wegeners granulomatous

Granulomatosis with polyangiitis (GPA), formerly named Wegner's granulomatosis is an antineutrophilic cytoplasmic antibody (ANCA) associated vasculitis of the small vessels. GPA can affect several organ systems even though predominantly affects respiratory and renal systems. Pathogenesis is initiated by activation of the immune system to produce ANCA, Cytoplasmic (C-ANCA) antibody, which thereby leads to widespread necrosis and granulomatous inflammation. Multisystem involvement with varied symptomatology makes GPA diagnosis more challenging...

Wegener's granulomatosis (WG) is a form of systemic vasculitis characterized by granulomatous inflammation of the upper and lower airways, vasculitis, and necrotizing glomerulonephritis. It is strongly associated with anti-neutrophil cytoplasmic antibodies against proteinase 3 (PR3-ANCAs). Various in vitro observations provided strong evidence that autoimmune PR3-ANCAs are directly involved in glomerular and vascular inflammation. However, little is known about the pathogenic significance of PR3-ANCAs in vivo...

BACKGROUND: Neurosyphilis (NS) is a rarely encountered scenario today. Manifestations are heterogeneous, and their characteristics have changed in the antibiotic era. A differential diagnosis of NS is not commonly thought of even with relevant clinical-radiological features, as it mimics many common neurological syndromes. OBJECTIVES: To study the manifestations of NS in the present era and the process of diagnosis. METHOD: The data of ten patients with NS was collected and analyzed...

Wegener's granulomatosis or granulomatosis with polyangiitis (GPA) is multisystemic vasculitis. Kidney involvement in GPA often presents with rapidly progressive renal failure and requires urgent treatment. A 60-year-old female presented with prolonged history of fever, generalized weakness, decreased appetite, and weight loss over 4 months. Her renal function was normal; urine culture was sterile. On further evaluation, she was found to have large, hypodense solid lesion in mid pole of the right kidney on CECT...

Granulomatosis with polyangiitis (GPA) is a rare cause of prostatitis. Our case illustrates a case of granulomatous prostatitis secondary to unrecognised GPA requiring multiple surgical interventions. The patient presented with lower urinary tract symptoms. They underwent two endoscopic prostatic resections for recurrent urinary obstruction due to granulomatous prostatitis before a diagnosis of GPA was made. The rarity of this pathology, challenges in making a diagnosis and the systemic nature of this disease are emphasised...

Granulomatosis with polyangiitis formerly known as Wegener's granulomatosis was first described by German pathologist Friedrich Wegener in 1936. It is a multi-system necrotizing noncaseating granulomatous vasculitis which affects small to medium-sized vessels. It can involve any organ system, most commonly the lungs and kidneys. American College of Rheumatology requires 2 of 4 criteria for diagnosis: Positive biopsy for granulomatous vasculitis, urinary sediment with red blood cells, abnormal chest radiograph and oral/nasal inflammation...

To present a case of successful pregnancy outcome in a granulomatosis with polyangiitis (GPA) patient with renal insufficiency. GPA, formerly known as Wegener's granulomatosis, is a rare necrotizing systemic vasculitis, presenting with classical clinical triad of manifestations involving upper and lower airway and glomerulonephritis. An association of Antineutrophil cytoplasmic antibodies with GPA has been established and the antibodies are present in most patients with active disease. Pregnancy with GPA is burdened with the risk of possible maternal and fetal complications, further leading to higher morbidity and mortality rate...

Granulomatosis with polyangiitis (GPA) is a rare vasculitis among adolescents. Its pulmonary manifestations may mimic tuberculosis. We report the case of a 16-years-old female patient with multiple excavated lung nodules revealed by a chronic cough, hemoptysis, epistaxis and weight loss. The diagnosis of GPA was achieved due to systemic pulmonary, ENT and renal involvement, the positivity of anti-neutrophil cytoplasmic antibody directed against proteinase 3 (C-ANCA) and bronchial and nasal biopsies showing granulomatous inflammation with a dense perivascular infiltrate destroying the vessel wall...

Since idiopathic granulomatous lobular mastitis, also known as idiopathic granulomatous mastitis or granulomatous lobulitis, was first described by Kessler and Wolloch in 1972, no consensus on the ideal and definitive treatment for this phenomenon has been reached thus far. Idiopathic granulomatous lobular mastitis mostly frequently observed in women of childbearing age within a few years of pregnancy with a higher incidence in patients of Hispanic, Native American, Middle Eastern, and African descent. This entity, per se , is a rare, benign, chronic inflammatory breast condition of unknown aetiology mimicking two common breast disorders...

