The lung in systemic vasculitis: radiological patterns and differential diagnosis

Transcription

1 The lung in systemic vasculitis: radiological patterns and differential diagnosis Poster No.: C-1530 Congress: ECR 2011 Type: Educational Exhibit Authors: B. Feragalli, C. Mantini, R. Polverosi, R. Patea, S. ganino, A Cotroneo ; Chieti/IT, PADOVA/IT, Chieti (CH)/IT Keywords: Connective tissue disorders, CT, Thorax, Lung DOI: /ecr2011/C-1530 Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do not in any way constitute or imply ECR's endorsement, sponsorship or recommendation of the third party, information, product or service. ECR is not responsible for the content of these pages and does not make any representations regarding the content or accuracy of material in this file. As per copyright regulations, any unauthorised use of the material or parts thereof as well as commercial reproduction or multiple distribution by any traditional or electronically based reproduction/publication method ist strictly prohibited. You agree to defend, indemnify, and hold ECR harmless from and against any and all claims, damages, costs, and expenses, including attorneys' fees, arising from or related to your use of these pages. Please note: Links to movies, ppt slideshows and any other multimedia files are not available in the pdf version of presentations. Page 1 of 32

2 Learning objectives The respiratory system may be involved in all systemic vasculitides, although with a variable frequency. Aim of our review is to describe radiographics and CT findings of pulmonary vasculitides and to correlate radiological findings with pathological results. Background INTRODUCTION The systemic vasculitides are a heterogeneous group of disorders characterized by an inflammatory destructive process affecting blood vessels. The lung is frequently involved with various clinical presentations. Pulmonary vasculitides may be: PRIMARY and often idiopathic disorders SECONDARY to conditions such as: infectious diseases, connective tissue diseases, malignancies and hypersensitivity disorders Diagnosis of primary pulmonary vasculitides requires knowledge of specific clinical, radiologic, laboratory and histopathologic features. CLASSIFICATION OF THE VASCULITIDES The pulmonary primary vasculitides are classified according to the size of the affected vessels (large, medium, small) Page 2 of 32

3 SYSTEMIC VASCULITIDES WITH PULMONARY INVOLVEMENT Pulmonary involvement is most commonly assciated with: PRIMARY SMALL-VESSEL ANTINEUTROPHIL CYTOPLASMIC AUTOANTIBODY (ANCA)-ASSOCIATED VASCULITIDES Wegener granulomatosis Churg-Strauss syndrome Microscopic Polyangiitis PRIMARY IMMUNE COMPLEX-MEDIATE VASCULITIDES Behçet disease Goodpasture syndrome Imaging findings OR Procedure details PRIMARY SMALL-VESSEL ANCA-ASSOCIATED VASCULITIDES ANCA-associated vasculitides include Wegener granulomatosis, Churg-Strauss Syndrome and Microscopic Polyangiitis and affect vessels smallar than arteries (arterioles, venules and capillaries) Page 3 of 32

4 ANCAs are antibodies against intracellular antigens of neutrophils and monocytes ANCA positivity is common but not always present; thus ANCA negativity does not completely rule out these vasculitides Diagnosis requires features of vasculitic involvement of capillaries and venules, such as purpura, glomerulonephritis or pulmonary capillaritis Respond to immunosuppressive treatment WEGENER GRANULOMATOSIS (WG) Wegener granulomatosis (WG) represent the most common ANCA-associated vasculitides with predilection for the respiratory tracts and the kidneys Is characterized clinically by the triad of: upper airway disease (nasal, oral and/or sinus inflammation) lower respiratory tract disease (airways and pulmonary parenchyma) glomerulonephritis The mean age at diagnosis is years The most common manifestations of upper airway involvement are rhinorrhea, epistaxis, sinusitis and otitis The most common HRCT abnormalities are lung NODULES, usually multiple and bilateral With progression of disease they tend to increase in size (can range from a few millimeters to more than 10 cm in diameter) and become cavitated. Cavities are usually thick walled with an irregular inner margin (1) The nodular lesions are often related to the vessels and they tend to involve mainly the subpleural regions but have no predilection for the upper or lower lung zones Page 4 of 32

