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.     

Ophthalmologic manifestations of systemic vasculitis: report of six cases and review of the literature

PURPOSE: Study of characteristics of ocular involvement in systemic vasculitis. METHODS: We describe six cases of systemic vasculitis with ocular involvement observed between 1992 and 2000. These cases are compared with those reported in the literature. RESULTS: Our patients suffered from Wegener's granulomatosis (four cases), periarteritis nodosa and Churg-Strauss syndrome. Ocular manifestations were conjunctivitis, scleritis, orbital pseudotumor, optic neuritis and extraocular muscle palsy. These manifestations are similar to those reported in the literature. Their treatment requires steroids and immunosuppressive drugs. In one of our cases, intravenous immunoglobulins were effective in controlling an optic neuritis. CONCLUSION: Ocular involvement in systemic vasculitis may concern any orbital structure. It usually occurs during the course of vasculitis but may be one of its first manifestations. It requires an appropriate treatment to prevent ophthalmic complications and especially blindness.     

Airway involvement in Wegener's granulomatosis

Wegener's granulomatosis is characterized by necrotizing granulomatous inflammation and necrotizing vasculitis affecting predominantly small arteries, arterioles, capillaries, and venules. In contrast to the well-described pulmonary parenchymal involvement of Wegener's granulomatosis, the lower airway (tracheobronchial) disease manifestations are less well recognized by clinicians. Consequently, mild disease of the airways is easily missed. There is a relative paucity of published information on various tracheobronchial manifestations of Wegener's granulomatosis. This article provides a comprehensive review of the diagnosis and management of the infraglottic tracheobronchial disease manifestations.     

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.     

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.     

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.     

Wegener's granulomatosis: ophthalmic manifestations and management

OBJECTIVES: To provide an up-to-date and comprehensive review of Wegener's granulomatosis (WG) as a disease entity, focusing on the ophthalmic manifestations and management options. METHODS: A search of Medline was undertaken between 1966 and 2005 regarding WG, systemic vasculitis, and the ocular manifestations of WG. Major ophthalmic and medical textbooks also were reviewed for content, as well as original references. RESULTS: Involvement of ocular and orbital structures in patients with WG is common and may be a presenting feature. The ocular manifestations range from mild conjunctivitis and episcleritis to more severe inflammation with keratitis, scleritis, uveitis, and retinal vasculitis. Involvement of the nasolacrimal system and orbital tissues also can occur. Except for some cases of anterior segment inflammation, the ocular involvement will not respond to topical agents, but rather to systemic antiinflammatory and immunosuppressive regimens. Surgical intervention may be of value for obtaining tissue diagnosis, in achieving orbital decompression in cases of significant orbital disease with optic nerve compromise, or in cases of nasolacrimal duct obstruction. CONCLUSION: WG is an important clinical entity that needs to be recognized early and treated appropriately. Ophthalmic manifestations are frequently encountered and can result in significant morbidity and even blindness. The management is challenging and often requires a multidisciplinary approach.     

Small intestinal perforation in Wegener's granulomatosis

Wegener's granulomatosis is a disease characterized by necrotizing vasculitis and granulomatous inflammation. Gastrointestinal (GI) involvement is uncommon in this disorder. Only a few cases of perforation of GI tract have been reported, but vasculitis has not been demonstrated to be a cause of perforation in these cases. We report a case of Wegener's granulomatosis in which a single perforation in the terminal ileum was disclosed on laparotomy and active necrotizing vasculitis was found in the submucosal layer of the resected specimen.     

Thyroid involvement in Wegener's granulomatosis: a case report

Wegener's granulomatosis is a systemic necrotizing vasculitis affecting small to medium-sized vessels, characterized by involvement of the upper airways, lungs and kidneys. Endocrine involvement, particularly the thyroid gland is extremely rare. We report a 56-year-old woman presenting a limited form of Wegener's granulomatosis with the following clinical manifestations: sinusitis, pulmonary nodules, diabetes insipidus and a cold thyroid nodule. A right hemithyroidectomy was performed and the histopathological examination revealed a necrotizing vasculitis surrounded by granulomatous lesions. She was treated by corticosteroids without immunosuppressive therapy with a favourable outcome.     

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.     

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.     

Less common manifestations and presentations of Wegener's granulomatosis.

Wegener's granulomatosis is a clinicopathologic syndrome of unknown etiology characterized by granulomatous vasculitis of the upper and lower respiratory tracts and by glomerulonephritis. Virtually any organ system can be affected, and many patients present with unusual features of disease. During the period covered by this review, several articles reported atypical manifestations of Wegener's granulomatosis, including diffuse pulmonary infiltrates, lymphadenopathy, diffuse pulmonary hemorrhage, and overlap with giant cell arteritis. Unusual features of upper airway, eye, gastrointestinal, nervous system, and genitourinary tract disease were also described, and less common histopathologic features of pulmonary and nasal disease were characterized.     

Incidence and presentation of the central neurological manifestations of Wegener's granulomatosis: a monocentric study of 14 cases

PURPOSE: Central nervous system manifestations are rare clinical features of Wegener's granulomatosis, and occur in 4-8% of the patients, but few studies were dedicated to them. METHODS: This retrospective study (1988-2001) include 14 consecutive patients suffering from Wegener's granulomatosis. Involvement of central nervous system was defined as follows: suggestive neurological signs, compatible cerebral imaging, efficacy of the specific treatment of the granulomatosis. RESULTS: Four patients had a central nervous system manifestation (29%), including three women (average age 51 years). The signs were inaugural in a case. The manifestations were the following: sensibility disturbance (three cases), motor weakness (two cases), aphasia (one case), and mood disorders (two cases). Cranial nerves were constantly involved. Cerebral magnetic resonance imaging findings were: pachymeningitis and venous thrombosis (one case), vasculitis (two cases). Under steroid therapy associated with cyclophosphamide, in spite of a recurrence at 27 months in one patient, all patients had a complete remission. There was no death, with a median follow-up of 66 months. Except the ocular signs, that were more common (three cases), these patients had the usual characteristics of the Wegener's granulomatosis: rhinosinusitis (four cases), pulmonary (three cases), renal (three cases), and peripheral nervous system involvement (three cases). CONCLUSIONS: Our study, based on precise criteria, indicates that the frequency of the central neurologic manifestations of Wegener's granulomatosis is probably under estimated. Cranial nerves involvement is highly evocative. The long-term prognosis seems good, in spite of the associated multivisceral disorder.     

