Wegeners granulomatosis

BACKGROUND: Granulomatosis with polyangiitis (Wegener's) causes progressive nasal collapse, nasal obstruction, and central face deformity. It is not known whether cartilaginous nasal reconstruction should be performed immediately or delayed (after disease 'burn-out'). OBJECTIVES: For Wegener's nasal collapse to: (1) Assess the functional and aesthetic outcomes following immediate versus delayed nasal reconstruction; (2) Measure the impact of psychosocial well-being (anxiety, depression, social isolation) in immediate versus delayed nasal reconstruction...

BACKGROUND: Granulomatosis with Polyangiitis (GPA) is a rare multi-system autoimmune disorder that may present with cardiac manifestations that are often under-recognized. In this report, we discuss a usual case of a patient who presented as a cardiac emergency with recurrent ST elevation and discuss the approach and management. CASE SUMMARY: A 44-year-old man presented with two episodes of chest pain associated with ST-segment elevation on 12-lead ECG. Under investigation over the past several weeks for fatigue, nasal congestion, and red eyes, his first presentation was associated with widespread ST-segment elevation and an echogenic myocardium suggestive of myocarditis that was confirmed on cardiac MRI...

Sarcoidosis, a multifaceted granulomatous disease primarily affecting the lungs, occasionally presents in atypical locations. Lacrimal gland involvement, though rare, poses distinct diagnostic challenges. This case report details a 52-year-old female with bilateral lacrimal gland swelling initially suggestive of metastatic tumor due to a history of breast cancer. Subsequent investigations, including CT and MRI, unveiled pulmonary sarcoidosis. Discussion emphasizes the diverse ocular manifestations of sarcoidosis, with lacrimal gland participation potentially indicating early stages...

BACKGROUND: Wegener's disease is an autoimmune condition affecting the respiratory tract and kidneys. Mental health assessment is crucial due to the impact of psychological disorders on the immune system. Despite this, there is limited community-based research on psychiatric disorders and personality traits among patients with Wegener's disease. OBJECTIVE: This study aimed to investigate the prevalence of psychiatric disorders and examine the predictive role of personality traits among patients with Wegener's disease...

Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, is a rare and potentially life-threatening autoimmune disease characterized by antineutrophil cytoplasmic antibody (ANCA)-associated vasculature inflammation. It presents as a systemic autoimmune disease with necrotizing granulomatous inflammation and pauci-immune small vessel vasculitis. This case initially posed a diagnostic challenge due to its atypical presentation and was misdiagnosed as hypersensitivity pneumonitis. The avian precipitin screening assay was positive in our patient, which may be consistent with bird breeder's lung disease or a non-specific reactivity of the chicken antigen test...

Granulomatosis with polyangiitis (GPA) is a rare vasculitis that can pose a significant mortality risk given its multiorgan involvement and is the most common of the three anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitides. Cardinal pathological features include necrotizing granulomas of the respiratory tract, small and medium vessel vasculitis, and glomerulonephritis. Early treatment is imperative to reduce permanent organ damage such as end-stage kidney disease. We describe the first case of GPA relapse 38 years after the initial pulmonary presentation...

BACKGROUND: Granulomatosis with polyangiitis (GPA) is characterised by granulomatous inflammation and small-to-medium vessel necrotising vasculitis, mainly affecting respiratory tract and kidneys. Renal involvement presenting as tumour-like lesions poses diagnostic and treatment challenges. METHODS: Following the observation of a GPA patient presenting with multiple renal tumour-like lesions, we conducted a systematic literature review on MEDLINE/PubMed, EMBASE, and Cochrane databases...

A Gentleman in his 40s presented to us with left-sided hemifacial pain and nasal discharge for the last three days. He has been on immunosuppressive therapy for Wegener's Granulomatosis for over five years. Crusting was seen in the Left middle meatus upon clinical examination. Based on the post-covid status, steroid therapy, KOH mount, and radiologic imaging, a provisional diagnosis of Sinonasal mucormycosis was considered. He was posted for emergency debridement followed by Liposomal Amphotericin B and steroid therapy...

Wegener's granulomatosis (WG), also termed granulomatosis with polyangiitis, is a vasculitic condition with numerous systemic manifestations. It is mainly a pauci-immune vasculitis which involves small and medium-sized vessels. Herein, we report a case of a 72-year-old female with a background history of type 2 diabetes mellitus and hypertension. She presented to the hospital with recurrent nasal congestion, headache, and intermittent facial pain for two months' duration. At the initial presentation, she was diagnosed with pansinusitis, for which she was treated with antibiotics and was discharged...

Granulomatous lobular mastitis (GLM) is a rare inflammatory breast disease with unknown etiology, characterized by non-caseous granulomatous inflammation of the lobules, which infiltrate lymphocytes, neutrophils, plasma cells, monocytes, and eosinophils may accompany. GLM is often misdiagnosed as breast cancer due to the lack of specificity in clinical and imaging examinations, and therefore histopathology is the main basis for confirming the diagnosis. This review provides an overview of the pathological features of granulomatous lobular mastitis and cystic neutrophil granulomatous mastitis (CNGM, a pathologic subtype of GLM)...

Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis (WG), is a condition marked by necrotizing vasculitis of the small-medium vessels that results in necrotizing granulomatous inflammation. Splenic involvement in GPA is a potentially life-threatening consequence of connective tissue disease and is rarely described as the main presenting feature. We present a case of a patient with perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA) who experienced spontaneous splenic rupture...

Introduction  Wegener granulomatosis (WG) appears with clinical symptoms, including recurrent respiratory infection, renal manifestations, and nonspecific systemic symptoms. Objective  To study the clinical manifestations of WG in Iranian ethnicities, and data on 164 patients were recorded from 2013 to 2018. Methods  The data included demographics, symptoms, and the Birmingham Vasculitis Activity Score (BVAS). The symptoms involved the following sites: the nose, sinus, glottis, ears, lungs, kidneys, eyes, central nervous system, mucous membranes, skin, heart, stomach, intestine, as well as general symptoms...

Objective: To determine the odds of head and neck cancer (HNC) in patients with a concurrent or prior diagnosis of granulomatosis with polyangiitis (GPA). Methods and Materials: The TriNetX Analytics Network, a federated research platform that aggregates de-identified electronic health record data of over 130 million patients worldwide, was queried for patients with at least one ICD-10 encounter diagnosis of GPA. Patients within this group with an encounter diagnosis of cancer of the sinonasal, oral cavity, oropharynx, nasopharynx, and larynx concurrent or after the initial encounter diagnosis of GPA were recorded and compared to a standardized control population to determine odds ratios with a 95% confidence interval (CI)...

RATIONALE: Granulomatosis with polyangiitis (GPA) is a systematic autoimmune disease. The typical clinical involvement of GPA entails the upper and lower respiratory tracts, and the kidneys. Gastrointestinal (GI) involvement is uncommon and unless detected and treated promptly, may lead to life-threatening complications such as perforation. We aim to review all available publications since the first description in 1982 dealing with GI perforation in patients with Wegener granulomatosis and draw attention to this serious situation...

In this article, we present an unusual case of granulomatosis with polyangiitis (GPA) in a 41-year-old man. The initial presentation of the disease was atypical, with persistent fever, cough, and fatigue, accompanied by elevated inflammatory markers in association with a large, solitary lung lesion observed at the chest X-ray. Despite the presence of an initial radiological picture suggesting pneumonia, the lack of response to antibiotics necessitated a more in-depth evaluation. The diagnosis was confirmed through a lung biopsy and serological tests positive for anti-neutrophil cytoplasmic antibodies (c-ANCA)...

This case report presents an uncommon instance of spontaneous rupture of the renal artery due to Wegner Granulomatosis which is an infrequent autoimmune disorder distinguished by the presence of granulomatous inflammation, primarily impacting the upper and lower respiratory tracts, along with renal involvement. The occurrence of spontaneous renal artery rupture in patients with Wegener's granulomatosis is a rare complication, as evidenced by the limited number of reported cases in the existing literature. The present case underscores the difficulties associated with the diagnosis and treatment of an uncommon complication while emphasizing the significance of timely intervention in achieving a positive outcome...

Primary nasal tuberculosis (TB) is a rare disease even in areas with high TB burden, possibly attributed to the protective mechanism of sinonasal mucosa. Its symptoms are subtle and can be mistaken for other granulomatous inflammatory conditions. We would like to report a case of a 70-year-old Indian lady who underwent a successful left endoscopic dacryocystorhinostomy three years ago and presented with recurrent left epiphora. During nasal endoscopy, multiple ulcerative masses with crusting were detected over the left nasal vestibule, anterior nasal septum, left inferior, and middle turbinate...

Granulomatosis with polyangiitis (GPA), formerly Wegener's granulomatosis, is a small- and medium-vessel vasculitis with characteristic cutaneous morphologic presentation and systemic involvement. Most patients have palpable purpura at some point in their disease course, but this is not always the presenting manifestation. This autoimmune disorder can affect a range of organs, with the upper and lower respiratory tract, kidneys, and nervous system being commonly implicated, while gastrointestinal and cardiac involvement is less frequent...

OBJECTIVE: Granulomatosis with polyangiitis is associated with otolaryngologic complaints in 70-95 % of cases, with the most common being serous otitis media. In rare cases, patients may experience facial nerve palsy in conjunction with otologic or nasal symptoms; and, often, initially present to an otolaryngologist. It is important for healthcare professionals to be able to recognize the nuisances of facial nerve palsy as a potential presentation of granulomatosis with polyangiitis...

Gingival hyperplasia may arise due to microbial-related local irritation, mouth breathing, drug administration, genetic disorders, leukemia, Wegener granulomatosis, Crohn's disease, and sarcoidosis. The background may be inflammatory, fibrotic, or combined. The aim of this study was to present the diagnostic procedure for a case of gingival enlargement, which was the only sign of a severe systemic disease in a young male adult. The patient was referred, complaining of persistent gingival bleeding in the posterior area of the maxilla, bilaterally...