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The presence of pulmonary vasculitis can be suggested by a clinical presentation that includes diffuse pulmonary hemorrhage, acute glomerulonephritis, chronic refractory sinusitis or rhinorrhea, imaging findings of nodules or cavities, mononeuritis multiplex, multisystemic disease, and palpable purpura. Serologic tests, including the use of cytopla...
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... in these three diseases, pul- monary vessels are rarely involved ( 1 , 15 ). Therefore, we do not discuss these three diseases in this review nor do we include these diseases in Figure 1 . The vasculitides that are not included in the Chapel Hill consensus classifi cation but involve pulmonary vasculature com- prise isolated pauci-immune pulmonary capillaritis, vasculitis associated with collagen vascular diseases that include ...
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... disease, SLE, drug-induced vas- culitis, Goodpasture syndrome, immu- noglobulin (Ig) A nephropathy, and Hughes-Stovin syndrome ( Fig 1 ). We will discuss these diseases in addition to the ones included in the original Chapel Hill classifi cation. ...
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... ANCA is positive in 40%-80% of patients. The Chapel Hill consensus conference recognized microscopic polyangiitis as its own entity ( 15 ) (Fig 1). ...
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... radiographic features of DAH con- sist of patchy, bilateral air-space opac- ities ( 71 ) ( Figs 8 -10 ). DAH is usually seen with extensive bilateral air-space consolidation and GGO, but may be more prominent in the perihilar areas and in the middle and lower lung zones, sparing the lung apices and costophrenic angles ( 71 ) ( Fig 8 ). ...
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... most common CT features of DAH are bilateral GGOs and consolida- tion ( Figs 8 -12 ). The lesions are diffuse in the upper and lower lobes in approx- imately three-fourths of patients ( Figs 9 -12 ) or are localized in the lower part of the lungs in 25% of patients ( 70 ) ( Fig 8 ). ...
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... fi ndings may show improvement in the course of hemorrhage resorption. Additionally, ill-defi ned centrilobular nodules may be present, refl ecting intra- alveolar accumulation of pulmonary macrophages ( Fig 11 ). Nodules have been reported to be uniform in size (1-3 mm in diameter) and are diffusely distributed with no zonal predominance ( 73 ). ...
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... effusion, small in amount, may occur, but is uncommon ( 70 , 74 ). The air-space lesions histopathologi- cally correlate with pulmonary hemor- rhage, both with or without capillaritis ( 71 ) ( Figs 8 , 10 ). Typical MR features attributable to paramagnetic properties of hemosiderin in DAH are expected to be observed, with diffusely increased signal intensity on T1-weighted MR images and mark- edly reduced signal intensity on T2- weighted MR images ( 75 ). ...
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... interstitial fi brosis, probably associated with recurrent hemorrhage, has been occasionally reported in pulmonary vas- culitis and DAH ( 18 ). CT fi ndings in this group of patients may simulate idio- pathic pulmonary fi brosis or nonspecifi c interstitial pneumonitis by showing hon- eycombing, reticulation, and traction bronchiectasis ( 18 ) ( Fig 13 ). ...
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... fi nd- ings of vascular involvement include medial thickening, elastic fi ber splitting, and perivascular round cell infi ltration ( 83 ). The four types of vascular lesions recognized include arterial occlusion, venous occlusion, aneurysms ( Figs 14 , 15 ), and varices. The presence of an aneu- rysm and occlusion are the most com- mon fi ndings ( 83 -85 ). ...
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... fi ndings include the presence of a lung mass due to aneu- rysms of pulmonary arteries ( Fig 14 ), wedge-shaped air-space consolidation due to infarction or hemorrhage, and mediastinal widening due to thrombo- sis of the superior vena cava ( Fig 15 ). Aneurysms in Behçet disease are fusi- form to saccular, are commonly multi- ple in number and bilateral, and are located in the lower lobes or the main pulmonary arteries ( 86 ). ...