Rheumatic manifestations of Wegener's granulomatosis

Wegener's granulomatosis is a necrotizing granulomatous vasculitis with multisystem involvement. We reviewed the rheumatic manifestations among 50 patients with Wegener's granulomatosis. Arthralgia, myalgia and/or arthritis occurred in 2/3 of patients and 28% had nonerosive and nondeforming polyarthritis. Noninflammatory synovial fluid was found upon arthrocentesis of one patient with a knee effusion. Unexpected findings included soft tissue calcifications in 9 patients, sacroiliitis in 3, and relapsing polychondritis in 2. Rheumatoid factor was present in 1/2 of the patients tested. Additionally, both antinuclear antibodies and anti-DNA antibodies were detected in 14 and 38%, respectively, of patients tested.     

Ophthalmological diagnosis in systemic vasculitis

Ocular manifestations of systemic vasculitides are extraordinarily pleomorph and have been described for many decades. In the introduction the special interaction of the immune system and the eye is outlined. Thereafter diagnostic procedures of patients suffering from systemic vasculitides are shown from an ophthalmological view point. Many examples, which show multiple possibilities of ocular manifestations on systemic vasculitides, are given. With regard to the anatomy of the eye, a review is given on the involvement of the 1) outer eye and a tear film, 2) lacrimal duct diseases, 3) diseases of the lid, 4) diseases of conjunctiva, 5) diseases of sclera, 6) diseases of the cornea, 7) diseases of the lens, 8) intraocular inflammation, 9) retinal affections, 10) optic nerve inflammation, and 11) orbiota involvement. Frequencies of these structures involved in the various systemic vasculitides are outlined. Principally every systemic vasculitis can induce every eye manifestation; however, larger patient groups show characteristic features of eye manifestations in systemic vasculitides. Special reference is given to Wegener's granulomatosis, giant-cell arteritis and Behcet vasculitis.     

WEGENER'S GRANULOMATOSIS: CLINICAL FEATURES AND OUTCOME IN SEVENTEEN PATIENTS

Seventeen patients with Wegener's granulomatosis are reviewed. Eleven males and six females, with a mean age of 46.9± 4.5 years, were followed for 35.7 ± 9.0 months. Mean duration from time of onset of symptoms to diagnosis was 8.5 ±3.1 months. Constitutional symptoms (100%), lower respiratory tract involvement (93%), renal involvement (87%), and upper respiratory tract involvement (80%) were the most frequent clinical manifestations. Arthritis (60%), dermal vasculitis (60%), and inflammatory ocular disease (40%) were also common. Elevated ESR (94%), anemia (70%), and lymphopenia (77%) were frequent laboratory findings prior to treatment. Five patients had renal failure at presentation and two patients progressed from no renal involvement at presentation to renal failure at diagnosis, while five patients progressed from renal involvement without impairment at diagnosis to end-stage renal failure. Seven patients died; six of these deaths were related to active Wegener's granulomatosis. The patients with a severe systemic vasculitis, and renal involvement had a poor outcome while predominant respiratory disease had a good prognosis.     

Extensive cerebral infarction due to involvement of both anterior cerebral arteries by Wegener''s granulomatosis.

The central nervous system is often affected in Wegener's granulomatosis (WG), but massive cerebral infarction due to occlusion of branches of the anterior cerebral arteries (ACA) by granulomatous lesions or thrombosis, or both, has seldom been reported. A case is reported here of a 67 year old man with WG complicated by generalised necrotising vasculitis in the lung, kidney, and gastrointestinal tract, and cerebral infarction in the territory of both anterior cerebral arteries, probably caused by thrombosis and a contiguous invasion of granulomatous lesion from the nasal cavity.     

Granulomatous gastritis in Wegener's disease: differentiation from Crohn's disease supported by a positive test for antineutrophil antibodies.

BACKGROUND: This report concerns the gastric manifestation of Wegener's granulomatosis in a 44 year old white female patient who initially presented with abdominal pain, vomiting, and iridocyclitis. FINDINGS: The clinical findings and the histopathological proof of granulomatous gastritis in the absence of necrotising vasculitis were initially considered to be indicative of a diagnosis of Crohn's disease showing isolated gastric involvement. A five month course of steroids resulted in temporary relief; thereafter the patient developed severe rhinitis with mucosal ulcerations. At this point biopsy of nasal mucosa disclosed the classic histopathological signs of Wegener's granulomatosis. A positive test for antineutrophil cytoplasmic antibodies (ANCAs) with a cytoplasmic pattern (c-ANCA) and antigenic specificity for proteinase 3 (PR-3) were found. The patient is in complete remission one year after diagnosis and treatment with steroids and cyclophosphamide. CONCLUSIONS: Wegener's granulomatosis can also involve the gastrointestinal tract. Granulomatous inflammation of the stomach, although a rare finding and non-specific, should include Wegener's disease in the differential diagnosis. The histological proof of necrotising vasculitis is dependent on the depth of the biopsy and therefore can be easily missed. Differential diagnosis can be clarified by ANCA testing.     

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.