Wegeners granulomatosis

Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, is a necrotizing vasculitis characterized by small-to-medium-sized vessel involvement and the presence of antineutrophil cytoplasmic antibodies (ANCA). We present a case of a 26-year-old Asian woman who was transferred to our center from a nearby hospital, where she presented with shortness of breath, tested positive for COVID-19, and was being managed for COVID-19 pneumonia. She also had hemoptysis, skin lesions, and left foot numbness...

Facial rejuvenation outcomes have not been well studied in patients with granulomatosis with polyangiitis (GP)-formerly Wegener granulomatosis. This report highlights a case of a 49-year-old female with a history of GP, presenting with facial aging and functional nasal concerns. The patient underwent facial rejuvenation and nasal reconstruction procedures, including primary open functional rhinoplasty, septal perforation repair with Alloderm, deep-plane rhytidectomy, platysmaplasty, nanofat grafting, and fractionated erbium laser to the face (sparing the nose and peri-incisional areas)...

Wegener's granulomatosis or Granulomatosis with polyangiitis is a rare multisystem autoimmune vasculitis disease which may become life threatening due to the various system involvement. Granular hyperplastic gingivitis, the so called "strawberry gingivitis" is a rare manifestation of this disease, yet pathognomic when present. Early diagnosis and treatment is very important.

To share a unique case of granulomatosis with polyangiitis (GPA) identified in a child with CNS involvement, specifically PRES (posterior reversible encephalopathy syndrome). Discuss this uncommon manifestation's clinical characteristics, diagnostic process, and treatment. We are currently discussing a 12-year-old female patient who presented with a chronic cough, shortness of breath, and a new-onset fever. Upon further examination, the patient was diagnosed with GPA, confirmed through positive cytoplasmic antineutrophil cytoplasmic antibodies (C-ANCA), a renal biopsy, and multiple lung cavitary lesions...

IgG4-related disease (IgG4-RD) is a fibroinflammatory condition that is characterized by storiform fibrosis, infiltration of IgG4-positive lymphocytes, obliterative phlebitis, and high IgG4 levels. Since IgG4-RD affects a wide variety of organs, a differential diagnosis must include multiple conditions. IgG4-RD is also believed to coexist with certain diseases. In recent years, case reports and case series describing the co-occurrence of IgG4-RD and ANCA-associated vasculitis (AAV) have been published. We intended to evaluate patients with IgG4-RD and AAV overlap in the literature using a case similar to one that was diagnosed and monitored in our department...

BACKGROUND: Ultrasonographic evaluation of fetal thymus size may be used to predict the adverse perinatal outcome in pregnant women with vasculitis. AIM: To compare fetal thymus size in pregnant women with vasculitis and healthy pregnant women and to evaluate whether fetal thymus size predicts the adverse perinatal outcome. METHODS: Twenty-two pregnant women with previously diagnosed vasculitis, 18 of them with Behçet's disease, three with Takayasu arteritis, and one with Wegener's granulomatosis, were included in the case group...

ABSTRAK Penyakit Granulomatosis Wegener merupakan suatu penyakit autoimun yang mengenai dinding pembuluh darah terutama pembuluh darah kecil dan sedang akibat adanya reaksi komplek imun. Sampai saat ini etiologi penyakit Granulomatosis Wegener belum diketahui secara pasti. Diagnosis Granulosis Wegener salah satunya adalah terdeteksinya antibodi cytoplasmic antineutrophilic cytoplasmic antibody (c-ANCA), akan tetapi pemeriksaan ANCA negatif atau tidak ditemukan pada kasWegener's granulomatosis is an autoimmune disease that affects the walls of small and medium-sized blood vessels due to an immune complex reaction...

Massive hemoptysis may be related to a wide spectrum of diseases whose differential diagnosis can be challenging, also due to the medical emergency condition.We present a case of a 33-year-old woman presented to our department with sudden, life-threatening hemoptysis from unknown etiology, which required a rescue pulmonary lobectomy after resuscitation maneuvers. Histology proved to be a localized Wegener granulomatosis. Our case shows that granulomatosis should always be considered among the possible, although rarer, causes of massive hemoptysis...

Granulomatous polyangiitis (GPA) is a rare systemic autoimmune vasculitis disease that is highly correlated with anti-neutrophil cytoplasmic antibodies (ANCAs). It was formerly called as "Wegener's granulomatosis." The clinical manifestations are diverse, mainly involving the upper respiratory tract, lungs, and kidneys, and this disease can involve the brain parenchyma as an isolated solid mass. Only one case has been reported thus far. To provide further information on this rare case, we report a case of GPA involving the fourth ventricle and review the relevant literature...

Granulomatosis with polyangiitis (GPA), formerly referred to as Wegener's disease, is a form of ANCA-associated vasculitis. It manifests mainly in the kidneys and the upper respiratory tract, but ocular involvement is not uncommon. In this article, four cases with ocular manifestations are presented with comprehensive photographic documentation. We describe the way to proper diagnosis, which may be long, the possible treatment, and the final outcomes. Our patients had the following ocular manifestations of GPA: retinal vasculitis, anterior necrotizing scleritis, medial orbital wall and orbital floor erosion with middle face deformation, compressive optic neuropathy due to retrobulbar inflammatory mass, and the abscess of the eyelids, inflammatory intraorbital mass causing exophthalmos and diplopia...

