scholarly journals Severe course of quickly progressing glomerulonephritis in a patient with ANTSA-associated vasculitis: review and case report

2020 ◽  
Vol 7 (3) ◽  
pp. 55-62
Author(s):  
Iu. V. Lavrishcheva ◽  
Y. S. Kaledinova ◽  
A. A. Yakovenko ◽  
I. A. Artemev
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Rapidly Progressive Glomerulonephritis ◽  
Granulomatous Inflammation ◽  
Clinical Work ◽  
Chronic Hemodialysis ◽  
Complete Loss ◽  
Vascular Lesions ◽  
Upper Respiratory Tract ◽  
Delay In Diagnosis ◽  
Necrotizing Glomerulonephritis

Granulomatosis with polyangiitis is characterized by necrotizing granulomatous inflammation, vasculitis with vascular lesions of small and medium caliber and focal necrotizing glomerulonephritis. A frequent and one of the most formidable complications is kidney damage, which in a large number of cases leads to a complete loss of organ function and a switch to renal replacement therapy. Given the rare occurrence of this disease in the clinical work of practitioners, and their low awareness of this pathology, problems often arise with the diagnosis and treatment of patients with HPA. Due to the diversity and non-specific nature of the manifestations of the disease, a delay in diagnosis may occur. The presented case illustrates the manifestations of granulomatosis with polyangiitis in the form of severe damage to the upper respiratory tract and kidneys, the diagnosis of which was difficult due to the rarity of the disease and the multiple organ pathology. This article presents a clinical case of severe progression of rapidly progressive glomerulonephritis in a patient with ANCA-associated vasculitis, a brief review of the literature is given. Despite adequate therapy, the disease progressed mainly due to deterioration of renal function, which subsequently led to a complete loss of kidney function and the transition to treatment with chronic hemodialysis.

scholarly journals A Clinical Approach of Renal Involvement in Granulomatosis with Polyangiitis

2020 ◽  
Vol 17 (2) ◽  
pp. 29-36
Author(s):  
Marilena Stoian ◽  
Lucia Indrei ◽  
Victor Stoica
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Hospital Admissions ◽  
Renal Involvement ◽  
Rapidly Progressive Glomerulonephritis ◽  
Granulomatous Inflammation ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
High Dose ◽  
Clinical Approach ◽  
Upper Respiratory Tract

AbstractGranulomatosis with polyangiitis (GPA, Wegener’s granulomatosis) is one of the antineutrophil cytoplasmic antibody (ANCA) - associated small vessel vasculitis, involving various organs such as nasal septum, sinuses, upper respiratory tract, lungs, and kidneys. GPA is pathologically characterized by necrotizing granulomatous inflammation. ANCA-associated small vessel vasculitis represent a major challenge in hospital admissions; therefore, early and accurate diagnosis with aggressive treatment is essential to improve the disease outcome.Renal involvement of granulomatosis with polyangiitis is characterized morphologically by extensive crescent formation (extracapillary proliferation in Bowman’s space), and clinically by crescentic or rapidly progressive glomerulonephritis that causes hematuria, erythrocyte casts, and proteinuria with progressive loss of renal function. The diagnosis of granulomatosis with polyangiitis is established most securely by biopsy specimens showing the triad of vasculitis, granulomata, and large areas of necrosis (known as geographic necrosis) admitted with acute and chronic inflammatory cells. Usually, renal involvement is severe and is the leading cause of mortality. The combination of high-dose corticosteroids and cyclophosphamide is the mainstay of treatment for vasculitis and disease resistance to this combination is rare.

scholarly journals GRANULOMATOSIS WITH POLYANGIITIS: TREAT THE PATIENT NOT SYMPTOMS

2018 ◽  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Treatment Strategies ◽  
Granulomatous Inflammation ◽  
Organ Damage ◽  
Upper Respiratory Tract ◽  
Childbearing Age ◽  
Immunological Mechanisms ◽  
Upper Respiratory ◽  
Tract Infections ◽  
In Pregnancy

