An Unusual Case of Seronegative Wegener's Granulomatosis: Acute Pancreatitis at Onset, Followed by Atrio-Ventricular Block, Neurological and Kidney Involvement (original) (raw)

Acute Pancreatitis as the First Presentation of Wegener's Granulomatosis

2000

Context Wegener's granulomatosis is a systemic vasculitis with prominent involvement of the respiratory tract and kidney. An association between acute pancreatitis and Wegener's granulomatosis is rarely reported and is even rarer as the first presentation. This can result in diagnostic difficulty and may allow severe pancreatitis to develop with potentially poor outcome. Case report We report a rare case with

Recurrent acute pancreatitis as a manifestation of Wegener's granulomatosis

Digestive Diseases and Sciences, 1990

We report a case of recurrent acute pancreatitis in a 57-year-old man with reactivation of Wegener's granulomatosis. An association between acute pancreatitis and Wegener's granulomatosis has not been reported previously. Six episodes of abdominal pain and hyperamylasemia occurred and were complicated by development of a pancreatic pseudocyst. New cavitary lung lesions typical of Wegener's granulomatosis led to treatment with increased glucocorticoid dosage, resulting in rapid resolution of the pancreatitis and pulmonary lesions. Acute pancreatitis may be a clinical manifestation of the histologic pancreatic vasculitis observed in Wegener's granulomatosis. Reactivation of Wegener's granulomatosis should be considered when a patient with the disorder develops otherwise unexplained acute pancreatitis.

Isolated primary pancreatic Wegener's granulomatosis: report of two cases

JOP : Journal of the pancreas, 2014

Chronic inflammatory vasculitis, namely Wegener's granulomatosis, is an autoimmune disease predominantly affecting the respiratory tract and rarely the mucosa of the upper or lower gastrointestinal tract. We report two cases of isolated pancreatic manifestation of Wegener's granulomatosis, mimicking a tumor. Two patients, a 57-year-old man and a 68-year-old woman, presented with isolated lesions thought to be pancreatic adenocarcinoma and both underwent surgical resection. On pathology, the lesions were found to be a granulomatous vasculitis and were considered to represent isolated pancreatic Wegener's granulomatosis, in the absence of other clinical features of the disease. Benign diseases such as Tuberculosis may mimic pancreatic tumours diagnosed on cross-sectional imaging. Wegener's granulomatosis should be included in the differential diagnosis of an isolated pancreatic mass, in the absence of other cardinal symptoms for malignancy, due to the extensive surgery...

Pancreatic mass as an initial presentation of severe Wegener's granulomatosis

Annals of gastroenterology : quarterly publication of the Hellenic Society of Gastroenterology, 2013

Acute pancreatitis or a pancreatic mass is a very rare initial presentation of Wegener's granu-lomatosis. A 62-year-old woman presented with tumor-like pancreatitis and otitis media Abdominal ultrasound and magnetic resonance suggested the presence of pancreatic tumor. Ultrasound-guided fine needle aspiration was negative. Distal pancreatic resection and splenectomy were performed and histopathology proved Wegener's vasculitis of the pancreas and spleen. Azathioprine and steroids were subsequently started and six months later the patient was asymptomatic. Involvement of the pancreas could be considered as a presenting symptom of Wegener's vasculitis.

Cardiac involvement in Wegener's granulomatosis

Heart, 1995

Wegener's granulomatosis is a systemic inflammatory disorder of unknown aetiology. The protean clinical presentations depend on the organ(s) involved and the degree of progression from a local to a systemic arteritis. The development of serological tests (antineutrophil cytoplasmic antibodies) allows easier diagnosis of a disease whose incidence is increasing. This is particularly helpful where the presentation is not classic-for example "overlap syndromes"-or where the disease presents early in a more localised form. This is true of cardiac involvement, which is traditionally believed to be rare, but may not be as uncommon as has hitherto been thought (< 44%). This involvement may be subclinical or the principal source of symptoms either in the form oflocalised disease or as part of a systemic illness. Pericarditis, arteritis, myocarditis, valvulitis, and arrhythmias are all recognised. Wegener's granulomatosis should therefore be considered in the differential diagnosis of any non-specific illness with cardiac involvement. This includes culture negative endocarditis, because Wegener's granulomatosis can produce systemic upset with mass lesions and vasculitis. Echocardiography and particularly transoesophageal echocardiography can easily identify and delineate cardiac and proximal aortic involvement and may also be used to assess response to treatment.

Wegener’s Granulomatosis with Cardiac İnvolvement

Wegener’s granulomatosis (Granulomatosis with polyangiitis) is a form of vasculitis of small-to-medium sized vessels and associated with diffuse anti-neutrophil cytoplasmic antibodies (cANCA). Cardiac involvement is not uncommon with %6-25 of unsellected patients and up to %44 of patients with severe renal involvement. We report a 23 year-old man with Wegener’s cardiomyopathy with 25% ejection fraction. The overall mortality rate of Wegener’s granulomatosis with cardiac involvement has been reported to be between 15–45%. So it is important to keep in mind that cardiac examination is a must to detect if cardiac involvement is present.

Unusual Presentations of Wegener's granulomatosis: Pitfalls in Early Diagnosis

2003

Wegener's Granulomatosis (WG) is a disorder characterized by necrotizing granulomatous vasculitis that primarily affects the upper and lower respiratory tracts and the kidneys. Although the cause is unknown, there is strong evidence that this is an autoimmune disease and immunosuppressive therapy with cyclophosphamide and corticosteroids efficiently relieves the symptoms and, prevents relapses. We report on four cases of WG that presented with unusual features. The first patient presented with vague joint pain, blackish discoloration of the skin and leg ulcers. The second patient presented as fever of unknown origin and gingival hyperplasia. The third patient presented with hearing loss while the fourth presented with arthritis, conjunctivitis and painful swelling of the ear. It is emphasized that WG is a complex disease and can involve multiple organ systems. Early recognition and institution of proper treatment are crucial for optimal outcome.

Wegener's granulomatosis: a diagnostic challenge

JPMA. The Journal of the Pakistan Medical Association, 2009

Wegener's Granulomatosis (WG) is a necrotizing granulomatous vasculitis that primarily involves small vessels in the body. Patient usually presents in the fourth to fifth decade. The clinical presentation is variable; however, majority of patients (90%) seek medical attention for nasal and sinus symptoms with or without lower respiratory symptoms of cough, dyspnoea and haemoptysis. The typical form of WG tends to involve the triad of upper and lower respiratory tract and the kidneys; while involvement of other organs like ocular, cutaneous, rheumatological, neural, gastrointestinal and lower genito-urinary tract is occasionally seen. A "limited" form with clinical findings isolated to the upper respiratory tract or lungs, occur in approximately one-fourth of cases. We present a case of Wegener's Gmnulomatosis in an eighty five year old lady who presented with an acute pneumonia-like illness. She underwent an extensive work-up to reach a definitive diagnosis.