Common and uncommon manifestations of Wegener granulomatosis at chest CT: radiologic-pathologic correlation

Wegener granulomatosis is an uncommon necrotizing vasculitis that classically manifests as a clinical triad consisting of upper and lower airway involvement and glomerulonephritis. Other less frequently involved organ systems include the central and peripheral nervous system and large joints. The diagnosis is based on a combination of clinical and laboratory findings. Because thoracic involvement often predominates, chest radiographic findings are often the first to suggest the diagnosis. However, chest computed tomography (CT) has superior sensitivity and specificity for evaluation of the airways, lung parenchyma, and mediastinum, particularly with the use of multiplanar reformatted and three-dimensional images. Common pulmonary radiologic findings include waxing and waning nodules, masses, ground-glass opacities, and consolidation. Airway involvement is usually characterized by circumferential tracheobronchial thickening, which can be smooth or nodular. Pleural effusions are the most common manifestation of pleural disease and can result from primary involvement or be secondary to renal failure. Mediastinal lymphadenopathy is a nonspecific finding and is usually reactive. Uncommon thoracic radiologic manifestations include involvement of the heart and great vessels. CT is the imaging modality of choice for diagnosis, surveillance, and follow-up in patients with Wegener granulomatosis.     

Chest radiograph in lymphomatoid granulomatosis: comparison with Wegener granulomatosis

A form of angiitis and granulomatosis principally involving the lung was identified as a variant form of Wegener granulomatosis only 15 years ago. Recent experience indicates that the most common form of pulmonary angiitis and granulomatosis is lymphomatoid granulomatosis. Chest radiographs of 16 patients with this condition were reviewed and the findings compared with those of Wegener granulomatosis. The frequency and distribution of nodular masses, cavitation, and migratory lesions were similar in the two disorders, but reticulonodular infiltrates occurred only in lymphomatoid granulomatosis. The study suggests that lymphomatoid granulomatosis and Wegener granulomatosis occasionally can be distinguished by radiographic criteria. Although radiologic patterns will suggest the diagnosis of pulmonary angiitis and granulomatosis, the specific diagnosis of lymphomatoid granulomatosis must rely on clinical, immunologic, and pathologic evidence. Accurate differentiation is essential since treatment of Wegener granulomatosis with cyclophosphamide is highly effective while treatment of lymphomatoid granulomatosis is infrequently successful.     

Spontaneous pneumothorax: an uncommon clinical first sign of the bronchial carcinoma (author's transl)

Seldom the bronchial carcinoma begins with spontaneous pneumothorax. About 20 cases are reported in the literature. A personal observation is described concerning a 63 year old patient with clinical symptoms of spontaneous pneumothorax. The histologic diagnosis was bronchial spinocellular carcinoma.     

Wegener granulomatosis of the breast

Two cases of Wegener granulomatosis of the breast are reported. In both cases, this rare disease was detected on mammographic examination and was diagnosed for the first time by a fine-needle aspiration biopsy of the breast. The mammographic findings can lead to misinterpretations and can be confused with advanced mammary carcinoma or the alterations caused by lymphoblastoma, leukemia, or Hodgkin disease. The total regression of the tumorlike lesions in the breast, evidence of involvement of the lung with only slight clinical symptoms, and a very protracted course of the disease were remarkable in both cases.     

Wegener granulomatosis: CT and MR findings.

PURPOSETo demonstrate the spectrum of CT and MR imaging findings in patients with Wegener granulomatosis and to determine how often these findings could be attributed to either direct extension from paranasal or orbital disease sites, remote granulomas, or central nervous system (CNS) vasculitis.METHODSWe retrospectively reviewed the CT or MR studies of 15 patients with Wegener granulomatosis.RESULTSAbnormal findings were seen in 7 patients (5 examined with MR imaging, 2 with CT). Findings included dural thickening and contrast enhancement (3 patients), infarcts (2 patients), regions of hyperintense signal on T2-weighted MR images (2 patients), and abnormal MR signal in the brain stem (2 patients). Three patients with imaging findings of dural enhancement and thickening were thought to have remote granulomatous lesions involving the dura. No patients had extension from sites external to the CNS or clinical findings suggestive of CNS vasculitis.CONCLUSIONThe spectrum of CT and MR findings in Wegener granulomatosis includes dural thickening and enhancements cerebral infarction, and MR signal abnormalities in the brain stem and white matter. Presumed remote granulomatous lesions were the most common causes of CNS findings in this study. Complications related to non-CNS disease (eg, hypertension, endocarditis) also appear to have played a role in some patients.     

Wegener granulomatosis involving the pterygopalatine fossa: an unusual case of trigeminal neuropathy

We report on a case of Wegener granulomatosis in the pterygopalatine fossa that was associated with trigeminal neuropathy. MR and CT examinations were useful in depicting the extent of the lesion and suggesting a perineural spread. Diagnosis was confirmed with positive serum assay findings for the presence of cytoplasmic antineutrophil cytoplasm antibody.     

Wegener granulomatosis: CT features of parenchymal lung disease

Chest CT from eight patients with pulmonary Wegener granulomatosis were reviewed. The CT features of parenchymal lung involvement included multiple nodules or masses (seven of eight; 88%), ranging in size from 0.3 to 5.0 cm. Lung nodules demonstrated distinct feeding vessels in seven of eight patients (88%); and lesions similar to pulmonary infarcts, i.e., peripheral wedge-shaped lesions abutting the pleura, were identified in seven cases (88%). Scarring, spiculation, and pleural tags emanating from pulmonary nodules were prominent features (seven of eight; 88%). Other findings included cavitation (four of eight; 50%), air bronchograms through nodules (two of eight; 25%), and pleural effusions (two of eight; 25%). One patient on immunosuppressant therapy also demonstrated scattered ground glass infiltrates due to complicating pneumocystis pneumonia. In comparison to corresponding conventional chest radiography, CT examinations revealed more parenchymal lesions in five of eight cases (63%) and bilateral disease instead of unilateral disease in one of eight cases (13%). Feeding vessels were only identified on CT. Wegener granulomatosis is a primary necrotizing granulomatous vasculitis, and when it affects the lung it demonstrates CT features that are similar to other vessel-related disorders of the lung such as septic emboli, pulmonary infarcts, and tumor emboli of hematogenous metastases. We believe that these CT features--nodules with feeding vessels and wedge-shaped lesions abutting the pleura--are related to the necrotizing angiitis that affects pulmonary arteries and veins in this disease.     