Immunological mechanisms of appearance and therapeutic treatment strategies were discussed on example of the rare granulomatosis with polyangiitis clinical case in young patient. This vasculitis, formerly known as Wegener’s granulomatosis, is a rare multisystem autoimmune disease with necrotizing granulomatous inflammation and pauci-immune vasculitis in small- and medium-sized blood vessels. Autoimmune diseases affect 5 to 7% of people, are commoner in women of childbearing age, and are frequently encountered in pregnancy. They may remit or improve during pregnancy, butcan flare or present in pregnancy with disastrous consequences. Otorhinolaryngologist is the first physician to contact for the majority of patients with GPA. This diagnosis must always be taken into consideration in patients with recurrent upper respiratory tract infections, otitis, mucosal ulcers and laryngitis. Proper and early diagnosis is crucial for imminent therapy implementation and allows avoiding irreversible organ damage.

scholarly journals Significance of Small Renal Artery Lesions in Patients with Antineutrophil Cytoplasmic Antibody-associated Glomerulonephritis

2014 ◽  
Vol 41 (6) ◽  
pp. 1140-1146 ◽  
Author(s):  
Akiko Endo ◽  
Junichi Hoshino ◽  
Tatsuya Suwabe ◽  
Keiichi Sumida ◽  
Koki Mise ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Rapidly Progressive Glomerulonephritis ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Pulmonary Involvement ◽  
Renal Arteries ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Necrotizing Glomerulonephritis ◽  
Group A ◽  
Group B

Objective.Antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis is a vasculitis affecting the glomerular capillaries and small renal arteries. Although crescent formation has been reported to be characteristic of this condition, the significance of coexisting vasculitis affecting the small renal arteries has not been investigated.Methods.Fifty patients with ANCA-positive rapidly progressive glomerulonephritis whose renal biopsy specimens contained arterioles and/or interlobular arteries were retrospectively evaluated. Cellular crescents and/or necrotizing glomerulonephritis were noted in all 50 patients. Ten patients had vasculitis of the small renal arteries (group A) and 40 patients were without such vasculitis (group B). The clinical features of these 2 groups were compared.Results.Group A comprised 4 patients who had granulomatosis with polyangiitis (GPA) and 6 with microscopic polyangiitis (MPA), while group B included 1 patient with GPA and 39 with MPA. No patient in either group had eosinophilic granulomatosis with polyangiitis. The C-reactive protein (CRP) level was significantly higher in group A compared with group B (11.58 ± 6.19 vs 2.7 ± 3.55 mg/dl, p < 0.05), and pulmonary involvement was more frequent in group A than group B (80% vs 37.5%, p < 0.05).Conclusion.In patients with ANCA-positive glomerulonephritis, vasculitis of small renal arteries may be associated with systemic vasculitis (including pulmonary involvement) because of elevated CRP, a systemic inflammatory marker related to overproduction of interleukin 6.

scholarly journals Renal involvement in granulomatosis with polyangiitis

2020 ◽  
Vol 8 (6) ◽  
pp. 158-159
Author(s):  
Aya Fraj ◽  
Olfa Berriche ◽  
Sondes Arfa ◽  
Jihen Chelli ◽  
Wafa Bel Abed ◽  
...  
Keyword(s):  
Renal Failure ◽  
Respiratory Tract ◽  
Granulomatosis With Polyangiitis ◽  
Renal Involvement ◽  
Rapidly Progressive Glomerulonephritis ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Upper Respiratory Tract ◽  
Terminal Renal Failure ◽  
Upper Respiratory

Granulomatosis with polyangiitis (GPA) is defined by the presence of small vessel vasculitis that affects the upper respiratory tract, lungs and kidneys. Renal involvement is frequent. The disease varies from asymptomatic to fulminant rapidly progressive glomerulonephritis forms, leading to terminal renal failure in days.

scholarly journals A Diagnosis Missed for Several Years- Wegener’s Granulomatosis

2012 ◽  
Vol 9 (3) ◽  
pp. 218-221 ◽  
Author(s):  
B P Paudyal ◽  
S Pantha ◽  
N Ranjitkar ◽  
A Manandhar ◽  
A Arjyal
Keyword(s):  
Wegener’S Granulomatosis ◽  
Systemic Vasculitis ◽  
Systemic Disease ◽  
Granulomatous Inflammation ◽  
Wegener's Granulomatosis ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Upper Respiratory Tract ◽  
Delay In Diagnosis ◽  
Organ Systems ◽  
Wide Range

