Esophageal involvement in Wegener's granulomatosis: a case report and review of the literature.

Wegener's granulomatosis is characterized by a granulomatous arteritis involving the upper and lower respiratory tracts, progressive glomerulonephritis and systemic symptoms attributable to small vessel vasculitis. Although multisystemic manifestations are frequent, involvement of the gastrointestinal tract is uncommon. Cases have been reported of intestinal perforation, ulceration and hemorrhage. A patient whose initial presentation of Wegener's granulomatosis was odynophagia secondary to esophageal vasculitis is described. Endoscopy revealed multiple punched out ulcerations in the esophagus, which resolved with standard therapy for systemic Wegener's granulomatosis. There are only two previous reports of symptomatic esophageal vasculitis in patients with Wegener's granulomatosis. These reports illustrate the need to consider odynophagia as a reflection of disease activity as opposed to complications of immunosuppressive therapy.     

韦格纳肉芽肿病23例临床分析

目的分析韦格纳肉芽肿病(Wegener granulomatosis，WG)的临床和病理特点，提高对本病的认识。方法对23例确诊的WG病人的临床表现、辅助检查及病理特点进行回顾性分析。结果发病年龄20．57岁，平均37．2岁，平均病程5．8个月。首发症状以上呼吸道为主(48％)，可累及多个系统或器官，肺脏受累87％，肾脏受累78%。胞质型抗中性粒细胞胞质抗体(cANCA)阳性率100%，病理表现为坏死性肉芽肿性炎症、炎细胞浸润的血管炎。结论WG的临床表现复杂多样，有多系统或器官病变的症候群，以上、下呼吸道及肾脏受累最多见：病理特点为坏死性肉芽肿和血管炎。     

Silent ischemic heart disease in patient with Wegener's necrotizing glomerulonephritis

Wegener's granulomatosis is a necrotizing vasculitis disorder that usually presents with clinical involvement of the upper and lower respiratory tract in association with renal disease. Although Wegener's granulomatosis can affect other systems, such as the eye, skin, joints, muscle, nerves, gastrointestinal tract and heart, cardiac involvement is traditionally believed to be rare, even though coronary vasculitis can be demonstrated at postmortem examination. We report a patient who has both respiratory and renal involvement who died unexpectedly following a silent myocardial infarct after a period of clinical improve induced by treatment with cyclophosphamide and prednisone.     

Acute upper gastrointestinal haemorrhage and colitis: an unusual presentation of Wegener's granulomatosis

Wegener's granulomatosis is a rare necrotizing vasculitis usually affecting the respiratory tract and kidneys. The aetiology is unknown and it usually occurs in patients over the age of 40. Involvement of the gastrointestinal tract in Wegener's granulomatosis is relatively rare and usually occurs long after the onset of initial symptoms. Acute colitis as a presenting feature of Wegener's granulomatosis is very rare with only a few reports in the literature. We describe a young woman who presented initially to hospital with gastrointestinal features and then developed a severe colitis and severe gastrointestinal haemorrhage. This preceded the development of respiratory tract features with severe pulmonary haemorrhage, haemoptysis and the development of rapidly progressive renal failure and nasal septal perforation. Following treatment with intravenous steroids and cyclophosphamide, gastrointestinal symptoms and signs improved dramatically, as did her pulmonary disease. She still remains dialysis dependent, due to end-stage renal disease secondary to glomerulonephritis.     

Wegener's granulomatosis: clinical features and prognosis in 37 patients

:Thirty-seven patients (21 female, 16 male) with Wegener's granulomatosis (WG)were reviewed. Patients were followed for a mean six years after diagnosis; 14 were followed for more than seven years. The clinical features were similar to those in previous studies. In this series, only 13 patients (35%) had renal disease at presentation and the cumulative incidence of renal involvement was 51%. Thirty-one patients received treatment which included cyclophosphamide (CP). The case fatality rate of the six patients not treated with CP was 83% (five deaths). By contrast, all CP treated patients improved and 21 (68%) had complete remissions. Nine (29%) were in complete remission for a mean 4.9 years after discontinuing all treatment. Two were disease free for over ten years. The actuarial probability of survival for these patients was 97% at one year and 71% at ten years. Only three CP treated patients (10%) progressed to end-stage renal disease. The case fatality rate was 26% (eight patients) and sepsis was the cause of death in five. Fourteen patients (45%) treated with CP had at least one relapse of vasculitis and seven (23%) had multiple (two or more) relapses. These data indicate that CP is effective in inducing remissions and prolonging survival in patients with WG; however, relapses are frequent. (Aust NZ J Med 1993; 23: 168–175.)     