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... fi ndings include the presence of a lung mass due to aneu- rysms of pulmonary arteries ( Fig 14 ), wedge-shaped air-space consolidation due to infarction or hemorrhage, and mediastinal widening due to thrombo- sis of the superior vena cava ( Fig 15 ). Aneurysms in Behçet disease are fusi- form to saccular, are commonly multi- ple in number and bilateral, and are located in the lower lobes or the main pulmonary arteries ( 86 ). ...
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... use of CT imaging can help characterize disease- induced or therapy-induced changes. Aneurysmal wall thickening represents subadventitial hematoma formation ( Fig 14 ), and perianeurysmal air-space consolidation or GGO is indicative of an impending rupture ( 87 -90 ). Additional fi ndings include peripheral mosaic per- fusion resulting from focal air trapping, thrombotic small-vessel occlusion ( Fig 15 ) or mechanical vascular compression by aneurysms, and organizing or eosin- ophilic pneumonia ( 90 ). ...
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... wall thickening represents subadventitial hematoma formation ( Fig 14 ), and perianeurysmal air-space consolidation or GGO is indicative of an impending rupture ( 87 -90 ). Additional fi ndings include peripheral mosaic per- fusion resulting from focal air trapping, thrombotic small-vessel occlusion ( Fig 15 ) or mechanical vascular compression by aneurysms, and organizing or eosin- ophilic pneumonia ( 90 ). Acute arterial hemorrhage is a well-recognized com- plication of vasculitis, and the rupture of a pulmonary artery into a bronchial lumen or into the parenchyma occurs in up to 50% of patients with pulmonary artery aneurysms ( 86 ). ...
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... artery aneurysm has been attributed to weakening of vessel walls due to infl am- mation. Pathologic features include systemic thrombi in the vena cava, ce- rebral sinuses, or limb veins; pulmo- nary artery occlusions due to emboli or thrombi; and one or more segmental pulmonary artery aneurysms, frequently associated with bronchial artery aneu- rysms ( 93 ) ( Fig 16 ). ...
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... vasculitis in SLE that mainly involves arterioles within the secondary pulmonary lobules may ap- pear with centrilobular small nodules or tree-in-bud opacities of vascular ori- gin at CT ( Fig 17 ). In SLE, pulmonary hypertension occurs in 0.5%-14% of patients ( 102 ). ...
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... gemcitabine, diphe- nylhydantoin, transretinoic acid, and crack cocaine may cause pulmonary capillaritis and DAH ( Fig 10 ) ( 1 , 104 - 107 ). Immune mechanisms that include ANCA-related vasculitis have been re- garded as responsible for the underly- ing pathogenesis of such drug-induced pulmonary vasculitis. ...
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... deposition of IgG and complement can be demon- strated in the basement membranes of the alveoli ( 110 ). As seen on CT images, DAH is the key feature of pulmonary in- volvement ( Fig 12 ) and typically resolves within a few days. On rare occasions, the pulmonary changes may be isolated without renal involvement ( 110 ). ...
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... involving capillaries or arterioles usually demon- strates DAH or small centrilobular nod- ules and tree-in-bud signs of vascular origin. However, the differential diagno- sis from other nonvascular diseases is not easy; therefore, it is essential to in- tegrate clinical, laboratory, and imaging fi ndings to make a specifi c diagnosis of pulmonary vasculitides ( Fig 18 ). Figure 18: Flowchart shows the approach for reaching a specifi c diagnosis of pulmonary vasculitis in patients with DAH, glomerulonephritis, upper respiratory tract disease, mononeuritis multiplex, multisystemic disease, or palpable purpura. ...
