci-immune necrotizing crescentic GN is the most common histopathological type of ANCA GN [3–5] and nephrotic range proteinuria is relatively rare. It is worth noting that ANCA GN, especially those with high levels of proteinuria, have been reported to often occur superimposed on other glomerular disease processes which are characterized by glomerular immune deposits [9–17]. In these cases, proteinuria may be potentiated by synergetic effects of the coexisting two types of diseases. To exclude the interference of the potential coexisting other types of GN, only patients with pauci-immune GN were enrolled in this study. We found pauci-immune ANCA GN with nephrotic proteinuria had severe lesions in renal pathology and a high incidence of ESRD.
Related Knowledge Centers
- Goodpasture Syndrome
- Granulomatosis With Polyangiitis
- Immunofluorescence
- Microscopic Polyangiitis
- Vasculitis
- Hypersensitivity
- Acute Proliferative Glomerulonephritis
- Diffuse Proliferative Nephritis
- Eosinophilic Granulomatosis With Polyangiitis
- Anti-Neutrophil Cytoplasmic Antibody