5 The CT HALO SIGN, characterized by a rim of ground-glass opacity surrounding the pulmonary lesion, is frequently seen in patients with WG; this sign represents alveolar hemorrhage around the nodule (2) on page 18 Page 5 of 32

6 An AIR BRONCOGRAM is frequently seen within polmonary nodules of WG (3) on page 19 After steroid treatment cavitary lesions usually became thin walled (4) on page 19 Page 6 of 32

7 In cases of DIFFUSE ALVEOLAR HEMORRHAGE (DAH) CT features consist of bilateral ground-glass opacities and consolidations (related to the alveolar filling by blood) usually prominent in the perihilar areas with a relative sparing of the subpleural pulmonary parenchima (5) on page 20 Within days of an acute episode of hemorrhage, interlobular septal thickening may be seen in association with ground-glass opacity (CRAZY-PAVING PATTERN) as hemosiderin-laden macrophages accumulate in the interstitium (6) on page 20 Page 7 of 32

8 CHURG-STRAUSS SYNDROME (CSS) Churg-Strauss Syndrome (CSS) is characterized by the clinical triad of asthma, hypereosinophilia and necrotizing vasculitis DIAGNOSTIC CRITERIA: asthma more than 10% eosinophilia in a differential white blood cell count mononeuropathy or polyneuropathy attributable to systemic vasculitis migratory or transient pulmonary opacities paranasal sinus abnormalities extravascular eosinophils in a biopsy specimen The lung is the most commonly involved organ, followed by the skin The most common abnormality at HRCT is bilateral areas of ground-glass opacity or consolidation with a bilateral symmetric distribution and a peripheral predominance (Fig 7) on page 21. Other relatively common finding is airway involvement consisting of bronchial dilatation, bronchial wall thickening and small peribronchial and centrilobular nodules (Fig 7-8) related to eosinophilic infiltration of the bronchial wall and asthma Page 8 of 32

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10 Another common finding is the presence of regular and smooth interlobular septal thickening which may reflects the presence of edema secondary to cardiac involvement or eosinophilic infiltration of the septa (Fig.9) Page 10 of 32

11 MICROSCOPIC POLYANGIITIS (MP) Microscopic polyangiitis (MP) is a nongranulomatous necrotizing systemic vasculitis It is the most common cause of pulmonary-renal syndrome, a syndrome characterized by the coexistence of pulmonary hemorrhage and glomerulonephritis Page 11 of 32

12 In patients who develop lung disease, DAH with pathologic capillaritis is the most common manifestation The diagnosis of MP should be suspected in patients with rapidly progressive glomerulonephritis in whom perinuclear ANCA is present and who show clinical and radiologic findings consistent with diffuse pulmonary hemorrhage Clinically, the main differential diagnosis is with other conditions that may cause pulmonary and renal manifestations, particularly Goodpasture syndrome, Wegener granulomatosis and systemic lupus erythematosus The HRCT pattern is characterized by ground-glass opacities frequently associated with areas of consolidation (related to the complete filling of the alveoli with blood) usually bilateral, diffused or patchy (10). Page 12 of 32

13 PRIMARY IMMUNE COMPLEX-MEDIATE VASCULITIDES The primary immune complex-mediate vasculitides that most commonly involve pulmonary vessels are rapresented by Behçet disease and Goodpasture syndrome BEHÇET DISEASE Behçet disease is a chronic multisystemic vasculitis characterized by recurrent oral and genital ulcerations, ocular anomalies (uveitis) and additional clinical manifestations in multiple organ systems Behçet disease can involve large, medium and small vessels of both the arterial and venous circulation Pulmonary arteries are the second most common site of arterial involvement, preceded by the aorta Haemoptysis is the most common presenting symptom and is one of the leading causes of death Page 13 of 32

14 Behçet disease is the most common cause of pulmonary artery aneurysms ( 11) caused by inflammation of the vasa vasorum of the tunica media with destruction of the elastic fibers and dilatation of the vessel lumen Pulmonary artery aneurysms can be partially or totally thrombosed Thrombosis of the superior vena cava and of other mediastinal veins is often present (11) The most common parenchymal lesions are alveolar groung glass opacities representing areas of hemorrhage or wedge-shaped peripheral consolidations representing pulmonary infarctions Page 14 of 32