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... the differential diagno- sis from other nonvascular diseases is not easy; therefore, it is essential to in- tegrate clinical, laboratory, and imaging fi ndings to make a specifi c diagnosis of pulmonary vasculitides ( Fig 18 ). Figure 18: Flowchart shows the approach for reaching a specifi c diagnosis of pulmonary vasculitis in patients with DAH, glomerulonephritis, upper respiratory tract disease, mononeuritis multiplex, multisystemic disease, or palpable purpura. Cytoplasmic ANCA positivity allows one to make a confi dent diagnosis of We- gener granulomatosis and perinuclear ANCA positivity suggests presence of CSS or MPA. ...
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Citations
... Cough occurred more frequently in GPA than MPA (p=0,02) perhaps due to more endobronchial lesions. CT imaging or, rarely, chest X-ray before bronchoscopies are helpful to analyse lung lesions, exclude other aetiologies, and guide sampling before bronchoscopy [20]. ...
Objectives
This study describes data from bronchoscopy performed at the diagnosis of ANCA-associated vasculitis (AAV).
Methods
We conducted a retrospective study between 2004 and 2019 in patients older than 18 years old with a diagnosis of microscopic polyangiitis (MPA) or granulomatosis with polyangiitis (GPA) who underwent bronchoscopy at onset of the disease. We collected bronchoalveolar lavage (BAL) and histological findings obtained during bronchoscopy.
Results
Two hundred and seventy-four patients with AAV were identified. Among 92 bronchoscopies, 62 were performed at diagnosis and 58 procedures were finally analysed. Cough was more frequent in patients with MPA than GPA (p=0.02). The presence of endobronchial lesions (24.1%) was found to be significantly associated with GPA (p<0.0001) and PR3-ANCA (p=0.01). The most frequent endobronchial lesions were inflammation and hyperaemia of the bronchial mucosa (50%), followed by stenoses (28%), ulcerations (21%) and mass-like granulomatosis (7%). The diagnostic yield of bronchial biopsies was useful for visible lesions (66.6% versus 0%; p=0.006). On BAL, diffuse alveolar haemorrhage (DAH) was detected in 31 patients (53.4%) and was more frequent in MPA patients (70.4% versus 38.7%; p=0.016). In 16.1% of DAH, BAL confirms the diagnosis despite the absence of clinical or biological arguments. The incidence of microbial infections on BAL (38%) was similar between MPA and GPA (p=0.54).
Conclusion
Bronchoscopy is an informative procedure at the onset of AAV disease in patients with respiratory manifestations. Endobronchial lesions are more frequently found in GPA and should be biopsied. BAL can be used to confirm DAH or diagnose superadded infection.
... This pattern raised our concern regarding the working diagnosis of COP, which is typically initially highly sensitive to corticosteroids, and led us to continue with invasive procedures. A differential diagnosis of "partially resolved with steroids" pulmonary infiltrates can be challenging and includes, among others lymphoma, DAH, different connective tissue diseases, and in rare cases vasculitis [3]. Signs of macro and microscopic alveolar hemorrhage on BAL, presence of intra-alveolar hemosiderin- laden macrophages, only mild septal thickening, and foci of chronic inflammation on cryobiopsy, along with the positive serological results of P-ANCA, allowed us to conclude that organizing pneumonia was the presenting syndrome, co-existing with DAH due to an underlying pulmonary ANCA associated-vasculitis. ...
A 57-year-old female presented with sudden shortness of breath and migratory pulmonary infiltrates on imaging, which corresponds with a diagnosis of cryptogenic organizing pneumonia. Initial treatment with corticosteroids showed only mild improvement during follow-up. BAL was performed and revealed diffuse alveolar hemorrhage. Immune testing showed positive P-ANCA with positive MPO, leading to a diagnosis of microscopic polyangiitis.
... Although thoracic involvement of ANCA-associated vasculitides is varied and poorly specific, some imaging features can help in the differential diagnosis. [37][38][39] The most common CT finding of GPA is bilateral multiple pulmonary nodules or consolidations that may cavitate. The other imaging findings are GGOs frequently with peribronchovascular and perihilar distribution, airway thickening, bronchiectasis, and centrilobular nodules. ...
