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Vasculitides
Published in Ayşe Serap Karadağ, Lawrence Charles Parish, Jordan V. Wang, Roxburgh's Common Skin Diseases, 2022
Ivy M. Obonyo, Virginia A. Jones, Kayla A. Clark, Maria M. Tsoukas
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.
Vasculitis
Published in Philip T. Cagle, Timothy C. Allen, Mary Beth Beasley, 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.
Vasculitis induced by drugs
Published in Philippe Camus, Edward C Rosenow, Drug-induced and Iatrogenic Respiratory Disease, 2010
Michiel De Vries, Marjolein Drent, Jan-Wil Cohen Tervaert
Microscopic polyangiitis (MPA) is another form of ANCA-associated systemic necrotizing vasculitis that histologically affects small vessels without granulomatous formation. Although MPA was originally considered a subclass of polyarteritis nodosa (PAN), the 1994 Chapel Hill consensus conference set a classification framework that differentiated PAN from MPA on the basis of the presence of vasculitis in small vessels. Most of the patients have MPO-ANCA, whereas in the remaining patients PR3 ANCA is found. Age at onset is 40–60 years. MPA seems more common in men than in women. Patients present with variable signs and symptoms such as microscopic haematuria and proteinuria, palpable purpura, abdominal pain, cough and haemoptysis. Histology reveals pauci-immune necrotizing glomerulonephritis with crescent formation and sometimes vasculitis involving arterioles and/or small interlobular arteries. Granulomatous lesions of the upper or lower respiratory tract are not found.43 The kidney is the most commonly affected organ in 90 per cent of the patients.44 Pulmonary involvement is seen in 30–50 per cent. Pulmonary features are cough, chest pain and haemoptysis. CT findings consisted of ground-glass attenuation in 94 per cent of cases. Other features are consolidations, thickening of bronchovascular bundles and honeycombing.45 Lung biopsy specimens frequently demonstrate only necrotic tissue or show non-specific inflammation with or without haemorrhage. Alveolar haemorrhage, vasculitis with alveolar haemorrhage, pulmonary fibrosis, bronchitis, bronchiectasis and bronchiolitis obliterans organizing pneumonia (BOOP) have been reported in surgical biopsies.45,46 Aggressive immunosuppressive therapy (corticosteroids and cyclophosphamide) is effective in inducing remission in about 80–90 per cent of cases. After 3–6 months cyclophosphamide is stopped and replaced by azathioprine. Other therapeutic options are mycophenolate mofetil or methotrexate.47–49 Rituximab, a chimeric anti-CD20 monoclonal antibody, has been shown to be effective.50 In severe lung haemorrhaging, pulse methyl-prednisolone and/or plasma exchange can be added.42 Antithyroid drugs such as PTU may induce MPA.51 Other drugs that are suspected to induce MPA are hydralazine and d-penicillamine. In these drug-induced cases, ANCA is frequently directed to two or more antigens including elastase.52
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.