Wegener's Granulomatosis: Clinical Manifestations, Differential Diagnosis, and Management of Ocular and Systemic Disease

2010 ◽  
Vol 55 (5) ◽  
pp. 429-444 ◽  
Author(s):  
Ahmad B. Tarabishy ◽  
Mark Schulte ◽  
George N. Papaliodis ◽  
Gary S. Hoffman
2020 ◽  
Vol 11 (SPL3) ◽  
pp. 1081-1084
Author(s):  
Nithyanandham Masilamani ◽  
Dhanraj Ganapathy

Wegener’s granulomatosis (WG) is a systemic disease characterized by necrotizing granulomatous inflammation of the upper and lower respiratory tract, glomerulonephritis, and vasculitis. The clinical manifestations and laboratory findings suggest an autoimmune disease resulting from a hypersensitivity reaction to some environmental agent. The purpose of the survey is for assessing the awareness about wegeners granulomatosis disease amongst dental students. A cross-sectional survey was done with questionnaire comprising a total of ten multiple-choice questions which were distributed to 100  undergraduate dental students. The questions elicited awareness about the etiology, clinical features, diagnostic tests, treatment methods and complications of wegeners granulomatosis. The responses were collected and analysed. 7% were aware of etiology of Wegeners granulomatosis, 9% were aware of clinical features of Wegeners granulomatosis, 5% were aware of diagnostic tests of Wegeners granulomatosis, 5% were aware of the treatment of Wegeners granulomatosis, and 4% were aware of complications of Wegeners granulomatosis. The awareness of Wegener's granulomatosis among dental students is inadequate. Granulomatosis of Wegener is a relatively uncommon, autoimmune condition of unknown origin. Without medication, thus many patients would then inevitably die of those same illness. Therefore, awareness and education initiatives must be pursued to enlighten students about this illness. 


1970 ◽  
Vol 5 (3) ◽  
pp. 53-67
Author(s):  
Aline Dos Santos ◽  
Ana Caroline Balducci Scafi ◽  
Luciene Azevedo Morais ◽  
Pablo Girardelli Mendonça Mesquita

RESUMOIntrodução: A Granulomatose de Wegener (GW) é uma vasculite rara e idiopática associada à presença do anticorpo Anticitoplasma de Neutrófilo (ANCA) que acomete, preferencialmente, os pequenos vasos. As manifestações clínicas são diversas, ocorrendo em mais de 90% dos casos, sintomas do trato respiratório. O comprometimento renal é tardio e preditor de mau prognóstico.  Sua morbidade a médio e longo prazo inclui insuficiência renal crônica. A probabilidade de sucesso de manutenção da função renal depende da concentração sérica de creatinina ao início do tratamento, o que indica a importância do diagnóstico e terapêutica adequada precoces. Casuística: Relata-se o caso de uma paciente do sexo feminino, 61 anos, portadora de GW com comprometimento renal avançado à apresentação não precedido por sintomas pulmonares esperados. O tratamento imunossupressor associado a plasmaferese permitiu a melhora da função renal da paciente poupando-a de tornar-se dialítica- dependente. Discussão: A paciente iniciou a doença através de insuficiência renal assintomática, com valores de função renal compatíveis com o estágio mais avançado de doença renal crônica, ultrassonografia dos rins sem alterações compatíveis e sem os sintomas respiratórios esperados. Segundo a literatura, a combinação de imunossupressores e plasmaferese associa-se à recuperação renal em três meses com sobrevivência sem necessidade de diálise por 12 meses, no caso relatado, obteve-se tal resultado em 22 dias sem a necessidade de diálise após um ano. Conclusão: Devido ao diagnóstico precoce, o tratamento adequado foi instalado rapidamente proporcionando à paciente um aumento da expectativa e da qualidade de vida, evitando dependência de terapia renal substitutiva.Palavras-Chave: Granulomatose de Wegener, Plasmaferese, Doença renal crônica.  ABSTRACTIntroduction: The Wegener's Granulomatosis (WG) is a rare and idiopathic vasculitis associated with the presence of Antineutrophil Cytoplasmic Antibody (ANCA), that affects, preferentially, the small vessels. The clinical manifestations are diverse, occurring in over 90% of cases, symptoms in the respiratory tract. Kidney damage is a late and bad prognostic predictor. Morbidity in the medium and long term includes chronic renal failure. The probability of renal function maintenance success depends on serum creatinine concentration at the beginning of treatment that indicates the importance of early diagnosis and deployment of an appropriate therapy. Case Report: We present a case of a 61-year-old female patient, carrier of GW with advanced renal impairment presentation, not preceded by expected pulmonary symptoms. The immunosuppressive treatment associated with plasmapheresis allowed the improvement of the patient’s renal function, saving her from becoming dialysis-dependent Discussion: The patient developed the disease through asymptomatic renal failure, renal function with values that are compatible with the most advanced stage of chronic kidney disease, ultrasound of the kidneys without compatible changes and without the expected respiratory symptoms. According to the literature, the combination of immunosuppressive drugs and plasmapheresis is associated with renal recovery in three months with survival without dialysis for 12 months. In this case, a result was obtained in 22 days without the need for dialysis after one year. Conclusion: Due to the early diagnosis, appropriate treatment was quickly installed giving the patient increased life expectancy and quality, preventing dependence on renal replacement therapy.Keywords: Wegener’s granulomatosis, Plasmapheresis, Chronic renal failure.