... The other imaging findings are GGOs frequently with peribronchovascular and perihilar distribution, airway thickening, bronchiectasis, and centrilobular nodules. 37,40 The main imaging findings of EGPA are bilateral transient, patchy, non-segmental GGOs and consolidations without predilection of any lung lobe. Interlobular septal thickening because of edema and eosinophilic infiltration, bronchial dilatation, and wall thickening due to airway involvement can also occur. ...
Coronavirus disease 2019 (COVID-19) is associated with pneumonia and has various pulmonary manifestations on computed tomography (CT). Although COVID-19 pneumonia is usually seen as bilateral predominantly peripheral ground-glass opacities with or without consolidation, it can present with atypical radiological findings and resemble the imaging findings of other lung diseases. Diagnosis of COVID-19 pneumonia is much more challenging for both clinicians and radiologists in the presence of pre-existing lung disease. The imaging features of COVID-19 and underlying lung disease can overlap and obscure the findings of each other. Knowledge of the radiological findings of both diseases and possible complications, correct diagnosis, and multidisciplinary consensus play key roles in the appropriate management of diseases. In this pictorial review, the chest CT findings are presented of patients with underlying lung diseases and overlapping COVID-19 pneumonia and the various reasons for radiological lung abnormalities in these patients are discussed.
... Approximately 70% of patients with EGPA present with pulmonary involvement, such as patchy, transient, and non-segmental opacities that have no predilection for any lung zone. Since intestinal vasculitis related to EGPA could only present as increased wall enhancement on MRE/CTE without gross mural thickening, attention should be paid beyond the GI tract searching for important clues such as infarction of the visceral organs or lung involvement, particularly in patients with a history of asthma and eosinophilia [81]. ...
Diagnosis of intestinal vasculitis is often challenging due to the non-specific clinical and imaging findings. Vasculitides with gastrointestinal (GI) manifestations are rare, but their diagnosis holds immense significance as late or missed recognition can result in high mortality rates. Given the resemblance of radiologic findings with some other entities, GI vasculitis is often overlooked on small bowel studies done using computed tomography/magnetic resonance enterography (CTE/MRE). Hereon, we reviewed radiologic findings of vasculitis with gastrointestinal involvement on CTE and MRE. The variety of findings on MRE/CTE depend upon the size of the involved vessels. Signs of intestinal ischemia, e.g., mural thickening, submucosal edema, mural hyperenhancement, and restricted diffusion on diffusion-weighted imaging, are common in intestinal vasculitis. Involvement of the abdominal aorta and the major visceral arteries is presented as concentric mural thickening, transmural calcification, luminal stenosis, occlusion, aneurysmal changes, and collateral vessels. Such findings can be observed particularly in large- and medium-vessel vasculitis. The presence of extra-intestinal findings, including within the liver, kidneys, or spleen in the form of focal areas of infarction or heterogeneous enhancement due to microvascular involvement, can be another radiologic clue in diagnosis of vasculitis . The link between the clinical/laboratory findings and MRE/CTE abnormalities needs to be corresponded when it comes to the diagnosis of intestinal vasculitis.
... 61 Pleural effusion can be seen in up to 50% of cases and is usually secondary to eosinophilic cardiomyopathy or pleuritis. 62 CT is better for characterization of the parenchymal findings of EGPA. In one of the retrospective studies, 61 CT showed pulmonary parenchymal abnormalities with a bronchiolocentric pattern in around 76% of the patients and with an airspace pattern in approximately 40% of patients. ...