Intestinal perforation in Wegener's granulomatosis.

Wegener's granulomatosis is a disease characterised by a necrotising vasculitis and granulomatous inflammation. The localised form involves the upper and/or lower respiratory tracts while in the commoner generalised form there is a widespread necrotising vasculitis and renal involvement. Intestinal involvement, which may be severe, has been detected at necropsy in 24% of cases. Gastrointestinal symptoms are uncommon, however, none being mentioned in a recent series of 87 cases. There has been only one reported case of intestinal perforation secondary to Wegener's granulomatosis. We report a case of small and large bowel perforations in a patient with Wegener's granulomatosis.     

Wegener's granulomatosis in North India

Summary Eleven patients with Wegener's granulomatosis were seen at this Institute over a period of 20 years. There were six men and five women. The average age of presentation was 38.3 years, and the mean duration of symptoms was 10.5 months. Constitutional symptoms (82%), cough (82%), ocular symptoms (64%), arthralgias (55%), rhinorrhoea (55%), haemoptysis (45%), nasal granuloma (45%), otorrhoea (36%), sinusitis (36%), skin lesions (27%), and renal failure (27%) were the clinical manifestations encountered. All patients had an elevated ESR, and 55% had leucocytosis. Proteinuria and haematuria were observed in 64% and 55% respectively. Chest radiographs were abnormal in 82%. In four patients the disease had a fulminant course and the patients died before adequate treatment was given. Two patients received corticosteroids alone and have since been lost to follow up. Five (45%) received adequate cytotoxic therapy and have done well for 8–46 months (mean, 24.8 months) after diagnosis. Wegener's granulomatosis in India is apparently similar to that seen elsewhere, but the high incidence of tuberculosis interferes with early diagnosis and treatment.     

Sinonasal NK/T-cell lymphoma mimicking Wegener's granulomatosis: a case report

In Western population, sinonasal malignant lymphoma is rare and constitutes 1.5% of all non-Hodgkin lymphoma (NHL) and 2.2% of extranodal lymphomas. Wegener's granulomatosis (WG) is the necrotizing vasculitis of small arteries and veins. WG is characterized by granulomatous vasculitis and involves the upper and lower respiratory tract together with glomerulonephritis. But there are some forms of WG named limited WG that involves the upper respiratory tract only without glomerulonephritis and even seronegative without renal involvement. Herein, we present a typical WG with isolated sinonasal tract involvement with clinical, and radiological findings with the final diagnosis of NK/T-cell angiocentric lymphoma by the repeated biopsies. Since both diseases have same clinical and radiological findings differential diagnosis may be difficult.     

Wegener's granulomatosis mimicking paraneoplastic syndrome

Wegener's granulomatosis is a distinct clinico-pathological entity characterised by a triad of upper and lower respiratory disease and renal involvement, although atypical presentations can be seen. These patients characteristically have small vessel vasculitis and or granulomatous vasculitis and are usually anti-neutrophilic cytoplasmic antibody (ANCA) positive. We present a case of Wegener's granulomatosis that clinically mimicked a lung neoplasm with a paraneoplastic syndrome. Biopsy and histopathological evaluation of a readily accessible subcutaneous nodule showed small vessel vasculitis. Correlation with clinical data and ANCA positivity led to a definite diagnosis.     

Severe glomerulonephritis with late emergence of classic Wegener's granulomatosis. Report of 4 cases and review of the literature

