Pulmonary angiitis and granulomatosis: a review

Vasculitis is a clinical-pathological process characterized by inflammation and necrosis of blood vessels. It has been effectively classified by Fauci. Granulomatosis in the lung may be angiocentric or bronchocentric in distribution. The angiocentric forms of granulomatosis and vasculitis include Wegener's granulomatosis, allergic angiitis and granulomatosis of Churg and Strauss, lymphomatoid granulomatosis, and necrotizing sarcoid granulomatosis. Wegener's granulomatosis is a well-defined syndrome characterized by necrotizing granulomatous vasculitis of the upper and lower respiratory tracts, segmental necrotizing glomerulonephritis, and systemic small vessel vasculitis. Allergic angiitis and granulomatosis is a less common multisystem vasculitis with many features similar to polyarteritis nodosa. Lymphomatoid granulomatosis is an angiocentric and angiodestructive lymphoreticular proliferative and granulomatous disease involving predominantly the lungs and resembling lymphoma. Necrotizing sarcoid granulomatosis is probably a variant of sarcoidosis in which an angiitis is a prominent feature. Because the radiology of these diseases can be similar, their important differences are highlighted. The appearance of multiple nodules, often with cavities, and pleural-based consolidations resembling pulmonary infarcts should suggest pulmonary angiitis and granulomatosis, especially if improvement occurs in one area while disease is progressing elsewhere. Bronchocentric granulomatosis is not a primary vasculitis but is discussed because of its similarity to the other diseases.     

韦格纳肉芽肿病肺部损害的影像分析

目的:探讨韦格纳肉芽肿病肺部影像学表现,提高对该病的诊断水平.方法:总结分析经病理证实的韦格纳肉芽肿27例肺部影像表现,男17例,女10例,年龄13～68岁,平均34.5岁.其中胸片27例,胸部CT平扫加增强扫描15例.结果:27例中肺部有异常表现 19例,占70.4%.异常表现有大片状浸润病灶5例,小片状浸润病灶4例,多发结节影(直径小于3 cm) 9例,肿块(直径大于3 cm) 2例,空洞11例,条状阴影4例,磨玻璃影3例,肺不张2例,支气管狭窄阻塞2例.13例多种病灶混合存在,并互相转化,病灶增多或减少.结论:韦格纳肉芽肿病肺部异常表现呈多样性,以片状浸润、结节和空洞病变最常见,薄壁空洞和环形空洞具特征性.此病要与肺癌、结核、肺炎与脓肿等疾病相鉴别.     

Pulmonary angiitis and granulomatosis

The presentation of a patient with multiple pulmonary nodules with or without cavitation and often with signs of a multisystemic vasculitis should suggest one of the pulmonary angiitis and granulomatosis syndromes. The five conditions traditionally considered together in the category of pulmonary angiitis and granulomatosis differ widely in their cause and pathogenesis and are more appropriately considered as variants or relatives of other processes. The radiologic features of this group of diseases, however, are similar, and it is useful to still consider them together. Table 1 summarizes the radiologic features of these conditions.     

Cavitary pulmonary nodules in atypical collagen disease and lupoid drug reaction. Report of two cases

The case histories of two patients with cavitary pulmonary nodules and the findings at chest radiography are reviewed. The first patient had a connective tissue disease with features common to systematic lupus erythematosus and Wegener's granulomatosis. In the second patient the lung changes developed as part of a drug reaction to carbamezapine and/or phenytoin. The common denominator of the cavitating nodules is probably the presence of granulomas, developing as a sequela of pulmonary vasculitis.     

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.     

Wegener's granulomatosis: findings from computed tomography of the chest in 10 patients.

Radiographs and computed tomography (CT) scans of the chest were reviewed for 10 patients with pathologically proven Wegener's granulomatosis. The CT scans revealed multiple pulmonary nodules in seven patients and a single nodule in one. The nodules ranged in diameter from 2 mm to 7 cm, and most had irregular margins. All of the nodules larger than 2 cm in diameter showed evidence of cavitation in the CT scans. Additional CT findings included associated areas of consolidation (in two patients), pleural thickening (in two) and pleural effusion (in two). Chest radiographs were available for eight patients, and the CT scans contributed information additional to that available from the radiographs for seven of these. In one patient lung nodules were visible in the CT scans but could not be distinguished from surrounding areas of consolidation in the chest radiographs. CT revealed additional nodules in five of the six patients in whom multiple nodules were seen in chest radiographs and in one of these also revealed cavitation tht was not visible in plain radiographs. CT excluded the possibility of a nodule that was suspected from the chest radiographs in a patient who had been treated previously for Wegener's granulomatosis. The authors conclude that Wegener's granulomatosis is characterized in CT scans by multiple nodules with irregular margins and by cavitation in nodules larger than 2 cm in diameter. CT may also demonstrate nodules and cavitation not apparent in radiographs.     