Granulomatosis with polyangiitis (GPA) is a granulomatous-necrotic systemic vasculitis with a lesion of predominantly the upper and lower respiratory tracts at the onset of the disease (vasculitis, accompanied by granulomatous inflammation), and subsequently renal (glomerulonephritis). In addition, GPA may manifest as inflammation of small arteries and veins. Despite many years of study of this disease, the etiology of GPA remains unknown. The present case is about a 47-year-old female, who had been suffering from necrotizing scleritis, corneal ulcer, and secondary glaucoma in both eyes for 3 months, and she was treated with anti-inflammatory and antimicrobial therapy that showed no effect; the patient's general condition became worse...

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...

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...

BACKGROUND: Granulomatosis with polyangiitis (GPA) is a rare autoimmune disease that involves small-to-medium-sized vessels and forms necrotizing vasculitis with granulomatous inflammation. The formation of a large vessel lesion in GPA patients has been scarcely reported, and it can cause confusion in the diagnosis. CASE SUMMARY: A 27-year-old man presented with mild left-sided pleuritic chest pain that started one year prior. An imaging study revealed up to 2.5 cm-sized two irregular nodular consolidation nodule in the left lower lobe...

Granulomatosis with polyangiitis (GPA) is a systemic necrotizing vasculitis involving small and medium-sized blood vessels and granulomatous inflammation of upper and lower respiratory systems and/or renal system. In the limited form of GPA, there is no systemic involvement of disease with sparing of kidneys. The respiratory system is the commonly involved organ in limited GPA. Herein, we report the case of a 40-year-old male who was initially diagnosed as sarcoidosis clinically. Lung biopsy revealed necrotizing granulomatous angiitis...

Pulmonary vasculitis can be the manifestation of several systemic illnesses such as primary systemic vasculitis, collagen vascular diseases, and systemic diseases associated with autoantibodies. It may be associated with granulomatous, eosinophilic, lymphoplasmacytic and neutrophilic inflammatory diseases. In this case report, we describe a 22-year-old female presented with intermittent fever, sweating and shivering, haemoptysis, sore throat, shortness of breath, fatigue, loss of appetite, nausea, non-projectile vomiting, dizziness, and dark coloured urine...

Gastric neuroendocrine tumors (G-NETs) express somatostatin receptors (SSTR), which can be imaged using radiolabeled somatostatin analogs, including 68 Ga-DOTA octreotide analogs. SSTR expression is also seen in activated lymphocytes and macrophages, which might result in false-positive results on SSTR imaging, in patients with coexistent granulomatous pathologies including tuberculosis, sarcoidosis, and Wegener's granulomatosis. We present a case where 68 Ga-DOTANOC positron emission tomography/computed tomography (PET/CT) showed primary G-NET, with SSTR expressing nonregional lymph nodes which on histopathology showed necrotizing granulomas with Langhans histiocytes...

Breast sarcoidosis is an extremely rare entity (about 1%). Conventional imaging significantly contributes to the detection of breast lesions, but it has been unable to establish a definite diagnosis. Histological examination should be mandatory, over imaging assessments, in order to confirm an early diagnosis and to avoid unjustified treatments. Malignancy should be excluded as a primary differential diagnosis. However, in the presence of granulomas, it is important to recognize other granulomatous disorders such as tuberculosis, Wegener's granulomatosis, or idiopathic granulomatous mastitis, since therapeutic strategies differ...

Granulomatosis with polyangiitis (GPA) formerly known as Wegener's granulomatosis, is an anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV). It is an uncommon disease with an estimated prevalence of 3 in 100,000 individuals with an equal distribution in both sexes. It is characterized by necrotizing granulomatous vasculitis that primarily affects the upper and lower respiratory tracts and the kidneys.  Our patient's initial presentation was abdominal pain with no typical pulmonary or renal manifestations...

Mycobacterium tuberculosis affects the middle ear in rare cases and is a challenging diagnosis. In this case, we present a 57-year-old patient diagnosed with anti-neutrophil cytoplasmic antibody (ANCA)-negative granulomatosis with polyangiitis (GPA) following a biopsy result of nasal granulomas, who was immediately started on immunosuppressive treatment. Years later, she developed progressive hypoacusis. Magnetic resonance imaging (MRI) revealed an extensive mass in the tympanic cavity extending to the mastoid...

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