Non-infectious granulomatous lung diseases represent a group of disorders with relatively non-specific clinical history and imaging findings. Following pathologies are enlisted, (1) Inflammatory: sarcoidosis and necrotizing sarcoid granulomatosis (NSG); (2) Pulmonary lymphoid lesions: lymphomatoid granulomatosis (LYG) and granulomatous-lymphocytic interstitial lung disease (GLILD); (3) Aspiration/exposure: aspiration pneumonia, talcosis, berylliosis, and hypersensitivity pneumonitis (HP); (4) Vasculitis: granulomatosis with polyangiitis and eosinophilic GPA (EGPA) (formerly called as Churg-Strauss syndrome); and (5) Collagen vascular disorders: rheumatoid lung nodules. Role of radiologist is to narrow the diagnosis combining the imaging findings with clinical findings thus following a multidisciplinary approach.
... Pulmonary involvement occurs in 50%-90% of patients. It is not very specific, with a cough (75% of cases), sometimes with purulent sputum, moderate dyspnea (50%), and hemoptysis (30%-40%) indicating a focal lesion (excavated nodule) or diffuse alveolar hemorrhage [10]. Chest pain is usually related to pleural origin (exudative pleurisy, pneumothorax complicating an excavated nodular parenchymal lesion, or bronchopleural fistula) [11]. ...
Granulomatosis with polyangiitis (GPA) is a necrotizing granulomatous vasculitis of medium- and small-caliber vessels associated with the presence of antineutrophil cytoplasm antibodies (ANCAs) and antibodies specific for proteinase 3 (anti-PR3). The interest of this case lies on the fact that these antibodies are directed against myeloperoxidase revealed by the presence of scattered multiple pulmonary nodules.
We report a 65-year-old-female patient who presented with a productive cough with mucus sputum associated with a cephalea for six months. The chest x-ray showed multiple pulmonary nodules, first suggesting a neoplastic origin. The initial etiological assessment was non-contributory. A month later, the patient developed pulmonary condensations and ocular signs. The etiological assessment then found ANCA anti-myeloperoxidase (anti-MPO)-GPA. A good knowledge of the clinical and radiological signs of GPA is important to quickly guide the diagnosis that will condition the prognosis of this disease.
... To enable standardized reporting, CO-RADS were coined by the Dutch Radiological Society reporting the typical CT pattern of COVID-19 pneumonia; characterized by the consistent presence of peripheral ground-glass opacities associated with multilobar, and posterior involvement, bilateral distribution, and subsegmental vessel enlargement [114]. Vessel enlargement described in the vicinity of ground-glass opacity areas was compatible with the thrombo-inflammatory processes [115][116][117][118][119][120]. Sub-segmental vascular enlargement (more than 3 mm diameter) in areas of lung opacity was observed in 89% of patients with confirmed COVID-19 pneumonia. ...
Thromboembolic manifestations of the eye can vary from a trivial tributary retinal vein occlusion to a catastrophic cerebral venous sinus thrombosis. These conditions can be classified as pathologies directly affecting the eye or those causing secondary lesions due to systemic issues and can be managed accordingly. Also, recently the incidence of thrombotic phenomenon affecting multiple organs (with the eye being no exception) is estimated to be around 25% among patients hospitalized in the intensive care unit for COVID-19, even though anticoagulant treatment was administered prophylactically. In this chapter, the various pathophysiologies of the ocular thrombotic events are highlighted with a special focus on the COVID-19 induced thrombotic ocular complications. Ophthalmologists, sometimes being the first responder, have a vigilant role to play with a heightened awareness of these atypical extrapulmonary thrombotic ocular manifestations, which are not only vision-threatening; in certain instances, life-threatening too. This chapter summarizes the recent advances in ocular thrombotic diseases with focal points on the current recommendations in COVID-19 induced ocular thrombotic complications. The potential diagnostic and preventive actions such as the prophylactic role of anti-thrombotic therapy, baseline non-contrast chest computed tomography, as well as recommendations for patients with COVID-19 infection are discussed in detail.