The radiology of lymphomatoid granulomatosis in the lung

This paper describes the salient clinical and radiological features of two patients with lymphomatoid granulomatosis of the lungs. We have, in addition, reviewed the previously documented 22 cases in which radiographs were published along with their case histories. The commonest radiographic appearance, that of bilateral multiple pulmonary nodules, should not be mistaken for multiple metastases.     

中线恶性肉芽肿的影像学研究(附10例报告)

目的　探讨中线恶性肉芽肿鼻、鼻窦、咽及肺部病变的影像学特征及鉴别诊断。材料与方法　回顾分析 10例CT及X线表现。结果　所有病例均经组织学证实。 10例中有 4例鼻腔、鼻窦、咽部与肺部病变并存。肺部表现为空洞及结节病变。结论　中线恶性肉芽肿在鼻腔及鼻窦的病变具有明确的CT特征 ,即弥漫浸润病灶伴骨质破坏。咽部病变与早期鼻咽癌和炎症区别困难 ,需靠组织学诊断。肺部空洞与结节病变在鼻、咽部中线恶性肉芽肿组织学确诊后可以考虑肺部受侵的诊断     

Small-vessel diseases of the lung: CT-pathologic correlates

Diseases that primarily affect the small vessels of the lung are difficult to diagnose. Many conditions are characterized by involvement of small pulmonary vessels, and pathologically they can be conveniently divided into occluding and inflammatory types. The former, typified by chronic pulmonary thromboembolism and primary pulmonary hypertension, are relatively cryptic in terms of imaging. In contrast, inflammatory vasculitides, which often cause pulmonary hemorrhage and infarction, result in florid but nonspecific radiographic abnormalities. The spectrum of thin-section computed tomographic abnormalities encountered in the inflammatory vasculitides is wide: For example, in Wegener granulomatosis the pattern ranges from cavitating nodules to lobar consolidation to ground-glass opacity. This review highlights some of the less obvious imaging manifestations of occlusive and inflammatory diseases of the small pulmonary vessels.     

Active disease and residual damage in treated Wegener's granulomatosis: an observational study using pulmonary high-resolution computed tomography

The purpose of this study was to determine to what extent high-resolution computed tomography (HRCT) of the lungs can distinguish active inflammatory disease from inactive cicatricial disease in patients treated for Wegener's granulomatosis (WG). Twenty-eight WG patients with active pulmonary disease underwent a first HRCT examination immediately before standard immunosuppressive treatment and a second examination after clinical remission had been achieved. Lesions remaining after treatment were categorized as residual damage and were compared with findings during active disease to see by what features active and cicatricial disease can be distinguished. During active disease 17 patients had nodules/masses, 12 had ground-glass opacities, 6 had septal lines and 6 had non-septal lines. After treatment, ground-glass opacities had resolved completely. Nodules/masses had resolved in 8 patients and had diminished in 7 patients. Residual nodules were distinguished from nodules/masses in active disease by lack of cavitation and a diameter of mostly <15 mm. In one-third of patients lines resolved, but in 8 instances new lines evolved during immunosuppression. During a follow-up period of a median 26.5 months (range 20.0–33.8), patients with residual nodules or lines had no more relapses than patients with completely cleared lungs. Treated pulmonary WG leaves substantial residual damage. High-resolution CT does assist in the distinction between active and inactive lesions. Ground-glass opacities, cavitating nodules/masses and masses measuring more than 3 cm represent active disease ordinarily. Non-cavitary small nodules and septal or non-septal lines can be either active or cicatricial lesions. The nature of these lesions needs to be clarified by longitudinal observation. Electronic Publication     

Pulmonary tuberculosis: another disease showing clusters of small nodules

OBJECTIVE: Pulmonary sarcoidosis was recently reported to show the "sarcoid galaxy" sign, indicating large pulmonary nodules composed of coalescent small nodules. The purpose of this study was to review cases of pulmonary tuberculosis showing CT features indistinguishable from the sarcoid galaxy sign. CONCLUSION: Large nodules arising from the coalescence of small nodules may be seen in active tuberculosis and in sarcoidosis. The CT finding was termed "clusters of small nodules" instead of the "sarcoid galaxy sign" in this article. A single cluster of small nodules, clusters of small nodules in the superior segment of the lower lobe, or clusters of small nodules not associated with lymphadenopathy or associated with tree-in-bud lesions would favor the diagnosis of active pulmonary tuberculosis rather than pulmonary sarcoidosis.     

