scholarly journals Granulomatosis with polyangiitis

2016 ◽  
Vol 9 (4) ◽  
pp. 217
Author(s):  
Zubayer Ahmad ◽  
Sadiqa Tuqan ◽  
Mirza Nazimuddin ◽  
Rama Biswas ◽  
Anindita Roy
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
High Titer ◽  
Granulomatous Inflammation ◽  
Needle Aspiration ◽  
Organ System ◽  
Lower Airway ◽  
Neurological Involvement ◽  
Cns Involvement ◽  
Intravenous Methylprednisolone ◽  
Airway Symptoms

<p>Granulomatosis with polyangiitis is a small and medium vessel vasculitis. It may affect any organ system. Neurological involvement, though less frequent, mostly manifests as peripheral neuropathy. Central nervous system (CNS) involvement as autoimmune cerebritis is rare but may be devastating. We report a patient having CNS involvement in the form of Parkinsonism long before the development of typical upper and lower airway symptoms. Granulomatosis with polyangiitis was diagnosed on the basis of cavitary lung lesions along with epistaxis, granulomatous inflammation from fine needle aspiration (FNAC) of lymph node and high titer of c-ANCA. The patient was commenced on intravenous methylprednisolone followed by mycophenolate mofetil and responded to treatment. Autoimmune cerebritis may present with subacute manifestation of diffuse CNS involvement.</p>

scholarly journals A Fulminant Case of Granulomatosis with Polyangiitis with Meningeal and Parenchymal Involvement

2015 ◽  
Vol 7 (1) ◽  
pp. 101-104 ◽  
Author(s):  
Ryuji Yajima ◽  
Yasuko Toyoshima ◽  
Yoko Wada ◽  
Tetsuya Takahashi ◽  
Hiroyuki Arakawa ◽  
...  
Keyword(s):  
Decompressive Craniectomy ◽  
Granulomatosis With Polyangiitis ◽  
Granulomatous Inflammation ◽  
Giant Cells ◽  
Cns Involvement ◽  
Small Vessels ◽  
Cerebrovascular Events ◽  
Brain Material ◽  
Perivascular Area ◽  
Paranasal Sinus Disease

Central nervous system (CNS) involvement, such as pachymeningitis and/or cerebrovascular events, is rare in patients with granulomatosis with polyangiitis (GPA). Furthermore, the details of pathological examinations of cases have rarely been described. We describe a case of GPA that manifested as an isolated paranasal sinus disease that invaded the subarachnoid space and caused a hemorrhagic venous infarction. We also describe the pathological characteristics of the biopsied brain material from the successful decompressive craniectomy. In particular, granulomatous inflammation with geographic necrosis and multinucleated giant cells were observed in the perivascular area of the thickened dura mater and leptomeninges. Small vessels in the meninges were involved in the granulomatous lesions, and the lumens of the veins were often occluded. In the cerebral cortices and white matter in these areas, hemorrhagic infarction was widely observed. We suggest that our findings represent a novel mechanism of CNS involvement in GPA. Moreover, we believe that the emergency decompressive craniectomy and partial lobectomy for the cerebral infarction in this patient with GPA likely contributed to his survival.

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

scholarly journals Atypical Granulomatosis with Polyangiitis Presenting with Meibomitis, Scleritis, Uveitis and Papillary Bladder Tumor: A Case Report and Literature Review

Diagnostics ◽  
2021 ◽  
Vol 11 (4) ◽  
pp. 680
Author(s):  
Takashi Kojima ◽  
Murat Dogru ◽  
Eisuke Shimizu ◽  
Hiroyuki Yazu ◽  
Aya Takahashi ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Early Stage ◽  
Topical Steroid ◽  
Granulomatous Inflammation ◽  
Oral Prednisolone ◽  
Meibomian Gland ◽  
Clinical Findings ◽  
Meibomian Gland Dysfunction ◽  
Oral Steroids ◽  
Atypical Cells

Granulomatosis with polyangiitis (GPA) presents with a variety of systemic findings, sometimes with ocular findings initially, but is often difficult to diagnose at an early stage. An 85-year-old male had complaints of ocular dryness and redness and was diagnosed with meibomian gland dysfunction with meibomitis. Despite an initial treatment with topical steroid and antibiotics, the meibomitis did not improve and the left eye developed scleritis and iridocyclitis. The patient was administered topical mydriatics and oral steroids. During follow-up, the patient developed left hearing difficulty and reported a darker urine. Urinalysis revealed microscopic hematuria. A blood test showed an elevated erythrocyte sedimentation rate, positivity for perinuclear anti-neutorophil cytoplasmic antibody, and elevations in blood urea nitrogen and serum creatinine. Nasal mucosal biopsy showed a non-necrotizing granulomatous inflammation. Renal biopsy revealed focal glomerulosclerosis. Cystoscopy and bladder wash followed by a planned transurethral resection revealed atypical cells and apical papillary tumors which were resected. Iridocyclitis and scleritis responded well to oral prednisolone with 0.1% topical betamethasone and prednisolone ointment. The patient is tumor free with no recurrences 24 months after resection. GPA may present atypically with meibomian gland dysfunction without showing representative clinical findings. Early detection and treatment are essential for visual recovery.