Spontaneous life-threatening perirenal haemorrhage: an uncommon urological emergency

Spontaneous perirenal haemorrhage is a rare but potentially life-threatening condition and requires urgent management. Diagnosis is based on clinical assessment combined with radiological investigations, as reported by Belville et al. (Radiology 172:733, 1989) and Zagoria et al. (J Urol 145:468, 1991). Reported is a case of spontaneous perirenal haemorrhage diagnosed on computed tomography scan and then successfully embolized using coils.     

Wegener granulomatosis of the colon: CT and histologic correlation

Wegener granulomatosis is a systemic vasculitis with distinct clinical and histologic features often characterized with involvement of the upper airway, lung, and kidneys. The reported incidence of gastrointestinal involvement is relatively rare, with histologic confirmation often lacking. We report a case in which gastrointestinal involvement was the principal feature, with CT and histologic correlation.     

Spontaneous pneumothorax in flight as first manifestation of alpha-1 antitrypsin deficiency

Alpha-1 antitrypsin deficiency is a genetic condition that increases the risk of a variety of conditions including pulmonary emphysema and chronic liver disease. We report on a 33-yr-old male fighter pilot with early-onset pulmonary emphysema with bullae who developed spontaneous pneumothorax during in-flight combat training. A CT scan of his chest after removal of the chest tube showed multiple variably sized bullae and pulmonary cysts scattered throughout both lungs. Serum levels of alpha-1 antitrypsin were markedly decreased. Genetic analyses showed a PI ZZ genotype. Alpha-1 antitrypsin deficiency often goes undiagnosed by flight surgeons, which is a risk to flight safety.     

鼻部韦格纳肉芽肿的CT诊断

目的：探讨鼻部韦格纳肉芽肿的CT表现及其诊断价值。方法：回顾性分析8例经活检或术后病理证实的鼻部韦格纳肉芽肿，均经过CT检查，主要临床表现包括慢性鼻窦炎症状、鼻粘膜干燥结痂和鼻出血。结果：3例韦格纳肉芽肿局限于鼻部，其余5例为全身性韦格纳肉芽肿。CT表现：①鼻窦炎表现。②鼻腔内充以软组织影，鼻甲、鼻中隔破坏。③早期上颌窦内壁破坏，晚期鼻窦骨质增生、硬化，窦壁增厚，出现“双线”征。④2例随访观察鼻甲、鼻中隔破坏较前明显，4例鼻甲、鼻中隔改变未见加重，6例上颌窦壁骨质明显增厚，出现不规则“双线”征。窦腔变小。结论：鼻甲、鼻中隔破坏。鼻腔充以软组织影，鼻窦壁“双线”征是诊断本病有价值的征象；CT可准确显示鼻部韦格纳肉芽肿侵及范围；也能够帮助与其他病变进行鉴别。     

MR imaging of cardiac mass in Wegener granulomatosis

Wegener granulomatosis is a disorder of unknown etiology characterized by a necrotizing granulomatous vasculitis that primarily affects the paranasal sinuses, lungs, and kidneys. It is usually a disease of adults with infrequent cardiac involvement. We report the unusual presentation of this disorder in an adolescent with a cardiac mass. The findings on two-dimensional echocardiography and MR imaging are discussed.     

Wegener granulomatosis: MR imaging findings in brain and meninges

PURPOSE: To determine the spectrum of intracranial magnetic resonance (MR) imaging appearances of Wegener granulomatosis. MATERIALS AND METHODS: MR imaging studies in 19 patients with Wegener granulomatosis and possible central nervous system involvement were reviewed by two neuroradiologists. Intermediate-weighted and T2-weighted fast spin-echo MR images of the brain had been acquired in all patients, and spin-echo T1-weighted nonenhanced and gadolinium-enhanced images had been acquired in 18 patients. RESULTS: MR imaging findings included diffuse linear dural thickening and enhancement (n = 6); focal dural thickening and enhancement contiguous with orbital, nasal, or paranasal disease (n = 5); infarcts (n = 4); nonspecific white matter areas of high signal intensity on intermediate-weighted and T2-weighted images (n = 10); enlarged pituitary gland with infundibular thickening and enhancement (n = 2); a discrete cerebellar lesion that was probably granulomatous in origin (n = 1); and cerebral (n = 8) and cerebellar atrophy (n = 2). CONCLUSION: MR imaging demonstrated the wide spectrum of findings of central nervous system involvement in patients with Wegener granulomatosis and was particularly useful for the evaluation of direct intracranial spread from orbital, nasal, or paranasal disease.     

MR Imaging in Wegener granulomatosis of the spinal cord

Wegener granulomatosis is a multisystemic disorder characterized by necrotizing vasculitis that primarily involves the respiratory tract. The orbits, heart, skin, joints, and nervous system are frequently involved. We describe the MR imaging findings of Wegener granulomatosis in the cervical spine and correlate them with the histopathologic features. MR imaging showed epidural liquid masses surrounded by granulomatous masses that compressed the cervical myelin from behind.