... In addition, nodular lesions may have a multiple and bilateral distribution along the bronco-vascular bundles-involving mainly the subpleural regions and having no craniocaudal predilection. In some cases, HRCT could also reproduce focal "halo sign" with a rim of groundglass opacity GGO surrounding a pulmonary solid nodule; diffuse alveolar hemorrhage ( Figure 8) and crazy paving pattern may be present [13][14][15][16]. GPA shows a histopathological pattern that comprehends areas of pulmonary consolidation with necrosis (in which neutrophilic micro abscesses are often present) and vasculitides; more in detail, arterioles, venules, capillaries may be involved, with focal and eccentric distribution. ...
Vasculitides represent a heterogeneous group of immune-mediated disorders, characterized by a systemic inflammatory destructive process of the blood vessels resulting either in ischemia or hemorrhage. The organ involved and vessel size influence the pattern of presentation of the pathology. The lung is commonly involved in systemic vasculitides, with heterogeneous clinical, radiological, and histopathological presentations. Primary vasculitides most commonly associated with lung parenchymal involvement include small-vessel antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides, such as granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), and microscopic polyangiitis (MPA). Several studies have reported cases of interstitial lung diseases (ILDs) associated with systemic vasculitis, particularly those positive for ANCA associated vasculitis/vasculitidis: AAV. We have selected from our case series different radiological features of pulmonary vasculitis (i.e., solitary or multiple nodules, cavitary lesions, nodules with centrilobular or peribronchial distribution, airspace consolidations, “crazy paving” appearance, interstitial disease), including cases with interstitial lung alterations. Therefore, the aim of this review is to describe the typical clinical manifestations of vasculitides and their main radiologic features (especially AAV).
... Background Granulomatosis with polyangiitis is a multi-system necrotizing vasculitis which typically involves respiratory system and kidneys [1][2][3][4] . Radiographic presentation of pulmonary manifestations is categorized into four patterns [5][6][7][8] . Most common radiologic presentation includes multiple nodules of various sizes in random distribution throughout the lungs with or without central cavitation [6][7][8] . ...
Granulomatosis with polyangiitis formerly known as Wegener's granulomatosis was first described by German pathologist Friedrich Wegener in 1936. It is a multi-system necrotizing noncaseating granulomatous vasculitis which affects small to medium-sized vessels. It can involve any organ system, most commonly the lungs and kidneys. American College of Rheumatology requires 2 of 4 criteria for diagnosis: Positive biopsy for granulomatous vasculitis, urinary sediment with red blood cells, abnormal chest radiograph and oral/nasal inflammation. Here we present a case of Granulomatosis with polyangiitis with brief review of literature.
... The common CT features of DAH are bilateral ground glass opacity and alveolar consolidation with air bronchogram, but these are generally nonspecific 54 . The CT features are characteristically seen as patchy or diffuse, and predominantly present in the upper and middle zones in two-third of the cases. ...
... The periphery of the lung is typically spared due to tapering of the pulmonary vasculature. With resorption of the hemorrhage, ill-defined centrilobular nodules may become visible resulting from the resolution of ground glass opacities and septal thickening may become visible 54 . ...
Microscopic polyangiitis (MPA) is an anti-neutrophil cytoplasmic antibody (ANCA)-associated necrotizing vasculitis, which mainly affects small vessels resulting in various organ involvement, especially the lung. The two key pulmonary manifestations, interstitial lung disease (ILD) and diffuse alveolar hemorrhage (DAH) increase the morbidity and mortality of patients with MPA. ILD is more common in MPA than in other ANCA-associated vasculitis subsets and is primarily associated with myeloperoxidase (MPO)-ANCA. Unlike alveolar hemorrhage due to pulmonary capillaritis, ILD can initially manifest as isolated pulmonary fibrosis. Of note, its most frequent radiographic pattern is the usual interstitial pneumonia pattern, similar to the characteristic pattern seen in idiopathic pulmonary fibrosis. We herein present the pathogenesis, clinical manifestations, radiographic and histopathologic features of ILD and DAH in MPA in this review. We also briefly summarize the outcome and therapeutic options for the two conditions.