scholarly journals Life-Threatening Subglottic Stenosis of Granulomatosis with Polyangiitis: A Case Report

Medicina ◽  
2021 ◽  
Vol 57 (5) ◽  
pp. 423
Author(s):  
Jin An ◽  
Jae-Won Song
Keyword(s):  
Immunosuppressive Therapy ◽  
Granulomatosis With Polyangiitis ◽  
Granulomatous Inflammation ◽  
Subglottic Stenosis ◽  
Emergency Tracheostomy ◽  
Intralesional Steroid ◽  
Intralesional Steroid Injection ◽  
Life Threatening ◽  
Carbon Dioxide Co2 ◽  
Laryngotracheal Resection

Granulomatosis with polyangiitis (GPA) is an autoimmune disease characterized by necrotizing granulomatous inflammation. Subglottic stenosis, which is defined as narrowing of the airway below the vocal cords, has a frequency of 16–23% in GPA. Herein, we present the case of a 39-year-old woman with subglottic stenosis manifesting as life-threatening GPA, which was recurrent under systemic immunosuppressive therapy. The patient underwent an emergency tracheostomy, intratracheal intervention, such as carbon dioxide (CO2) laser surgery and intralesional steroid injection via laryngomicroscopic surgery, and laryngotracheal resection with remodeling. Severe subglottic stenosis treatment requires active intratracheal intervention, surgery, and systemic immunosuppressive therapy.

scholarly journals Look granulomatosis with polyangiitis (GPA) straight in the face: missed opportunities leading to a delayed diagnosis

2019 ◽  
Vol 10 (1) ◽  
Author(s):  
N. Rolle ◽  
M. Muruganandam ◽  
I. Jan ◽  
F. M. Harji ◽  
J. Harrington ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Delayed Diagnosis ◽  
Organ System ◽  
Atypical Presentation ◽  
Mural Thrombus ◽  
Missed Opportunities ◽  
Original Diagnosis ◽  
The Face ◽  
High Index Of Suspicion

Abstract Granulomatosis with polyangiitis (GPA) is a systemic vasculitis with a potential to involve any organ system. It remains an important cause of kidney related morbidity and mortality. Early diagnosis can be difficult and requires high index of suspicion in all patients, but especially in cases with atypical presentation. We report a case with GPA, which was diagnosed only after new and advancing symptoms belied the original diagnosis of bilateral facial palsy and aortic mural thrombus.

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 Laryngeal Subsite Analysis of Granulomatosis With Polyangiitis (Wegener’s)

OTO Open ◽  
2021 ◽  
Vol 5 (3) ◽  
pp. 2473974X2110363
Author(s):  
Natasha J. Minaya ◽  
Vishwanatha Rao ◽  
Matthew R. Naunheim ◽  
Phillip C. Song
Keyword(s):  
Vocal Fold ◽  
Chart Review ◽  
Granulomatosis With Polyangiitis ◽  
Age At Onset ◽  
Retrospective Chart Review ◽  
Tertiary Referral Center ◽  
Voice Changes ◽  
Airway Symptoms ◽  
Related Quality ◽  
Underlying Mechanisms

Objective To analyze specific intralaryngeal findings associated with granulomatosis with polyangiitis (GPA). Study Design Retrospective chart review. Setting Tertiary referral center. Methods A retrospective chart review was performed on all patients diagnosed with GPA who were evaluated at the laryngology division of Massachusetts Eye and Ear Infirmary between January 2006 and September 2019. Results Forty-four patients (14 male, 30 female) were evaluated for laryngeal pathology. The mean age at onset was 48 years. Nine patients (21%) were identified with only vocal fold disease, 11 (25%) with subglottic disease, and 8 (18%) with disease at the glottis and subglottis (transglottic). The remaining 16 patients (36%) had a normal airway upon examination although they presented with laryngeal symptoms. Patients with glottic disease had statistically significantly lower voice-related quality of life scores than patients with isolated subglottic stenosis. Conclusions Although laryngeal manifestations of GPA is often described as a subglottic disease presenting with respiratory symptoms, subsite analysis show that only 25% of patients had subglottic disease alone, with similar rates of glottic disease alone. Laryngeal subsites have different epithelial mucosa, function, and physiology, and understanding the specific sites of involvement will determine symptoms and enable better analysis of the underlying mechanisms of disease. Glottic disease is associated with a reduction in vocal fold motion and voice changes. Subglottic involvement presents more frequently with airway symptoms. Further research is necessary to better define the specific regions of laryngeal involvement in patients diagnosed with GPA.

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