Necrotizing sarcoid-like granulomatosis: radiologic-pathologic correlation

Necrotizing sarcoid-like granulomatosis is one of five forms of pulmonary disease characterized histologically by angiitis and granulomata formation. Although there have been several reports of the clinical and pathological characteristics of the disease, there has been little reported radiographic correlation. We present two patients with necrotizing sarcoid-like granulomatosis with radiographic and pathologic correlation. The radiographic finding of diffuse lesions, either bilateral nodules or nodular densities with or without cavitation, should prompt definitive diagnostic evaluation. Tissue diagnosis is imperative as necrotizing sarcoid-like granulomatosis has a relatively good prognosis when compared with other similar granulomatous diseases.     

Wegener肉芽肿影像表现

目的提高对Wegener肉芽肿影像认识。方法收集本院1997-01～2004-04资料完整临床确诊为Wegener肉芽肿病例10例,回顾性分析其影像学表现。结果8例鼻窦受累,表现为双侧鼻窦黏膜不同程度增厚,其中3例中线结构破坏。2例眼眶浸润。1例喉狭窄。7例肺部病变,其中3例表现为典型的多发结节和空洞,其余表现为双肺散在渗出、小结节,多种征象并存,1例支气管阻塞合并左上肺不张。结论Wegener肉芽肿累及多器官,影像表现多样和复杂,诊断应结合临床、实验室检查和病理动态观察。     

高频彩超及弹性成像对甲状腺结节良恶性的鉴别诊断价值

目的：对甲状腺小结节高频彩超及弹性成像表现进行多因素 Logistic 回归分析,探讨其对良恶性鉴别诊断的价值。方法：回顾性分析经手术病理确诊的126个直径≤2 cm 的甲状腺结节的超声表现,根据每个结节的高频超声及弹性成像声像图特征进行分类,采用二分类多因素 Logistic 回归分析,筛选出评价甲状腺恶性结节的因素,建立概率方程。结果：根据多因素 logistic 回归分析结果,边界不清、纵横比≥1、微钙化、极低回声、弹性成像评分是鉴别甲状腺小结节良恶性的关键因素。回归模型预测超声诊断甲状腺结节良恶性的符合率为89．7％,其中良性结节的诊断符合率为90．0％,恶性结节的诊断符合率为89．3％,ROC 曲线下面积为0．959。结论：多因素 Logistic 回归模型可筛选出对甲状腺小结节良恶性有鉴别诊断意义的特征性变量,综合评价各个变量,有利于甲状腺结节良恶性的鉴别诊断。     

Serial high-resolution computed tomography imaging in patients with Wegener granulomatosis: differentiation between active inflammatory and chronic fibrotic lesions

Purpose: To evaluate pulmonary pathologies in Wegener granulomatosis with sequential computed tomography (CT) in order to differentiate active inflammatory lesions from chronic fibrotic lesions.

Material and Methods: Serial CT findings in 38 patients with Wegener granulomatosis were retrospectively analyzed (mean follow-up period, 21 months). The presence, extension, and distribution of the following findings were evaluated with CT: parenchymal nodules, masses, ground-glass attenuation, airspace consolidation, bronchial wall-thickening, bronchiectasis, linear areas of attenuation, pleural irregularities, pleural effusions, hilar and mediastinal lymphadenopathy.

Results: Observed in 92% of patients, nodules were the most common CT pathology. Areas of ground-glass attenuation, consolidation, masses of linear attenuation, and tracheal/bronchial wall-thickening were detected in 24%, 26%, 32%, 39%, and 68% of patients. At follow-up, the clearance of lesions was most consistent for areas of ground-glass attenuation (89%), masses (87%), and cavitated nodules (85%). In the follow-up scan, 58% of all nodules, 47% of pulmonary consolidations, and 66% of bronchial wall-thickening were completely resolved. Areas of bronchiectasis and septal/non-septal lines remained stable in 70% and 71% of patients.

