-
Clinical Journal of the American... Oct 2017ANCA vasculitis has an associated autoimmune response that produces ANCAs that induce distinct pathologic lesions. Pauci-immune necrotizing and crescentic GN is a... (Review)
Review
ANCA vasculitis has an associated autoimmune response that produces ANCAs that induce distinct pathologic lesions. Pauci-immune necrotizing and crescentic GN is a frequent component of ANCA vasculitis. ANCA vasculitis is associated with ANCA specific for myeloperoxidase (MPO-ANCA) or proteinase 3 (PR3-ANCA). A diagnosis of ANCA vasculitis should always specify the serotype as MPO-ANCA positive, PR3-ANCA positive, or ANCA-negative. To fully characterize a patient, the serotype also should be accompanied by the clinicopathologic variant if this can be determined: microscopic polyangiitis, granulomatosis with polyangiitis (Wegener), eosinophilic granulomatosis with polyangiitis (Churg-Strauss), or renal-limited vasculitis. ANCA vasculitis is most prevalent in individuals >50 years old. There are racial/ethnic and geographic influences on the prevalence, serotype frequencies, and clinicopathologic phenotypes. There is clinical, , and animal model evidence that ANCAs cause disease by activating neutrophils to attack small vessels. Immunomodulatory and immunosuppressive therapies are used to induce remission, maintain remission, and treat relapses. Over recent years, there have been major advances in optimizing treatment by minimizing toxic therapy and utilizing more targeted therapy.
Topics: Animals; Antibodies, Antineutrophil Cytoplasmic; Churg-Strauss Syndrome; Female; Glomerulonephritis; Granulomatosis with Polyangiitis; Humans; Immunosuppressive Agents; Male; Microscopic Polyangiitis; Middle Aged; Myeloblastin; Peroxidase; Prevalence; Recurrence; Remission Induction; Risk Factors; Treatment Outcome
PubMed: 28842398
DOI: 10.2215/CJN.02500317 -
British Journal of Hospital Medicine... Mar 2023Orofacial granulomatosis is a chronic relapsing-remitting inflammatory condition that shares a similar phenotypic presentation to some other granulomatous diseases,... (Review)
Review
Orofacial granulomatosis is a chronic relapsing-remitting inflammatory condition that shares a similar phenotypic presentation to some other granulomatous diseases, particularly Crohn's disease. However, subtle clinical and pathological differences justify it as a separate disease entity. Previous studies have assessed the effectiveness of interventions used in the management of orofacial granulomatosis. This article reviews the management options available. A literature search was conducted to identify studies, in English, which assessed the effect of non-pharmacological and pharmacological interventions in the treatment of orofacial granulomatosis. The interventions were categorised into dietary modification, pharmacological (topical, intralesional and systemic therapy), surgery and psychological. A combination of interventions is often required to effectively manage each patient. There is convincing evidence that diet plays a role in disease severity. In patients where dietary manipulation alone is unsuccessful, topical, intralesional and/or systemic treatment may be considered to manage the condition.
