scholarly journals A Rare Case of Double Antibody-Positive Rapidly Progressive Glomerulonephritis: A Therapeutic Challenge

2021 ◽  
Vol 9 ◽  
pp. 232470962110131
Author(s):  
Stephen Bell ◽  
Natalia Lattanzio ◽  
Qassem Abdelal ◽  
Yeshanew Teklie ◽  
Talal Alkayali ◽  
...  
Keyword(s):  
Crescentic Glomerulonephritis ◽  
Staining Pattern ◽  
Rapidly Progressive Glomerulonephritis ◽  
Pattern Of Injury ◽  
Elderly Female ◽  
Double Antibody ◽  
Anca Associated Vasculitis ◽  
Characteristic Features ◽  
Mixed Pattern ◽  
Chronic Risk

Crescentic glomerulonephritis, also known as rapidly progressive glomerulonephritis, is a syndrome characterized by progressive and rapid deterioration of renal function over the course of weeks to months. Oliguria, hematuria, azotemia, and hypertension are characteristic features of this condition. Crescentic glomerulonephritis is further classified according to the staining pattern on immunofluorescence. In rare instances, a mixed pattern of injury is encountered as in the case of double antibody-positive rapidly progressive glomerulonephritis (RPGN). This case illustrates the challenge in treatment of double antibody-positive RPGN in an elderly female with no previous renal disease. The patient was found to be positive for anti-GBM antibody and MPO-ANCA. Treatment was initially targeted against MPO-ANCA as the biopsy was most consistent with this process; however, the patient failed to respond to treatment and was subsequently transitioned to oral cyclophosphamide directed against anti-GBM disease. In cases of doubly antibody-positive RPGN with anti-GBM disease and ANCA-associated vasculitis, initial treatment should focus on inducing remission of anti-GBM disease as double antibody-positive disease often presents with the aggressive morbidity and mortality seen in anti-GBM disease, and the chronic risk of relapse seen in ANCA-mediated vasculitis.

scholarly journals Renal limited ANCA-positive vasculitis: a rare manifestation of a rare disease

2020 ◽  
Vol 8 ◽  
pp. 232470962097487
Author(s):  
Ali Jawad Jang Khan ◽  
Noman Ahmed Jang Khan
Keyword(s):  
Rare Case ◽  
Crescentic Glomerulonephritis ◽  
Kidney Biopsy ◽  
Rapidly Progressive Glomerulonephritis ◽  
The United States ◽  
High Dose ◽  
Shortness Of Breath ◽  
Good Clinical Response ◽  
Anca Associated Vasculitis ◽  
Rare Manifestation

Pauci-immune crescentic glomerulonephritis is the most common variant of rapidly progressive glomerulonephritis, accounting for approximately 80% of total cases. Most of the cases are associated with the presence of anti-neutrophil cytoplasmic antibody (ANCA) and are usually referred to as ANCA-associated vasculitis. A 68-year-old male with no previous renal history presented with complaints of shortness of breath, cough, and bilateral leg swelling for 2 weeks. Initial workup was significant for creatinine elevated at 2.9 mg/dL, blood urea nitrogen at 65 mg/dL, and glomerular filtration rate of 27 mL/min. Further workup was unremarkable for any significant abnormality. Subsequently patient’s kidney function worsened, and temporary hemodialysis was started. Kidney biopsy was performed, which later came back significant for necrotizing arteritis, multifocal, with focal necrotizing and crescentic glomerulonephritis, pauci-immune type. High-dose corticosteroids were administered, and good clinical response was noticed. This is a very rare case of renal limited pauci-immune crescentic glomerulonephritis with annual incidence of 7 to 10 cases per million every year in the United States. The absence of involvement of other organs makes our case even rarer. Mortality is as high as 90% in untreated patients and aggressive therapy with glucocorticoids and cyclophosphamide or rituximab are the mainstay of treatment. The presence of significant renal impairment in the absence of other organs involvement in our patient makes it a very unique presentation of ANCA-positive vasculitis.

