Wegener’s Granulomatosis Presenting as Mediastinal Soft Tissue Mass Invading the Tracheal Wall

Wegener’s granulomatosis (WG) is a clinicopathologic entity of unknown cause characterised by a necrotising granulomatous vasculitis affecting multiple organs, especially the upper and lower respiratory tracts, lung and kidney. The lung is the most frequently, and sometimes the only involved organ. Single or multiple nodules, with or without cavitation, are the most common pulmonary manifestations in WG, but mediastinal involvement is atypical. The sole tracheal involvement is rare and hilar and mediastinal involvement has been thought not to be part of the clinical feature. We experienced a rare case of WG presenting as paratracheal mediastinal lesions with tracheal wall invasion, which responded dramatically to corticosteroid treatment. We present this case with a review of the literature. Received: 22 October 1999 / Accepted: 20 April 2000     

Panhypopituitarism due to Wegener's granulomatosis

Wegener's granulomatosis (WG) is a multi-system necrotizing granulomatous vasculitis which classically affects the upper respiratory tract, lungs and kidneys. Pituitary participation has been described in 24 patients in the literature to date. The aim of this article is to report a case of pituitary involvement in WG, and to present a literature review on this association. We present a female patient with WG who evolved with central diabetes insipidus (CDI), panhypopituitarism, and mild hyperprolactinemia. MRI showed an infiltrative pattern. Pituitary involvement has been reported in around 1% of patients with WG, mostly in women. It is represented by CDI and hypopituitarism. MRI generally shows pituitary enlargement, stalk thickening and loss of hyperintensity of the neurohypophysis. Permanent endocrine therapy is generally needed. WG should be considered in cases of CDI and hypopituitarism, essentially if a vasculitis is suspected and more common sellar disorders have been ruled out.     

Wegener's granulomatosis of the prostate gland

Wegener's granulomatosis (WG) is a systemic granulomatous vasculitis affecting medium and small arteries, venules, and arterioles. The upper and lower respiratory tract and kidney are primarily involved. Patients with classic WG essentially present with upper airway and pulmonary involvement. Renal disease is common. Involvement of other organ systems is also relatively frequent, most often heart, joints, muscles, eyes, skin, and central and/or peripheral nervous system. We present a patient in whom WG was diagnosed primarily because of prostate involvement. This seems to be a rare manifestation.     

Sudden death and Wegener's granulomatosis of the pituitary

Involvement of brain parenchyma or meninges in ANCA-associated small-vessel vasculitis such as Wegener's granulomatosis (WG) is not uncommon. In contrast, involvement of the pituitary is exceedingly rare with only a few cases reported so far. The diagnosis is usually made on the basis of imaging techniques and abnormal pituitary function tests in the setting of active systemic vasculitis. However, histology-proven involvement of the pituitary by WG has not been reported so far. We report a case of WG with histology-proven granulomatous necrotizing inflammation of the pituitary and hypothalamo-pituitary stalk, disclosed at autopsy after the patient had died suddenly and unexpectedly in his sleep. In a setting of histology-proven WG, these findings were regarded as a pituitary manifestation of the disorder. A distinct cause of death could not be found, hence we speculate that hypothalamo-pituitary inflammation due to WG may have caused the sudden death in this patient.     

A case of Wegener’s granulomatosis with intracranial involvement

We report a case of Wegener’s granulomatosis (WG) associated with intracranial involvement and brain magnetic resonance imaging (MRI) findings. A 44-year-old woman was diagnosed as having WG with involvement of an ear, lungs and kidneys. A brain computed tomography scan showed a space-occupying lesion with a ring-like enhancement in the left temporal lobe. Findings on a brain MRI, different from those characteristic of a brain abscess, supported an intracerebral granulomatous inflammation due to WG. Immunosuppression, which included corticosteroids, was an effective treatment.     