We have analyzed an unusual group of 19 patients (15 previously reported) with Wegener's granulomatosis, who presented with severe glomerulonephritis and developed diagnostic respiratory lesions only after 4 to 78 months. Necrotizing glomerulonephritis, often with crescents, and rarely with vasculitis, was the predominant renal lesion. Wegener's granulomatosis was unsuspected initially, since systemic manifestations, such as fever, arthralgias, malaise, and even pulmonary hemorrhage, were nonspecific or transient, and because renal biopsy findings resembled those seen in microscopic polyarteritis or idiopathic crescentic nephritis. Despite therapy, usually with corticosteroids, only 4 patients maintained adequate renal function. Most patients were receiving chronic dialysis when respiratory involvement developed. Cavitary nodular pulmonary infiltrates were seen in 12 of the 17 patients with lung involvement, and otorhinological disease occurred in 10 patients. Arthralgias, fever, and cough, with or without hemoptysis, were common. Wegener's granulomatosis was diagnosed by lung biopsy in 15 cases and by nasal biopsy in 4. Specific treatment was required for the respiratory disease and was delayed in many patients, because of lack of awareness that Wegener's granulomatosis may present with primary glomerulonephritis and become active during chronic renal failure or dialysis. Nevertheless, all but 1 patient eventually responded to treatment, although 3 additional patients died of late complications.     

Clinical features and outcome of pediatric Wegener's granulomatosis

OBJECTIVE: Wegener's granulomatosis (WG) is a predominantly small-vessel vasculitis associated with antineutrophil cytoplasmic antibodies (ANCAs). There are few reports describing its clinical features and outcome in children. We report on the experience at a single tertiary referral center over 21 years. METHODS: We conducted a retrospective chart review of all patients diagnosed with WG at The Hospital for Sick Children between 1984 and 2005. RESULTS: Twenty-five patients were identified. Median age at diagnosis and median followup were 14.5 years and 32.7 months, respectively. Male-to-female ratio was 1:4. Median duration of symptoms before diagnosis was 2 months. Of 22 patients, 21 were ANCA positive during their disease course (classic ANCA 78.9%). Constitutional symptoms were the most common clinical feature at presentation (24 of 25). Glomerulonephritis was present in 22 patients at presentation. Only 1 of 11 patients who presented with or developed renal impairment had normalization of serum creatinine. Upper airway involvement occurred in 21 patients at presentation and 24 over followup; only 1 had subglottic stenosis. Twenty patients had initial pulmonary involvement, most commonly nodules (44%) and pulmonary hemorrhage (44%). Five patients required ventilation for pulmonary hemorrhage. Four patients (16%) had venous thrombotic events (VTEs). Treatment included prednisone (100%), cyclophosphamide (76%), azathioprine (40%), and methotrexate (32%). CONCLUSION: Pediatric WG typically presents in adolescence and has a female predominance. Glomerulonephritis and pulmonary disease are common at diagnosis and frequently present as a pulmonary-renal syndrome. Loss of renal function is common and rarely completely reversible. As in adults, children with WG are at risk of VTEs.     

Unusual renal manifestations of Wegener's granulomatosis. Report of two cases

Two patients with previously undescribed renal manifestations of Wegener's granulomatosis are described. A 24 year old man, who presented with typical necrotizing granulomatous sinus disease and cavitary pulmonary lesions, had multiple bilateral renal arterial aneurysms demonstrated angiographically. One of these aneurysms ruptured, leading to a massive perinephric hematoma. The bleeding artery was successfully occluded with Gelfoam embolization, thereby obviating the need for nephrectomy. A 60 year old woman presented with glomerulonephritis and mononeuritis multiplex two years before the development of classic necrotizing granulomatous inflammation of her sinuses and nose, along with pulmonary nodules. In addition, her left ureter became obstructed due to necrotizing vasculitis of the periureteral vessels. Both patients responded dramatically to cyclophosphamide therapy. The diagnosis of Wegener's granulomatosis should be considered in patients who present with multiple renal aneurysms, a spontaneous perinephric hematoma, necrotizing glomerulitis or ureteral obstruction due to vasculitis, even though the characteristic granulomatous respiratory involvement may be absent at that time. It is important to recognize these unusual renal manifestations as features of Wegener's granulomatosis because of the therapeutic efficacy of cytotoxic immunosuppressive agents in this disease.     