scholarly journals Pauci-Immune Crescentic Glomerulonephritis: An ANCA-Associated Vasculitis

2015 ◽  
Vol 2015 ◽  
pp. 1-8 ◽  
Author(s):  
Rafeel Syed ◽  
Amina Rehman ◽  
Gautam Valecha ◽  
Suzanne El-Sayegh
Keyword(s):  
Crescentic Glomerulonephritis ◽  
Rapidly Progressive Glomerulonephritis ◽  
Small Vessel Vasculitis ◽  
Complex Spectrum ◽  
Crescent Formation ◽  
Glomerular Proteinuria ◽  
Anca Associated Vasculitis ◽  
Necrotizing Glomerulonephritis ◽  
Common Etiology ◽  
Genetic And Environmental Factors

Rapidly progressive glomerulonephritis(RPGN) is a syndrome signified by a precipitous loss of renal function, with features of glomerulonephritis including dysmorphic erythrocyturia and glomerular proteinuria. RPGN is associated with extensive crescent formation, and, thus, the clinical term RPGN is often used interchangeably with the pathologic termcrescentic glomerulonephritis(CGN). From an immunopathologic standpoint, primary RPGN is divided intopauci-immune GN(PICG), anti-GBM GN, and immune complex GN. PICG, the most common etiology of primary RPGN, refers to a necrotizing glomerulonephritis with few or no immune deposits by immunofluorescence (IF) or electron microscopy (EM). In most patients, pauci-immune CGN is a component of a systemic small vessel vasculitis such as granulomatosis with polyangiitis (GPA). Approximately 90% of patients with PICG have circulating ANCA antibodies, leading to the nomenclatureANCA-associated vasculitis(AAV). Recent research has identified several other antibodies associated with PICG, which is now understood to be a complex spectrum of disease with considerable overlap in terms of clinical phenotype and outcomes. In addition, several genetic and environmental factors have recently been implicated in the pathogenesis of this disorder. With new prognostic classifications, enhanced understanding of immunopathologic mechanisms, and novel treatment paradigms, clinical and experimental interest in PICG remains high.

scholarly journals ANCA-Associated Glomerulonephritis and Anti-Phospholipid Syndrome in a Patient with SARS-CoV-2 Infection: Just a Coincidence?

2021 ◽  
pp. 214-220
Author(s):  
Federica Maritati ◽  
Maria Ilaria Moretti ◽  
Valentina Nastasi ◽  
Roberta Mazzucchelli ◽  
Manrico Morroni ◽  
...  
Keyword(s):  
Rapidly Progressive Glomerulonephritis ◽  
Kidney Injury ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Hemodynamic Instability ◽  
Tubular Necrosis ◽  
Complex Processes ◽  
Anca Associated Vasculitis ◽  
High Incidence ◽  
New Diagnosis ◽  
The Relationship

Many reports have described a high incidence of acute kidney injury (AKI) among patients with COVID-19. Acute tubular necrosis has been reported to be the most common damage in these patients, probably due to hemodynamic instability. However, other complex processes may be involved, related to the cytokine storm and the activation of innate and adaptive immunity. Here, we describe a patient who developed an antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis with rapidly progressive glomerulonephritis and lung involvement and an antiphospholipid syndrome soon after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. After viral pneumonia was excluded by bronchoalveolar lavage, the patient has been treated with rituximab for amelioration of kidney function and resolution of thrombosis without any adverse event. We conclude that COVID-19 may trigger autoimmune diseases including ANCA-associated vasculitis. Thus, this diagnosis should be taken in consideration in COVID-19 patients, especially when they develop AKI with active urinary sediment. In addition, considering the relationship between these 2 diseases, SARS-CoV-2 infection should be excluded in all patients with a new diagnosis ANCA-associated vasculitis before starting immunosuppressive therapy.