Wegener’s granulomatosis is a form of systemic vasculitis of small to medium sized vessels and affects upper respiratory tract, lungs and kidneys along with various organs. It causes necrotizing granulomatous inflammation of the affected parts and presents with positive antineutrophil cytoplasmic antibodies in more severe forms. Being a systemic disease with the potential to affect any organ-systems with a wide range of clinical presentations, it is associated with a risk of delay in diagnosis with resultant setback in institution of appropriate treatment. Confusion may arise due to an extent of histological similarity between Wegener’s granulomatosis and the more prevalent tuberculosis, both causing granulomatous inflammation of the affected parts. Here, we present two cases of this rare disorder where the diagnosis was missed for several years in the beginning causing a delay in institution of specific therapy which led to the development of complications.DOI: http://dx.doi.org/10.3126/kumj.v9i3.6309 Kathmandu Univ Med J 2011;9(3):218-21 

Granulomatosis with Polyangiitis (Wegener's Granulomatosis): Evolving Concepts in Treatment

2018 ◽  
Vol 39 (04) ◽  
pp. 434-458 ◽  
Author(s):  
Ariis Derhovanessian ◽  
Henry Tazelaar ◽  
John Belperio ◽  
Joseph Lynch
Keyword(s):  
Respiratory Tract ◽  
Wegener’S Granulomatosis ◽  
Granulomatosis With Polyangiitis ◽  
Treatment Strategies ◽  
Granulomatous Inflammation ◽  
Wegener's Granulomatosis ◽  
Upper Respiratory Tract ◽  
Small Vessels ◽  
Successful Induction ◽  
Indolent Disease

AbstractGranulomatosis with polyangiitis (GPA), formerly termed Wegener's granulomatosis, is the most common of the pulmonary vasculitides. GPA typically involves the upper respiratory tract, lower respiratory tract (bronchi and lung), and kidney, with varying degrees of disseminated vasculitis. Cardinal histologic features include a necrotizing vasculitis involving small vessels, extensive “geographic” necrosis, and granulomatous inflammation. The spectrum and severity of the disease is heterogeneous, ranging from indolent disease involving only one site to fulminant, multiorgan vasculitis. Circulating antibodies against cytoplasmic components of neutrophils (ANCAs) play a role in the pathogenesis, and often correlate with activity of the disease. Treatment strategies are evolving. Cyclophosphamide (CYC) plus corticosteroids was the mainstay of therapy for generalized, multisystemic GPA since the 1970s. However, within the past decade, rituximab (RTX), a monoclonal antibody directed against B cells, has been shown to be at least as effective (and possibly more effective) as CYC. Furthermore, the use of RTX may reduce the need for maintenance immunosuppression. Optimal therapy for GPA remains controversial, and additional studies are required to determine the role and duration of maintenance therapy following successful induction therapy.

scholarly journals Atypical Endobronchial Carcinoid with Postobstructive Pneumonia Obscuring the Diagnosis of Granulomatosis with Polyangiitis

2015 ◽  
Vol 2015 ◽  
pp. 1-8
Author(s):  
Robert Ali ◽  
Candice Baldeo ◽  
Jesse Onyenekwe ◽  
Roshan Lala ◽  
Cristian Landa ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Clinical Manifestations ◽  
Granulomatous Inflammation ◽  
Lower Limbs ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Small Vessel Vasculitis ◽  
American College Of Rheumatology ◽  
Abnormal Chest ◽  
Necrotizing Glomerulonephritis

Granulomatosis with polyangiitis (GPA), previously termed Wegener’s Granulomatosis, is an autoimmune small vessel vasculitis which is highly associated with antineutrophil cytoplasmic antibodies (ANCA) and has varied clinical manifestations. Diagnosis hinges on identifying a combination of clinical features of systemic vasculitis, positive ANCA serology, and histological evidence of necrotizing vasculitis, necrotizing glomerulonephritis, or granulomatous inflammation from a relevant organ biopsy. The American College of Rheumatology has also developed a classification criteria focusing specifically on nasal or oral inflammation, abnormal chest radiograph, and abnormal urinary sediment, along with granulomatous inflammation, which helps to distinguish GPA from other forms of systemic vasculitis. In the case presented below, the diagnosis of GPA was delayed as the patient had a concomitant atypical endobronchial carcinoid which predisposed to postobstructive pneumonia. Fortunately, the papular lesions that developed across her lower limbs prompted further investigations. The return of appropriate serology coincided with progression to alveolar hemorrhage, offering a more complete clinical picture, and when she responded to the combination of steroid, cyclophosphamide, and plasma exchange, the diagnosis of GPA was cinched.

scholarly journals Novel pediatric granulomatosis with polyangiitis with a marked bloody pericardial effusion and bloody stool: a case report