2001 ◽  
Vol 115 (1) ◽  
pp. 46-47 ◽  
Author(s):  
A. Banerjee ◽  
J. M. Armas ◽  
J. H. Dempster

Wegener’s granulomatosis is a systemic disease characterized by a granulomatous lesion that can affect any organ throughout the body.This case-report illustrates the problem posed by a patient presenting with bilateral serous otitis media with marked sensorineural hearing loss, facial nerve paralysis and an atypical serological picture. The importance of early diagnosis and the protocol for the management of a patient with an uncertain diagnosis is discussed. Due to atypical presentations, only a high index of suspicion will ensure an early diagnosis.


1996 ◽  
Vol 10 (4) ◽  
pp. 239-246
Author(s):  
Anders Cervin ◽  
Michael Dictor ◽  
Olof Kalm

The clinical course of 12 patients with sinonasal T-cell lymphoma retrospectively diagnosed using in situ hybridization for Epstein-Barr virus RNA was compared with that of 10 recently treated patients with Wegener's granulomatosis (WG) in the upper airways. In particular, we studied the presenting signs and symptoms of both diseases, which commonly offer a problem in differential diagnosis at the clinical and pathological level. A bimodal age distribution was suggested in both T-cell lymphoma and WG; five patients with T-cell lymphoma developed disease prior to 40 years of age. Four of the 12 lymphoma patients had a history of “chronic rhinitis” for several years before developing mucosal ulcerations, which were initially unilateral, as opposed to the bilateral ulcerations in early sinonasal WG. Two lymphoma patients had swelling of the nasal dorsum and cheek. In contrast to the WG patients, cases of T-cell lymphoma did not exhibit associated clinical signs of arthritis, conjunctivitis, pulmonary lesions, or nephritis in the early stage of the disease. Nine of the patients with T-cell lymphoma presenting as a sinonasal lesion developed disseminated disease, variably including infiltrates in intestine, lung, CNS, and skin. Four of these patients died from gastrointestinal complications of their disease. We conclude that unilateral ulcerative or hemorrhagic polypoid mucosal lesions in the sinonasal area are suggestive of lymphoma rather than WG, and nonspecific symptoms, at least in Western patients, may be present as early as the second or third decade of life. A biopsy specimen containing T lymphocytes positive for the EBV ribonucleoprotein EBER1 on in situ hybridization offers reliable confirmation of T-cell lymphoma and is of differential diagnostic value against WG.


2022 ◽  
Vol 1 ◽  
pp. 21-23
Author(s):  
K. S. Lakshmi Srividya ◽  
Vidyasagar P

Angioedema is a transient, non-pitting oedema that involves subcutaneous or submucosal tissue. Angioedema in children can have varied aetiology and clinical manifestations, unlike that in adults. We report a case of angioedema of penis in a child resulting from insect bite and treated successfully with anti-histamine and leukotriene inhibitor. Penile angioedema should be kept in mind as a differential diagnosis of penile swelling, as early diagnosis and management may prevent fatal complications.


2009 ◽  
Vol 123 (12) ◽  
pp. 1375-1377 ◽  
Author(s):  
J E Peters ◽  
A D Salama ◽  
P W Ind

AbstractObjective:We report a patient with a 20-year history of apparently idiopathic airways stenoses, who presented with an antineutrophil cytoplasmic antibody (ANCA) associated, acute, systemic vasculitis with necrotising glomerulonephritis, subsequently diagnosed as Wegener's granulomatosis.Methods:We present a case report and a review of the world literature on airway stenosis in Wegener's granulomatosis.Results:To our knowledge, this is the first report of Wegener's granulomatosis manifesting as local airway disease for such a prolonged period, before transforming into a systemic vasculitis.Conclusions:This case highlights the need for physicians to be alert to the possibility of Wegener's granulomatosis as a cause of apparently idiopathic airway stenosis, and to be aware that systemic disease may occur in very long-standing, limited Wegener's granulomatosis.


2014 ◽  
Vol 96 (2) ◽  
pp. 244-246 ◽  
Author(s):  
Sotirios Tsiodras ◽  
Garyfalia Poulakou ◽  
Konstantinos Leventakos ◽  
Helen Panopoulou ◽  
Antonia Elezoglou ◽  
...  

Prostatic involvement in granulomatosis with polyangiitis (GWP), formerly known as Wegener's granulomatosis, is rare, mostly arising in the context of systemic involvement. Prostatic involvement as the first manifestation of this systemic disease is exceptionally rare. We hereby present the case of a 41-year-old male patient who underwent transurethral prostate resection for what was initially diagnosed as suppurative, focally necrotizing prostatitis. Prolonged postoperative fever that did not respond to various treatments, as well as the subsequent appearance of a left pleural effusion, a left upper pulmonary lobe lesion and cutaneous nodules, led to a reevaluation of histological slides which, along with the determination of serum c-ANCA/anti-PR3 antibody levels, established the diagnosis of GWP. Physicians, and especially urologists and infectious diseases specialists, should be aware of this rare association and consider GWP in the event of nonresolving prostatitis, especially when characteristic symptoms from other systems appear.


2001 ◽  
Vol 78 (SUPPLEMENT) ◽  
pp. 283
Author(s):  
Jennifer DeVita ◽  
Robert Parks ◽  
Lisa Fanciullo ◽  
Gerald Selvin ◽  
Robert Dunphy ◽  
...  

Sign in / Sign up

Export Citation Format

Share Document