Pituitary involvement in Wegener’s granulomatosis

Wegener’s granulomatosis (WG) is an anti-neutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitis of small and medium-sized vessels. Pituitary involvement in WG is rare with only 22 previous case reports in the English medical literature between 1966 and 2006. Herein we report another patient with WG-related diabetes insipidus (DI) and partial disruption of the anterior pituitary axes. We also review the clinical features, imaging findings, treatment and outcome of WG-related pituitary involvement. Isolated pituitary involvement in the absence of lung or renal complications in WG is rare and described in only one previous patient. Pituitary involvement in WG is usually associated with other organ involvement (96% of cases)—commonly upper respiratory tract (93%), lungs (73%) and kidneys (67%). Abnormalities are often seen in the hypothalamo-pituitary region on magnetic resonance imaging (MRI) or computed tomography (CT) of the head (90% of cases). In 65% of reported cases, cyclophosphamide-based induction therapy was used with a subsequent relapse rate of 27%, occurring at a median of 10.5 months (range: 7–36 months) after initiation of treatment. In comparison, induction treatment without cyclophosphamide was associated with relapse in 50% at a median of 4.5 months (range: 4–18 months after starting treatment) suggesting more frequent and earlier relapse. Therefore, we recommend treatment with cyclophosphamide-based regimen. Despite treatment of WG, only 17% (4 patients) had full recovery in their pituitary function. The long-term prognosis of patients with WG and pituitary involvement is not known.     

Esophageal involvement in wegener's granulomatosis

Wegener's granulomatosis (WG) is characterized by granulomatous vasculitis, renal disease, and upper and lower respiratory tract disease. Although most organ systems can be involved, gastrointestinal (GI) manifestations are notably uncommon. We describe a patient with WG whose presentation was unique for the prominence of odynophagia. Esophagoscopy revealed erosive esophagitis, which on biopsy was shown to be due to direct involvement by the underlying vasculitis. This is first antemortem documentation of esophageal disease secondary to WG. The GI manifestations of WG are reviewed.     

Wegener's granulomatosis presenting as diabetes insipidus.

Pituitary gland involvement in Wegener's granulomatosis (WG) occurs most commonly in the form of central diabetes insipidus (CDI). However, CDI as a presenting manifestation of WG is very rare. We report two such cases; one of them had multi-organ involvement at presentation, while other developed it during follow-up. CDI was reversible following cytotoxic drug therapy in one of them.     

A case of Wegener’s granulomatosis with intracranial involvement

Abstract

We report a case of Wegener’s granulomatosis (WG) associated with intracranial involvement and brain magnetic resonance imaging (MRI) findings. A 44-year-old woman was diagnosed as having WG with involvement of an ear, lungs and kidneys. A brain computed tomography scan showed a space-occupying lesion with a ring-like enhancement in the left temporal lobe. Findings on a brain MRI, different from those characteristic of a brain abscess, supported an intracerebral granulomatous inflammation due to WG. Immunosuppression, which included corticosteroids, was an effective treatment.     

Limited Wegener’s granulomatosis—is it limited?

Complete heart block associated with Wegener’s granulomatosis (WG) is rare especially in the limited form of the disease. We describe a case of a 43-year-old woman with a limited form of WG who developed a complete heart block. Prompt treatment with steroids and cyclophosphamide led to temporary regression of complete heart block. Further involvement of lung was treated successfully by tumor necrosis factor-alpha inhibitor infliximab. Cardiac rhythm abnormalities should always be kept in mind both in diagnosis and follow-up of WG.     

Meningeal involvement in Wegener's granulomatosis

We describe a patient with Wegener's granulomatosis (WG) who developed neurological symptoms attributed to meningeal involvement. The diagnosis of WG was complicated by persistently negative antineutrophil cytoplasmic antibodies (ANCA) and lack of specificity in the histopathological findings from multiple anatomical sites. This rare neurological manifestation of WG was treated successfully with oral cyclophosphamide and the patient has continued remission for 3 years taking oral methotrexate.     