Topics: Humans; Granulomatosis, Orofacial; Crohn Disease; Administration, Cutaneous
PubMed: 36989148
DOI: 10.12968/hmed.2022.0416 -
International Journal of... Jun 2016Granulomatosis with polyangiitis (GPA), formerly Wegener's granulomatosis (WG), is an uncommon immunologically mediated systemic small-vessel vasculitis that is... (Review)
Review
Granulomatosis with polyangiitis (GPA), formerly Wegener's granulomatosis (WG), is an uncommon immunologically mediated systemic small-vessel vasculitis that is pathologically characterised by an inflammatory reaction pattern (necrosis, granulomatous inflammation and vasculitis) that occurs in the upper and lower respiratory tracts and kidneys. Although the aetiology of GPA remains largely unknown, it is believed to be autoimmune in origin and triggered by environmental events on a background of genetic susceptibility.In Europe, the prevalence of GPA is five cases per 100,000 population, with greater incidence in Northern Europe. GPA can occur in all racial groups but predominantly affects Caucasians. Both sexes are affected equally. GPA affects a wide age range (age range, 8-99 years).Granulomatosis with polyangiitis is characterised by necrotising granulomatous lesions of the respiratory tract, vasculitis and glomerulonephritis. Classically, the acronym ELK is used to describe the clinical involvement of the ear, nose and throat (ENT); lungs; and kidneys. Because the upper respiratory tract is involved in 70-100% of cases of GPA, classic otorhinolaryngologic symptoms may be the first clinical manifestation of disease. The nasal cavity and the paranasal sinuses are the most common sites of involvement in the head and neck area (85-100%), whereas otological disease is found in approximately 35% (range, 19-61%) of cases.Diagnosis of GPA is achieved through clinical assessment, serological tests for anti-neutrophil cytoplasmic antibodies (ANCA) and histological analysis. The 10-year survival rate is estimated to be 40% when the kidneys are involved and 60-70% when there is no kidney involvement.The standard therapy for GPA is a combination of glucocorticoids and cyclophosphamide. In young patients, cyclophosphamide should be switched to azathioprine in the maintenance phase.A multidisciplinary approach, involving otorhinolaryngologists, oral and maxillofacial surgeons, oral physicians, rheumatologists, renal and respiratory physicians, and ophthalmologists, is necessary for the diagnosis and therapeutic treatment of GPA. ENT physicians have a determining role in recognising the early onset of the disease and starting an appropriate therapy.
Topics: Azathioprine; Cyclophosphamide; Glucocorticoids; Granulomatosis with Polyangiitis; Humans; Incidence; Inflammation; Kidney; Respiratory System
PubMed: 26684637
DOI: 10.1177/0394632015617063 -
European Annals of Otorhinolaryngology,... Mar 2020
Topics: Aged; Female; Granulomatosis with Polyangiitis; Humans; Necrosis; Sinusitis
PubMed: 31982361
DOI: 10.1016/j.anorl.2020.01.003 -
The Israel Medical Association Journal... Feb 2019
Topics: Granulomatosis with Polyangiitis; Humans; Immunoglobulin G4-Related Disease
PubMed: 30772965
DOI: No ID Found -
Head and Neck Pathology Sep 2019Granulomatous lesions of the orofacial region are a heterogeneous group of disorders characterized by a granulomatous reaction to a variety of stimuli. Infectious... (Review)
Review
Granulomatous lesions of the orofacial region are a heterogeneous group of disorders characterized by a granulomatous reaction to a variety of stimuli. Infectious agents, foreign material, systemic inflammation and metabolic disorders can all be associated with granulomatous inflammation. In the orofacial region primary causes of granulomatosis include foreign body reaction, delayed hypersensitivity to topical agents and idiopathic orofacial granulomatosis. Secondary causes of granulomas include infectious agents, sarcoid, and Crohn disease. For this review, infectious causes of orofacial granulomatosis (OFG) including bacteria, parasites and fungi will not be discussed.
Topics: Granulomatosis, Orofacial; Humans
PubMed: 30627963
DOI: 10.1007/s12105-018-00997-w -
BMJ Case Reports Mar 2021COVID-19 and granulomatosis with polyangiitis share many clinical and radiological features, making it challenging for clinicians to distinguish between the two. In this...
COVID-19 and granulomatosis with polyangiitis share many clinical and radiological features, making it challenging for clinicians to distinguish between the two. In this case report, we describe a patient who was diagnosed with COVID-19 in October 2020. One month later, she presented with persistent fatigue, shortness of breath and anaemia with worsening renal functions, found to have elevated antineutrophil cytoplasmic antibodies and antiproteinase 3 antibodies, and diagnosed with granulomatosis with polyangiitis.
Topics: Anti-Inflammatory Agents; Biopsy; COVID-19; Diagnosis, Differential; Female; Granulomatosis with Polyangiitis; Humans; Immunologic Factors; Kidney; Methylprednisolone Hemisuccinate; Middle Aged; Rituximab; SARS-CoV-2
PubMed: 33737283
DOI: 10.1136/bcr-2021-242142 -
Obstetric Medicine Jun 2020The association of granulomatosis with polyangiitis and pregnancy is rare and therapeutic options are limited by the risk of teratogenicity and fetotoxicity. There is a...