15 GOODPASTURE SYNDROME Rare small-vessel vasculitis (frequency of 1 case per million/year) characterized by the presence of antibodies that selectively targets antigens of the glomerular and alveolar basement membrane; it is a cause of pulmonary-renal syndrome. The radiographic lung feature consist of diffuse alveolar hemorrhage (12). Page 15 of 32

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17 DIFFUSE ALVEOLAR HEMORRHAGE (DAH) Diffuse alveolar hemorrhage is a clinical syndrome usually related to a primary smallvessels vasculitis in the lung. Is defined by the presence of haemoptysis, diffuse alveolar infiltrates and a drop in hematocrit level Wegener Granulomatosis, microscopic polyangiitis and Goodpasture syndrome are the most common cause of DAH Images for this section: Page 17 of 32

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28 Conclusion The pulmonary primary vasculitides are rare disorders, and their diagnoses are often difficult and delayed because their signs and symptoms are nonspecific and overlap with those of infections, connective tissue diseases, and malignancies. The knowledge of typical clinical, radiologic and laboratory findings is the clue for an earlier diagnosis. Page 28 of 32

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30 Personal Information Beatrice Feragalli, Cesare Mantini, Roberta Polverosi, Rosa Lucia Patea, Serena Ganino, Antonio Raffaele Cotroneo Istituto di Radiologia, Università "g. d'annunzio", Chieti References 1.Heeringa P, Schreiber A, Falk RJ, Jennette JC: Pathogenesis of pulmonary vasculitis. Semin Respir Crit Care Med 2004;25(5): Marten K, Schnyder P, Schirg E, Prokop M, Rummeny EJ, Engelke C: Pattern-based differential diagnosis in pulmonary vasculitis using volumetric CT. AJR Am J Roentgenol 2005;184: Frankel SK, Cosgrove GP, Fischer A, Meehan RT, Brown KK: Update in the diagnosis and management of pulmonary vasculitis. Chest 2006;129: Hiller N, Lieberman S, Chajek-Shaul T, Bar-Ziv J, Shaham D: Thoracic manifestations of Behçet disease at CT. RadioGraphics 2004;24: Chae EJ, Do KH, Seo JB, et al.: Radiologic and clinical findings of Behçet disease: comprehensive review of multisystemic involvement. RadioGraphics 2008;28(5): Frankel SK, Sullivan EJ, Brown KK: Vasculitis: Wegener granulomatosis, Churg-Strauss syndrome, microscopic polyangiitis, Page 30 of 32

31 polyarteritis nodosa, and Takayasu sarteritis. Crit Care Clin 2002;18(4): Press, 2006; Lee KS, Kim TS, Fujimoto K, et al.thoracic manifestation of Wegener's granulomatosis: CT findings in 30 patients. Eur Radiol 2003;13(1): Lohrmann C, Uhl M, Kotter E, Burger D, Ghanem N, Langer M: Pulmonary manifestations of Wegener granulomatosis: CT findings in 57 patients and a review of the literature. Eur J Radiol 2005;53(3): Ananthakrishnan L, Sharma N, Kanne JP: Wegener's granulomatosis in the chest: high-resolution CT findings. AJR Am J Roentgenol 2009;192(3): Kim YK, Lee KS, Chung MP, et al: Pulmonary involvement in Churg-Strauss syndrome: an analysis of CT, clinical, and pathologic findings. Eur Radiol 2007;17: Silva CI, Müller NL, Fujimoto K, Johkoh T, Ajzen SA: Churg AChurgStrauss syndrome: high resolution CT and pathologic findings. J Thorac Imaging 2005;20(2): Ando Y, Okada F, Matsumoto S, Mori HThoracic manifestation of myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA)related disease: CT findings in 51 patients. J Comput Assist Tomogr 2004;28(5): Ioachimescu OC. Alveolar hemorrhage. In: Laurent GL, Shapiro SD, eds. Encyclopedia of respiratory medicine. Amsterdam, the Netherlands: Academic Press, 2006; Page 31 of 32

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