2021 ◽  
Vol 17 (1) ◽  
Author(s):  
Memi Kato ◽  
Keisuke Jimbo ◽  
Masumi Nagata ◽  
Yoshiko Endo ◽  
Kosuke Kashiwagi ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Pericardial Effusion ◽  
Cardiac Tamponade ◽  
Granulomatosis With Polyangiitis ◽  
Granulomatous Inflammation ◽  
Pericardial Fluid ◽  
Bloody Stool ◽  
Upper Respiratory Tract ◽  
Gastrointestinal Complications ◽  
First Case

Abstract Background Granulomatosis with polyangiitis (GPA) is a syndrome of refractory vasculitis involving the upper respiratory tract, lungs, kidneys, and systemic small and medium-sized arteries that affects all age groups. No pediatric case with a bloody pericardial effusion resulting in cardiac tamponade and co-existing hematochezia has been reported. Case presentation A 14-year-old boy was referred for evaluation of prolonged fever, chest pain, and intermittent hematochezia. Diagnostic imaging showed a prominent pericardial effusion. Immediately after admission, his systolic blood pressure decreased. Emergent pericardiocentesis resulted in aspiration of a massive amount of bloody pericardial fluid. This was diagnosed as cardiac tamponade because his blood pressure recovered immediately after the drainage. The patient had an elevated serine proteinase 3-anti-neutrophil cytoplasmic antibody (PR3-ANCA) level on serological examination. Head MRI showed thickening of nasal and sinusoidal mucosa and a cystic mass in the left sphenoid sinus. After ruling out malignancy based on the cytology of the effusion, chest MRI, and gallium scintigraphy, total colonoscopy showed multiple irregular-shaped aphthae from the right transverse colon to the cecum on the contralateral side of the mesenteric attachments. Biopsy specimens of aphthous lesions confirmed necrotizing granulomatous inflammation. A diagnosis of GPA was made based on these findings, and oral prednisolone (PSL) and azathioprine were started. The hematochezia disappeared rapidly, and no recurrence of pericardial effusion was seen after PSL tapering was completed. The PR3-ANCA level decreased into the normal range immediately after the initial therapy. Conclusions Pericarditis is a common cardiac complication of GPA, but there have been no reports of resultant cardiac tamponade. This is the first case of pediatric GPA with cardiac and gastrointestinal complications preceding the common symptoms such as respiratory or renal symptoms. A case of pediatric GPA with hematochezia is also extremely rare. In conclusion, serial measurement of ANCA levels is important in patients with persistent fever and bloody stool, such as in inflammatory bowel disease, to make the diagnosis of a vasculitic syndrome.

Liver rheumatoid nodules imitating liver malignancy: a rare occurrence

2020 ◽  
Vol 13 (12) ◽  
pp. e234366
Author(s):  
Jason Wee ◽  
Salar Sobhi ◽  
Bastiaan De Boer ◽  
Dan Xu
Keyword(s):  
Histological Analysis ◽  
Granulomatous Inflammation ◽  
Needle Aspiration ◽  
Upper Respiratory Tract ◽  
Positron Emission Tomography Scan ◽  
Liver Malignancy ◽  
Positron Emission ◽  
Right Lobe ◽  
Upper Respiratory ◽  
Tomography Scan

We describe a case of a 61-year-old man with a background of rheumatoid arthritis who presented to the emergency department with a single-reported episode of haemoptysis on the background of an upper respiratory tract infection. A CT scan revealed an incidental 40 mm mass in upper right lobe of the liver abutting the diaphragmatic surface. A subsequent positron emission tomography scan confirmed the mass and raised the possibility of another lesion in the liver raising the suspicion of malignancy. The case was complicated by the inability to perform a fine needle aspiration biopsy due to the mass’ proximity to the diaphragm. After discussion with the patient, it was decided to resect the affected liver segment. Histological analysis of the mass revealed localised necrotising granulomatous inflammation suggestive of a rheumatoid nodule, which is seldom reported in the literature.

scholarly journals OP0054 NEW ROLE FOR PROTEINASE 3 IN IL-16 BIOACTIVITY CONTROL IN GRANULOMATOSIS WITH POLYANGIITIS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 28.1-29
Author(s):  
A. Kerstein-Staehle ◽  
C. Alarcin ◽  
J. Luo ◽  
G. Riemekasten ◽  
P. Lamprecht ◽  
...  
Keyword(s):  
Cell Death ◽  
Western Blot ◽  
Disease Activity ◽  
Chronic Inflammation ◽  
Immune Cells ◽  
Peripheral Blood ◽  
Granulomatosis With Polyangiitis ◽  
Granulomatous Inflammation ◽  
Proteinase 3 ◽  
Sds Page