The association of granulomatosis with polyangiitis and pregnancy is rare and therapeutic options are limited by the risk of teratogenicity and fetotoxicity. There is a paucity of published literature to guide clinical decision-making in these cases. We report the case of a 26-year-old woman with no medical history who presented at 21 weeks of gestation with a bilateral sudden loss of hearing and erosive rhinitis. The diagnosis of granulomatosis with polyangiitis was confirmed radiologically and biologically. Corticosteroids were not enough to stabilize the disease and she received intravenous immunoglobulins with remission. A successful delivery of a healthy male newborn was done at 36 weeks. A review of all published literature on granulomatosis with polyangiitis in pregnancy between 1970 and 2017 is presented. : Not applicable.
PubMed: 32714439
DOI: 10.1177/1753495X18822581 -
Orbital granulomatosis with polyangiitis (Wegener granulomatosis): clinical and pathologic findings.Archives of Pathology & Laboratory... Aug 2014The pathology of granulomatosis with polyangiitis (GPA), formerly Wegener granulomatosis, typically features a granulomatous and sometimes necrotizing vasculitis... (Review)
Review
The pathology of granulomatosis with polyangiitis (GPA), formerly Wegener granulomatosis, typically features a granulomatous and sometimes necrotizing vasculitis targeting the respiratory tract and kidneys. However, orbital involvement occurs in up to 60% of patients and is frequently the first or only clinical presentation in patients with systemic or limited forms of GPA. Orbital GPA can cause significant morbidity and potentially lead to complete loss of vision and permanent facial deformity. Fortunately, GPA is highly responsive to medical treatment with corticosteroids combined with cyclophosphamide or, more recently, rituximab. Therefore, it is imperative for this disease to be accurately diagnosed on orbital biopsy and distinguished from other histologically similar orbital lesions. Herein, we review the clinical and pathologic findings of orbital GPA, focusing on the differentiation of this disease from other inflammatory orbital lesions.
Topics: Adrenal Cortex Hormones; Antibodies, Monoclonal, Murine-Derived; Biopsy; Cyclophosphamide; Diagnosis, Differential; Drug Therapy, Combination; Early Diagnosis; Granulomatosis with Polyangiitis; Humans; Immunosuppressive Agents; Microvessels; Orbit; Orbital Diseases; Prognosis; Rituximab
PubMed: 25076302
DOI: 10.5858/arpa.2013-0006-RS -
Dermatology (Basel, Switzerland) 2021To systematically review all cases of orofacial granulomatosis (OFG) and evaluate the association between OFG and Crohn disease (CD).
BACKGROUND
To systematically review all cases of orofacial granulomatosis (OFG) and evaluate the association between OFG and Crohn disease (CD).
SUMMARY
This review was conducted according to PRISMA guidelines and a search of the PubMed, MEDLINE, and Embase databases, and the Cochrane Library in March 2020, using keywords and MeSH terms associated with "orofacial granulomatosis," "Crohn disease," and their variants, with no language restrictions and across all age groups. All relevant articles were accessed in full text. Single case reports and articles on sarcoidosis, allergy, ulcerative colitis, and infectious diseases were excluded from the analysis.
RESULTS
We retrieved 507 reports on OFG. The mean age at onset was 23.3 years (range 2-89 years). A total of 240 (47.3%) females and 267 (52.6%) males were included. CD was present in 93 children aged <16 years (68.3%) and in 43 adults (31.9%). In most cases, the OFG appeared before the CD. The most common clinical manifestations were intraoral mucosa abnormalities (n = 251; 49.5%), lower-lip swelling (n = 249; 49.1%), upper-lip swelling (n = 227; 44.7%), and gingivae (n = 193; 38.7%). Patients with concurrent CD were more likely to experience involvement of the buccal sulcus. Key Messages: OFG presents primarily as a solo entity. The OFG that was associated with CD was present in 93 children aged under 16 years (68.3%) and in 43 adults (31.9%). Childhood onset of OFG carries with it a higher risk of developing CD.
Topics: Crohn Disease; Granulomatosis, Orofacial; Humans
PubMed: 33582676
DOI: 10.1159/000513446