A case report of the limited form Wegener's granulomatosis]

The classical from of Wegener's granulomatosis (WG) is a necrotizing granulomatous angiitis that involves the upper and lower airways, and kidneys. A limited form of WG is characterized by pulmonary lesions identical to those of classical form WG without renal involvement. The authors report a case of limited form WG. A 58-year-old Japanese woman was admitted because of an abnormal pulmonary shadow. Pathological examination revealed granulomatous angiitis consistent with WG. No other organ involvement was found. The pulmonary shadow improved with cyclophosphamide therapy. The patient is now well and without evidence of exacerbation of the disease 18 month after the discharge.     

Central nervous system involvement in Wegener granulomatosis

Wegener granulomatosis (WG) is an antineutrophil cytoplasmic antibody (ANCA)-associated granulomatous vasculitis of small and medium-sized vessels. This vasculitis involves mainly the upper and lower respiratory tracts and kidneys, although WG may affect any organ. Central nervous system (CNS) involvement is an uncommon manifestation of WG, reported in 7%-11% of patients. Three major mechanisms have been incriminated as causing CNS disease in WG: contiguous invasion of granuloma from extracranial sites, remote intracranial granuloma, and CNS vasculitis. Herein we describe 6 patients with WG-related CNS involvement, 2 of whom had chronic hypertrophic pachymeningitis, 3 with pituitary involvement, and 1 with cerebral vasculitis. CNS involvement was present at disease onset in 2 patients and occurred 5-18 years after WG diagnosis in the remaining 4. Based on these observations and a review of the literature, we discuss the pathogenic mechanisms, clinical features, imaging findings, treatment, and outcome of meningeal, pituitary, and vascular involvement, with an emphasis on differential diagnoses, prognosis, and therapeutic management of WG-related CNS involvement.     

A relapsing case of Wegener's granulomatosis presenting as an endobronchial mass

Wegener's granulomatosis (WG) relapse is frequent. Although lung involvement occurs in 85% of patients, endobronchial presentation of the disease is uncommon. We reported a relapsing case of WG presenting as an endobronchial mass. A 56- year-old man present ed with recurrence of WG following 14 months of cyclophosphamide and prednisolone therapy and 36 months of complete remission. At his first presentation, he was diagnosed as having WG with involvement of kidney, lung, upper airways, skin, joints and eyes. His chest X-ray showed bilateral patchy consolidation. Cytoplasmic-anti-neutrophil cytoplasmic antibodies (c-ANCA) was also present in high titres. c-ANCA was negative after therapy. At the time of relapse, he presented with nasal symptoms and hemoptysis. His chest X-ray showed right paracardiac opacity. Fiberoptic bronchoscopy revealed a mass lesion subtotally obstructing the proximity of right lower lobe. He has been given prednisolone in tapering doses and cyclophosphamide for 10 months.     

Wegener’s granulomatosis presenting with life-threatening lung hemorrhage in a 7-year-old child

Wegener’s granulomatosis (WG) is an idiopathic systemic disease that usually onsets in adolescence and is rare in young children. Its diagnosis is usually based on the presence of fever with arthralgia and weight loss, associated with symptoms of upper and/or lower respiratory tract involvement and renal disorders. We describe the appearance of a life-threatening lung hemorrhage in the absence of hemoptysis in a 7-year-old girl with a completely negative previous clinical history, who was subsequently diagnosed as having WG. The teaching message is that immediate bronchoscopy with bronchoalveolar lavage seems to be advisable in the presence of severe respiratory distress and bilateral lung as well as renal involvement. When a diffuse alveolar hemorrhage syndrome is demonstrated, WG should be considered among the main etiologies even in a relatively young child without a clinically suggestive history.     

Can sulfasalazine therapy induce or exacerbate Wegener's granulomatosis?

Sulfasalazine (SSZ) can induce serological and clinical autoimmune reactions but the occurrence of SSZ-related Wegener's granulomatosis (WG) has not been reported before. We describe two patients with rheumatoid factor (RF)-positive rheumatoid arthritis (RA) who developed biopsy-proven WG with serious organ involvement during SSZ therapy. The pathogenetic mechanism that explains the relationship between SSZ and the occurrence of a de novo anti-neutrophil cytoplasmic antibody (ANCA)-related vasculitis or a flare is discussed. We propose that WG can be a rare complication of SSZ therapy and that this, like other autoimmune adverse events of this drug, is mediated by SSZ-induced apoptosis.     