UNLABELLED: Granulomatosis with polyangiitis (GPA) is an ANCA-associated autoimmune disorder that typically affects small and/or medium-sized blood vessels. Being a life-threatening disease, with timely suspicion, targeted laboratory investigations, and collaborative management from the ophthalmologist and rheumatologist led to long-term remission of the disease. CASE: A 38-year-old female complaining of recurrent deep boring pain with redness in her left eye for many years, which was diagnosed as nodular scleritis with peripheral ulcerative keratitis...

Granulomatosis with polyangiitis (GPA) is an antineutrophil cytoplasmic antibody (ANCA)-associated necrotizing vasculitis of small to medium-sized blood vessels. The typical presentation is the classic triad of upper airway, pulmonary, and renal involvement. However, it can rarely present with the involvement of a single organ system known as limited granulomatosis with polyangiitis. We present a case of a 53-year-old male with chronic rhinosinusitis as the only manifestation of limited GPA...

Wegener's granulomatosis is a necrotising vasculitis affecting both arterioles and venules. The classical triad involves acute inflammation of upper airway along with inflammation of lower respiratory tract and renal involvement, however other organ system may also be affected. Our patient presented with severe unilateral earache, ear discharge, hearing loss and ipsilateral facial nerve palsy as the manifestations of the disease, which are rarely reported in medical literature1 .

Granulomatosis with polyangiitis (GPA) is a relatively rare systemic autoimmune disorder of small and medium size blood vessels affecting multiple organs with a wide range of clinical presentations. We present a 57-year-old Caucasian male who presented to the ER with midsternal chest pain. He was hospitalized for non-ST elevated myocardial infarction (NSTEMI) and later diagnosed with pauci-immune necrotizing crescentic glomerulonephritis confirmed with renal biopsy.

Antineutrophilic cytoplasmic antibody (ANCA)-associated vasculitis is a small vessel vasculitis with a positive ANCA in the serum. One of three diseases that fall under this category is granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis. This case report presents a patient with an ocular manifestation of GPA, rendering a difficult diagnosis and multi-specialty approach to managing the disease.

This report presents a rare case of scleritis with peripheral ulcerative keratitis secondary to granulomatosis with polyangiitis (GPA). A 65-year-old Caucasian male presented to a regional ophthalmology service with an atypical red eye. His immune work-up demonstrated positive anti-neutrophil cytoplasmic IgG autoantibodies (ANCA) with anti-proteinase 3 antibody (PR3) elevation. Multi-system vasculitis was discovered including lung, liver, bladder, prostate, nasal and paranasal sinuses involvement. His ocular sequelae included significant peripheral corneal thinning requiring cyanoacrylate gluing, juxtalimbal conjunctival resection, and bandage lens placement...

Granulomatosis with polyangiitis (GPA) is an autoimmune disease that affects small and medium-sized vessels. It is classically known to present with renal and respiratory tract symptoms. However, the disease can manifest in other organ systems, especially cardiovascular involvement. Though there are multiple reports of cardiac involvement in GPA, it is not commonly evaluated and is often overlooked in patients with GPA. Heart disease in GPA has a wide range of presentations ranging from subacute and silent to severe abnormalities, which can prove fatal if not identified and treated appropriately...

CASE PRESENTATION: A 63-year-old man presented with fever, thoracalgia, weight loss, diffuse lymphadenopathy, and a massive pleural effusion. Extensive laboratory and radiologic investigations for possible autoimmune, infectious, hematologic, and neoplastic conditions all resulted negative. A lymph node biopsy showed a granulomatous necrotizing lymphadenitis, suspicious for tuberculosis. Although mycobacterium tuberculosis (MT) was never isolated and tuberculin skin test resulted negative, diagnosis of extrapulmonary tuberculosis was made and anti-tubercular therapy was started...

Granulomatosis with polyangiitis (GPA) is a rare disease with a prevalence of about three in 100,000 persons in the United States. GPA is an antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis affecting predominantly small-sized vessels. It can present with localized or systemic symptoms with multiple organ involvement, thus making diagnosis challenging. Common skin lesions in GPA are palpable purpura, petechiae, ulcers, and livedo reticularis. These lesions usually have underlying vasculitis with or without granuloma on histology findings...

BACKGROUND: CT-P10 was the first licensed rituximab biosimilar. This Korean post-marketing surveillance study evaluated CT-P10 safety and effectiveness in approved indications. RESEARCH DESIGN AND METHODS: This prospective, open-label, observational, phase 4 study collected routine clinical practice data across 27 centers in the Republic of Korea. Patients received their first CT-P10 treatment, per prescribing information, for non-Hodgkin's lymphoma (NHL), chronic lymphocytic leukemia (CLL), rheumatoid arthritis (RA), granulomatosis with polyangiitis (GPA), or microscopic polyangiitis (MPA) during the surveillance period (16 November 2016-15 November 2020)...