Crescentic glomerulonephritis

2009 ◽  
pp. 399-434
Author(s):  
Patrick Nachman ◽  
Richard J. Glassock
Keyword(s):  
Renal Function ◽  
End Stage Renal Disease ◽  
Crescentic Glomerulonephritis ◽  
Rapidly Progressive Glomerulonephritis ◽  
Clinical Syndrome ◽  
Aggressive Treatment ◽  
Stage Renal Disease ◽  
End Stage ◽  
Glomerular Tuft

The term crescentic glomerulonephritis (CrGN) refers to a diverse collection of disorders of widely different etiology and pathogenesis having in common the development of extensive proliferation of cells within Bowman's space (Couser, 1988; Glassock et al., 1995; Nachman et al., 1998; Pusey and Rees, 1998; Morgan et al., 2006; Lionaki, et al., 2007). The resulting accumulation of cells gives rise to a ‘crescent’ enveloping the glomerular tuft itself. Polymerization of fibrinogen in Bowman's space due to passage of fibrinogen through gaps in the capillary wall, the elaboration of procoagulant factors by infiltrating monocytes and impaired fibrinolysis all contribute to the pathogenesis of the crescent (Couser, 1988, Glassock et al., 1995). Usually 〉50% of glomeruli are involved with crescentic lesions. Such patients also frequently manifest rapid and progressive deterioration of renal function leading to the clinical syndrome of rapidly progressive glomerulonephritis. Early and aggressive treatment can often delay or prevent the development of end-stage renal disease (ESRD). See Table 10.1 for an etiologic and pathogenetic classification of CrGN.

scholarly journals For fulminant rapidly progressive glomerulonephritis in a 17-year-old female patient with ANCA-associated vasculitis.

2018 ◽  
Vol 96 (2) ◽  
pp. 180-185
Author(s):  
Nadezhda B. Gordovskaya ◽  
E. M. Shilov ◽  
Yu. V. Korotchaeva ◽  
E. V. Stavrovskaya ◽  
S. V. Roshchupkin
Keyword(s):  
Renal Failure ◽  
Kidney Transplantation ◽  
Rapidly Progressive Glomerulonephritis ◽  
Proteinase 3 ◽  
Substitution Therapy ◽  
Anca Vasculitis ◽  
Terminal Renal Failure ◽  
Anca Associated Vasculitis ◽  
Morphological Picture ◽  
Systemic Manifestations

A case of rapidly progressive glomerulonephritis in a 17-year-old patient associated with antibodies against the cytoplasm of neutrophils (ANCA) vasculitis - ANCA-associated vasculitis is associated with antibodies to proteinase-3 and morphological picture extracapillar glomerulonephritis with sclerotic lesion of up to 80% of the glomeruli. The peculiarity of the case is the presence of morphologically confirmed when alloimmune rapidly progressive glomerulonephritis type III a pronounced glow-focal granular nature of immunoglobulin classes G and M on the basement membrane of capillaries. The appointment of immunosuppressive therapy led to a decrease in systemic manifestations of vasculitis, but there was a rapid increase in terminal renal failure, which required substitution therapy with hemodialysis. The possible mechanisms of the rapid-training course of the disease in the observed patient, prospects for kidney transplantation are discussed.

scholarly journals ANCA-Associated Vasculitis Following Johnson and Johnson COVID-19 Vaccine

2021 ◽  
Author(s):  
Rukesh Yadav ◽  
SANGAM SHAH ◽  
Santosh Chhetri
Keyword(s):  
Rare Case ◽  
Rapidly Progressive Glomerulonephritis ◽  
Cytoplasmic Granules ◽  
Vaccine Administration ◽  
Small Vessels ◽  
Anca Associated Vasculitis ◽  
Antineutrophil Cytoplasmic Autoantibodies

Antineutrophil cytoplasmic autoantibodies associated vasculitis(AAV) is characterized by antibodies against antigens in cytoplasmic granules of neutrophils and predominantly affects small vessels. AAV after COVID-19 mRNA vaccination has been reported. We report rare case of AAV in a patient who presented with rapidly progressive glomerulonephritis(RPGN) after Johnson & Johnson vaccine administration.

Sign in / Sign up

Export Citation Format

Share Document