Background:The immunomodulatory cytokine IL-16 is increased in several inflammatory and autoimmune diseases1. IL-16 recruits and activates CD4+ immune cells such as T cells, dendritic cells, or monocytes. IL-16 is produced by various immune and non-immune cells, but synthesis and storage of IL-16 is regulated differentially depending on the cell type and stimulation. For its biological activity, IL-16 cleavage by caspase-3 is required1. Necrotizing granulomatous inflammation is a hallmark of granulomatosis with polyangiitis (GPA) with neutrophil dysregulation as a central driver of chronic inflammation and autoimmunity2. Earlier studies showed a correlation between increased serum IL-16 and disease parameters in AAV, including GPA3, but functional evidence for a direct link between IL-16 and neutrophils in granulomatous inflammation is missing so far.Objectives:In this study we aim to identify a functional link between increased IL-16, neutrophils, and the autoantigen proteinase 3 (PR3) with regard to chronic inflammation and autoimmunity in GPA.Methods:IL-16 was measured in sera of GPA patients (n = 40) and healthy controls (HC, n = 50) by ELISA and correlated with clinical features, such as disease activity (BVAS), creatinine, GFR, VDI and PR3-ANCA status. IL-16 protein expression was analyzed in peripheral blood mononuclear cells (PBMC) and polymorphonuclear cells (PMN) from GPA patients and HC (n = 5, each) by SDS-PAGE and western blot. Binding affinity of recombinant pro-IL-16 to native human PR3 was assessed by microscale thermophoresis. Cleavage of pro-IL-16 by active human PR3 was performed at various time points at 37°C. Cleavage products were analyzed by SDS-PAGE and western blot.Results:Circulating IL-16 was significantly increased in GPA patients compared to HC. Elevated IL-16 positively correlated with BVAS, creatinine, VDI and PR3-ANCA status and negatively correlated with GFR. In PMBC and PMN from GPA and HC we identified different expression patters of precursor and active forms of IL-16. In healthy PBMC we found high amounts of precursor (80kD), pro-IL-16 (55kD) and active IL-16 (17kD). In contrast, PBMC from GPA patients had lower amounts of pro-IL-16 and no active IL-16, indicating activation and secretion of IL-16 due to inflammatory stimulation, as shown earlier5. In GPA PMN we detected no precursor IL-16, but pro-IL-16 and its active form, in contrast to very low amounts of all IL-16 forms in healthy PMN. Processing and release of IL-16 in neutrophils has been linked to apoptosis and secondary necrosis5. By interaction studies we demonstrated direct binding of pro-IL-16 to PR3 with a Kd of 10 nM. In a subsequent cleavage assay we confirmed IL-16 processing by PR3 in a time-dependent manner.Conclusion:Correlation of serum IL-16 with clinical features of GPA suggests that IL-16 is associated with markers of disease activity, tissue damage and autoreactivity. We showed that PBMC and PMN represent a source of IL-16 in GPA. By the identification of PR3 as an additional IL-16-activating enzyme we could demonstrate a potential link between excessive PR3 expression, cell death and IL-16-dependent mechanisms, contributing to chronic granulomatous inflammation and autoimmunity in GPA.References:[1]Glass, W. G. et al. Not-so-sweet sixteen: The role of IL-16 in infectious and immune-mediated inflammatory diseases. J. Interf. Cytokine Res. 26, 511–520 (2006).[2]Millet, A. et al. Proteinase 3 on apoptotic cells disrupts immune silencing in autoimmune vasculitis. J. Clin. Invest. 125, 4107–4121 (2015).[3]Yoon, T. et al. Serum interleukin-16 significantly correlates with the Vasculitis Damage Index in antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Res. Ther. 22, 1–6 (2020).[4]Elssner, A. et al. IL-16 Is Constitutively Present in Peripheral Blood Monocytes and Spontaneously Released During Apoptosis. J. Immunol. 172, 7721–7725 (2004).[5]Roth, S. et al. Secondary necrotic neutrophils release interleukin-16C and macrophage migration inhibitory factor from stores in the cytosol. Cell Death Discov. 1, 15056 (2015).Disclosure of Interests:None declared

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