Unusual cardiac complications of Wegener's granulomatosis

Wegener's granulomatosis most commonly involves the sinuses, lungs and kidneys with necrotizing granulomatous vasculitis. In 12 percent of a large series of patients with Wegener's granulomatosis there was cardiac involvement, largely manifested by pericarditis and coronary arteritis. We present three patients with this disease who developed unusual cardiac complications. Patient 1 had renal failure requiring hemodialysis, pericardial tamponade requiring pericardiocentesis, and later developed constrictive pericarditis requiring pericardiectomy. Patient 2 developed pericarditis and high grade atrioventricular block, and patient 3 developed pericarditis and atrial tachycardia resistant to pharmacologic and transesophageal atrial pacing methods. All three patients greatly improved with cyclophosphamide therapy. The rhythm disturbances seen in patients 2 and 3 were attributed to coronary arteritis. The renal failure in patient 1 was due to Wegener's granulomatosis, but whether the constrictive pericarditis was due to uremic pericarditis or the pericarditis of Wegener's granulomatosis is uncertain. As patients with Wegener's granulomatosis live longer with cyclophosphamide therapy and because inpatient arrhythmia monitoring and recording has become more widespread, these uncommon manifestations of Wegener's granulomatosis may be seen more often.     

Two cases of Wegener's granulomatosis with pancreatic pseudocyst treated by endoscopic ultrasonography (EUS)-guided drainage

Wegener's granulomatosis is a systemic vasculitis with prominent involvement of the respiratory tract and kidney. There are 10 patients with Wegener's granulomatosis in the literature who were documented as acute pancreatitis. We present two cases with Wegener's granulomatosis presenting with acute pancreatitis and pancreatic pseudocyst. Endosonography-guided drainage of the pancreatic pseudocyst led to rapid clinical improvement. Pancreatic pseudocyst in Wegener's granulomatosis is not reported in the literature, and these are the first cases of Wegener's granulomatosis to be managed by endosonography-guided cyst drainage. The safety of endosonography-guided pancreatic pseudocyst drainage and the clinical features of the previous Wegener's granulomatosis cases with acute pancreatitis are discussed.     

The clinical spectrum of primary renal vasculitis

BACKGROUND AND OBJECTIVES: The vasculitides are potentially severe and often difficult to diagnose syndromes. Many forms of vasculitis may involve the kidneys. This review will focus on the clinical and histopathological aspects of renal involvement in the systemic vasculitides. METHODS: We searched the MEDLINE database using as key terms the MeSH terms and textwords for different forms of vasculitis and for renal involvement, creating a database of more than 2200 relevant references. RESULTS: The frequency of renal involvement in vasculitis varies among different syndromes. It is more frequent in Wegener's granulomatosis and microscopic polyarteritis, while it is uncommon to rare in other forms of vasculitis such as Beh?et's disease and relapsing polychondritis. The vessels affected include the renal artery in Takayasu arteritis, medium-size renal parenchymal artery in classic polyarteritis nodosa, and glomerular involvement in Wegener's granulomatosis and microscopic polyarteritis. The clinical expression of renal vasculitis depends on the size of the affected vessels and includes renovascular hypertension, isolated nonnephrotic proteinuria, interstitial nephritis, and glomerulonephritis, which can be rapidly progressive. Diagnosis is established by a combination of history, clinical manifestations, laboratory findings (eg, urine sediment, urine protein, antineutrophil cytoplasmic antibodies), imaging techniques (renal angiography, especially when there is a suspicion of medium-to-large vessel disease, and chest radiograph), and finally, renal biopsy. Prognosis varies from unfavorable in the rapidly progressive glomerulonephritis of microscopic polyarteritis, which can lead to renal failure, chronic dialysis, and renal transplantation, to benign, as in the case of Henoch Schonlein purpura, in which the majority of patients recover. CONCLUSIONS: The manifestations and prognosis of renal vasculitis range widely. Renal involvement greatly influences prognosis and dictates the need for early and prompt immunosuppressive therapy. Thus, the clinician should be alert for the timely diagnosis and treatment of renal vasculitis.     

Wegener’s granulomatosis presenting as multiple bilateral renal masses: case report and literature review

Wegener's granulomatosis (WG) is a disease of unknown etiology characterized by necrotizing granulomatous vascularitis. The upper and lower respiratory tract and kidney involvements are very common; however, its presentation as bilateral renal masses is unusual. We report a case of a 59-year-old female patient who presented with multiple bilateral renal masses. The patient presented with sinusal and ocular symptoms suggestive of WG, and positive antineutrophil cytoplasmic antibodies (c-ANCA) with an anti-PR3 pattern. Histopathologic examination of the renal biopsy specimen revealed granulomatous inflammation with vasculitis and fibrinoid necrosis. The patient management, including prednisone and cyclophosphamid, induced a marked improvement of the renal masses. This case illustrates that WG should be considered in the differential diagnosis of renal masses.     