Conclusion: The majority of the lesions decreased or resolved completely with or without areas of linear attenuation. Ground-glass attenuation, cavitated nodules and masses appear to represent active inflammatory lesions. In most probability, areas of bronchiectasis and septal/non-septal lines more often represent chronic fibrotic changes rather than active inflammatory changes. In combination with clinical evaluation and bronchoscopy, CT assists in the assessment of disease activity.     

CT presentation of Wegener's granulomatosis in a child: rapidly progressive changes of pulmonary nodules to cavities

We present a child with Wegener's granulomatosis who showed lung lesions progressing from nodules to cavities within a 1-month period on CT.     

Halo sign on high resolution CT: findings in spectrum of pulmonary diseases with pathologic correlation.

The halo sign in a pulmonary nodule refers to the condition in which soft tissue attenuation of a pulmonary nodule is surrounded by peripheral ground glass attenuation on high resolution CT. The halo sign can be caused by several pathologic processes: hemorrhagic pulmonary nodules, tumor cell infiltration, and nonhemorrhagic inflammatory lesions. Hemorrhagic pulmonary nodules may occur in infectious diseases including invasive pulmonary aspergillosis, mucormycosis, and candidiasis and noninfectious diseases including Wegener granulomatosis and primary and metastatic hemorrhagic tumors. Tumor cell infiltration in bronchioloalveolar carcinoma, pulmonary lymphoma, and pulmonary metastatic neoplasm may appear with the halo sign. Eosinophilic lung disease and organizing pneumonia are representative of inflammatory lesions showing the sign.     

肺韦格纳肉芽肿病CT征象分析

目的：探讨肺韦格纳肉芽肿痛的临床及CT表现,进一步提高对该病的认识及诊断水平。方法：回顾分析经病理证实的11例肺韦格纳肉芽肿痛的临床及影像学资料,总结其临床及影像学特点。结果：儿例患者中,多发肿块、结节影10例（91％）,伴空洞形成8例,病灶周围毛刺征3例,胸膜凹陷征1例,晕征4例,血管供给征6例。浸润性阴影4例（36％）,其中3例伴多发结节及空洞形成。气道受累3例（27％）,胸膜受累2例（18％）。抗中性粒细胞胞质抗体（c—ANCA）阳性10例,cANCA阴性1例。结论：多发病灶周围的毛刺征、晕征以及伴发的气道改变对韦格纳肉芽肿病的鉴别诊断具有重要意义。     

MRI appearance of pulmonary Wegener's granulomatosis with concomitant splenic infarction

The MRI appearance of pulmonary lesions in a patient with Wegener's granulomatosis is presented. Pulmonary involvement was characterized by multiple solid and cavitated nodules connected to vessels. Transbronchial biopsy demonstrated typical findings of granuloma. The spleen presented diffuse low signal intensity and an enhancing capsule on T1-weighted images, and inhomogeneously high signal intensity on T2-weighted images. This appearance of diffuse infarction could result from the vasculitis caused by Wegener's disease. Received: 10 April 1997; Accepted 7 July 1997     

Thoracic manifestation of Wegener's granulomatosis: CT findings in 30 patients

Our objective was to describe the CT findings of thoracic Wegener's granulomatosis. At presentation, both conventional and thin-section CT scans were available in 30 patients with Wegener's granulomatosis. Serial CT scans (range of intervals: 1–25 months, mean 4.5 months) were available in 20 patients. The initial and follow-up CT scans were analyzed retrospectively by two observers in terms of pattern and extent of parenchymal and airway lesions. Positive CT findings were seen in 29 of 30 (97%) patients at initial presentation. The most common pattern was nodules or masses seen in 27 of 30 (90%) patients. They were multiple in 23 of 27 (85%) patients, bilateral in 18 (67%), subpleural in 24 (89%), and peribronchovascular in 11 (41%) in distribution. Bronchial wall thickening in the segmental or subsegmental bronchi was seen in 22 (73%) patients. Large airways were also abnormal in 9 (30%) patients. Patchy areas of consolidation and ground-glass opacity were seen in 7 (23%) patients, respectively. In 17 of 20 (85%) patients in whom follow-up CT scans were available, the parenchymal or airway lesion showed complete or partial improvement with treatment. The CT findings of Wegener's granulomatosis, although multiple and variable, consist mainly of bilateral subpleural or peribronchovascular nodules or masses and bronchial wall thickening in the segmental or subsegmental bronchi. Parenchymal and airway lesions improve with treatment in most patients. Electronic Publication