Cardiac involvement in patients with Wegener's granulomatosis

Wegener's granulomatosis (WG) is a systemic necrotizing vasculitis, which could potentially affect any organ system. However, there have only been a few reports on cardiac involvement. We described the echocardiographic findings in nine patients affected by WG. A complete M-mode, two-dimensional, Doppler and color-Doppler transthoracic echocardiogram was performed in nine patients (seven females and two males) affected by WG. In each patient, cardiac abnormality, for example, valvular damage, left ventricular global systolic dysfunction, or pericardial effusion, was detected. In particular, heart valve disease was found in eight patients, and in three cases, aortic valve insufficiency, which was severe enough to require surgical valve replacement, was observed. Cardiac involvement in patients with WG is common. In particular, there is a high frequency of aortic valve abnormalities. Thus, an echocardiographic study should be routinely performed. Received: 21 October 1999 / Accepted: 28 April 2000     

Urogenital manifestations in Wegener granulomatosis: a study of 11 cases and review of the literature

We describe the main characteristics and treatment of urogenital manifestations in patients with Wegener granulomatosis (WG). We conducted a retrospective review of the charts of 11 patients with WG. All patients were men, and their median age at WG diagnosis was 53 years (range, 21-70 yr). Urogenital involvement was present at onset of WG in 9 cases (81%), it was the first clinical evidence of WG in 2 cases (18%), and was a symptom of WG relapse in 6 cases (54%). Symptomatic urogenital involvement included prostatitis (n = 4) (with suspicion of an abscess in 1 case), orchitis (n = 4), epididymitis (n = 1), a renal pseudotumor (n = 2), ureteral stenosis (n = 1), and penile ulceration (n = 1). Urogenital symptoms rapidly resolved after therapy with glucocorticoids and immunosuppressive agents. Several patients underwent a surgical procedure, either at the time of diagnosis (n = 3) (consisting of an open nephrectomy and radical prostatectomy for suspicion of carcinoma, suprapubic cystostomy for acute urinary retention), or during follow-up (n = 3) (consisting of ureteral double J stents for ureteral stenosis, and prostate transurethral resection because of dysuria). After a mean follow-up of 56 months, urogenital relapse occurred in 4 patients (36%). Urogenital involvement can be the first clinical evidence of WG. Some presentations, such as a renal or prostate mass that mimics cancer or an abscess, should be assessed to avoid unnecessary radical surgery. Urogenital symptoms can be promptly resolved with glucocorticoids and immunosuppressive agents. However, surgical procedures, such as prostatic transurethral resection, may be mandatory in patients with persistent symptoms.     

A rare case of bronchial stenosis in Wegener's granulomatosis. Dramatic response to intravenous cyclophosphamide and oral cotrimoxazole.

We describe a patient with Wegener's granulomatosis (WG) who developed as the only pulmonary manifestation a severe proximal bronchial stenosis despite conventional treatment with steroids and oral cyclophosphamide. The patient subsequently responded to a combined therapy with intravenous (IV) cyclophosphamide and oral cotrimoxazole. We stress the rarity of bronchial involvement in WG and discuss the role of IV cyclophosphamide and cotrimoxazole in the management of this disease.     

Unilateral phrenic nerve paralysis in a patient with Wegener's granulomatosis

A 35-year-old male diagnosed with Wegener's granulomatosis (WG) in 1989 recently came to our hospital with the complaint of left lumbar pain. He was found to have left nephrolithiasis and left diaphragmatic elevation. Fluoroscopic study and electromyographic examination revealed findings compatible with unilateral phrenic paralysis. We could not detect any cause other than WG which could be responsible for the unilateral diaphragmatic paralysis. Although different types of lung involvement have been reported in WG, to our knowledge this is the first in which unilateral phrenic nerve paralysis and diaphragmatic elevation associated with WG have been diagnosed.