Ocular involvement in patient with Wegener's granulomatosis--a case report

Wegener's granulomatosis is a systemic vasculitis. Most often granulomatous inflammation is situated in upper and lower respiratory tracts as well as kidneys. Ocular involvement affects 50-60% of patients with Wegener's granulomatosis. Herein is reported the case of a man with Wegener's granulomatosis with ocular involvement which was a symptom of high disease activity. Intensive immunosuppressive treatment and local treatment were ineffective in eye involvement inhibition.     

Alveolar hemorrhage in Wegener's granulomatosis

The authors describe massive, fatal alveolar hemorrhage in an 82-year-old woman in whom a diagnosis of Wegener's granulomatosis was made at postmortem examination. A literature review revealed a total of 18 case reports of this entity that were described in sufficient detail to permit abstraction of clinical characteristics. When compared with a large series of patients with more "typical" Wegener's granulomatosis, patients with alveolar hemorrhage had more evidence of systemic vasculitis and glomerulitis and less evidence of upper airway disease. Alveolar hemorrhage was associated with greater than 50% mortality, reflecting in part, the fulminant nature of this complication of Wegener's granulomatosis. All survivors had been treated with cytotoxic agents; those who died had either no specific therapy or were treated either with corticosteroids alone or died before cytotoxic therapy could be expected to be effective. Wegener's granulomatosis should be in the differential diagnosis for patients who present with alveolar hemorrhage with or without renal failure. An aggressive diagnostic approach and the earliest possible administration of cytotoxic drugs in combination with corticosteroids offer the best chance of survival in this fulminant condition.     

The ocular manifestations of Wegener's granulomatosis. Fifteen years experience and review of the literature.

Ocular manifestations of Wegener's granulomatosis may occur secondary to contiguous granulomatous sinusitis or as a result of focal vasculitis. Contiguous granulomatous sinus disease causes nasolacrimal duct obstruction, proptosis and ocular muscle or optic nerve involvement. Focal vasculitis unrelated to contiguous upper respiratory tract disease is manifested by conjunctivitis, episcleritis, scleritis, corneoscleral ulceration, uveitis, and granulomatous vasculitis of the retina and optic nerve. A review of 29 cases of Wegener's granulomatosis and three cases of lymphomatoid granulomatosis studied over the past 15 years at the National Institute of Allergy and Infectious Diseases (NIAID) disclosed single or multiple ocular manifestations of disease in 15 patients (47 per cent). The pattern of ocular disease, its relationship to systemic involvement, diagnostic methods and the response to therapy are discussed.     

Wegener’s Granulomatosis

Wegener's granulomatosis is an organ- and/or life-threatening autoimmune disease of as yet unknown etiology. The classic clinical triad consists of necrotizing granulomatous inflammation of the upper and/or lower respiratory tract, necrotizing glomerulonephritis, and an autoimmune necrotizing systemic vasculitis affecting predominantly small vessels. The detection of antineutrophil cytoplasmic antibodies directed against proteinase 3 (PR3-ANCA) is highly specific for Wegener's granulomatosis. ANCA positivity is found only in about 50% of the patients with localized Wegener's granulomatosis (which is restricted to the respiratory tract and affects < or = 5% of the patients), whereas PR3-ANCA positivity is seen in 95% of the patients with generalized Wegener's granulomatosis. Studies showing an expansion of circulating tumor necrosis factor-(TNF-)alpha-producing Th1-type CD4(+)CD28(-) T-cell effector memory T-cells and their presence as Th1-type cytokine profile- driving cell population within granulomatous lesions provide the rationale for using TNF-alpha-blocking agents in Wegener's granulomatosis refractory to standard induction therapy with cyclophosphamide and corticosteroids ("Fauci's scheme"). Vasculitis is an independent risk factor for diffuse endothelial dysfunction and may be a consequence of TNF-alpha action on endothelial cells. Recently, another study has shown intima-media thickening of the wall of the common carotid artery and bulb, as well as a significantly increased incidence of stroke, myocardial infarction and occlusive artery disease in Wegener's granulomatosis. This study suggests that systemic inflammation and vasculitis contribute to accelerated arteriosclerosis in Wegener's granulomatosis.