Respiratory symptoms including cough, exertional dyspnea, and chest pain have been described in one-half of the patients with IgG4-related lung disease, while the remaining patients were asymptomatic [41]. Constitutional symptoms such as fever and weight loss can occur. Symptoms related to the extrapulmonary organ involvement (pancreas) may be present. Elevated serum IgG4 level is found in 70Ð90 % of the patients.

The most common symptoms include fever, persistent productive cough, dyspnea, and chest tightness [43]. The presentation is typically insidious, and lung lesions may wax and wane. Constitutional symptoms such as weight loss, malaise, and fatigue may be present. Symptoms related to the other organ involvement of the skin, central nervous system, and kidney can occur.

IgG4-related lung disease can be categorized into four types on the basis of the predominant CT Þndings: a solitary large nodular lesion including a mass, round-shaped ground-glass opacity, the thickening of bronchovascular bundles and interlobular septa, and bronchiectasis [28] (Fig. 3.10).

CT–Pathology Comparisons

Large nodules on CT histopathologically consist of diffuse lymphoplasmacytic inÞltration with Þbrosis [28]. Sclerosing inßammation extends along the interlobular septa and alveolar walls, and these Þndings correspond to the CT Þndings of spiculation around a nodule. Small nodules histologically correspond to the areas of sclerosing inßammation in the peribronchiolar area. Thickened bronchovascular bundles or interlobular septa at CT histologically correspond to lymphoplasmacytic inÞltration with stromal Þbrosis. Bronchi or bronchioles involved in the lesions are slightly dilated.

Patient Prognosis

In general, IgG4-related lung disease responds well to corticosteroid therapy. The optimal dose and duration of corticosteroid therapy remains to be determined. The relapse is not uncommon when corticosteroid administration is discontinued.

Characteristic CT Þndings are pulmonary nodules and masses with central low attenuation and peripheral rim enhancement and ground-glass opacity halo. The nodules and masses are lower lobe predominant with a peribronchovascular or subpleural distribution [29] (Fig. 3.11). Coarse irregular opacities and small thin-walled cysts are also seen [44].

CT–Pathology Comparisons

Pulmonary nodules and masses are histologically caused by intravascular and perivascular inÞltration of atypical lymphoid cells [44]. A low attenuation center corresponds to the histologic Þndings of central necrosis, and peripheral rim enhancement of the nodules is related to the angioinvasive and angio-destructive nature of lymphomatoid granulomatosis [29].

Patient Prognosis

Lymphomatoid granulomatosis is usually an EBV-driven lymphoproliferative disease in immunocompromised patients; thus, immunosuppressive agents should be discontinued if at all. SpeciÞc therapy with corticosteroids, anti-CD20 monoclonal antibodies such as rituximab, interferon-alpha-2b, and combination chemotherapy have showed a variable success rate [43].

Lymphomatoid Granulomatosis

Pathology and Pathogenesis

Lymphomatoid granulomatosis is an extranodal angiocentric and angio-destructive lymphoproliferative disorder, composed of a polymorphous inÞltrate of atypical B cells (Fig. 3.11), which are infected by EpsteinÐBarr virus (EBV), and more abundant reactive T cells. They show a spectrum of grade, which is related to the proportion of EBV-positive B cells. They may progress to an EBV-positive diffuse large B-cell lymphoma [42].

References

1. Leung AN, Miller RR, Muller NL. Parenchymal opaciÞcation in chronic inÞltrative lung diseases: CT-pathologic correlation. Radiology. 1993;188:209Ð14.

2. Hansell DM, Bankier AA, MacMahon H, McLoud TC, Muller NL, Remy J. Fleischner Society: glossary of terms for thoracic imaging. Radiology. 2008;246:697Ð722.

3. Jeong YJ, Lee KS. Pulmonary tuberculosis: up-to-date imaging and management. AJR Am J Roentgenol. 2008;191:834Ð44.

4. Jung JI, Kim H, Park SH, et al. CT differentiation of pneumonictype bronchioloalveolar cell carcinoma and infectious pneumonia. Br J Radiol. 2001;74:490Ð4.

5. Bae YA, Lee KS, Han J, et al. Marginal zone B-cell lymphoma of bronchus-associated lymphoid tissue: imaging Þndings in 21 patients. Chest. 2008;133:433Ð40.

6. Gaeta M, Minutoli F, Ascenti G, et al. MR white lung sign: incidence and signiÞcance in pulmonary consolidations. J Comput Assist Tomogr. 2001;25:890Ð6.

7. Tanaka N, Matsumoto T, Kuramitsu T, et al. High resolution CT Þndings in community-acquired pneumonia. J Comput Assist Tomogr. 1996;20:600Ð8.

8. Gaeta M, Ascenti G, Mazziotti S, Contiguglia R, Barone M, Mileto A. MRI differentiation of pneumonia-like mucinous adenocarcinoma and infectious pneumonia. Eur J Radiol. 2012;81: 3587Ð91.

9. Revel MP, Triki R, Chatellier G, et al. Is it possible to recognize pulmonary infarction on multisection CT images? Radiology. 2007;244:875Ð82.

10.Kim SJ, Lee KS, Ryu YH, et al. Reversed halo sign on highresolution CT of cryptogenic organizing pneumonia: diagnostic implications. AJR Am J Roentgenol. 2003;180:1251Ð4.

11.Husain AN. Chapter 15. The lung. In: Maitra A, Kumar V, editors. Robbins and Cotran pathologic basis of disease. International edition, 8th ed. Philadelphia: Elsevier/Saunders; 2010. p. 706.

12.Chan SS, Yuen EH, Kew J, Cheung WL, Cocks RA. Communityacquired pneumoniaÐimplementation of a prediction rule to guide selection of patients for outpatient treatment. Eur J Emerg Med. 2001;8:279Ð86.

13.British Thoracic Society Standards of Care Committee. BTS guidelines for the management of community acquired pneumonia in adults. Thorax. 2001;56 Suppl 4:IV1Ð64.

14.Travis WD, Brambilla E, Noguchi M, et al. International Association for the Study of Lung Cancer/American Thoracic Society/European Respiratory Society International Multidisciplinary ClassiÞcation of Lung Adenocarcinoma. J Thorac Oncol. 2011;6:244Ð85.

15.Akira M, Atagi S, Kawahara M, Iuchi K, Johkoh T. High-resolution CT Þndings of diffuse bronchioloalveolar carcinoma in 38 patients. AJR Am J Roentgenol. 1999;173:1623Ð9.

16.Austin JH, Garg K, Aberle D, et al. Radiologic implications of the 2011 classiÞcation of adenocarcinoma of the lung. Radiology. 2013;266:62Ð71.

17.Shah RM, Balsara G, Webster M, Friedman AC. Bronchioloalveolar

cell carcinoma: impact of histology on dominant CT pattern. J Thorac Imaging. 2000;15:180Ð6.

18.Lee KS, Kim Y, Han J, Ko EJ, Park CK, Primack SL. Bronchioloalveolar carcinoma: clinical, histopathologic, and radiologic Þndings. Radiographics. 1997;17:1345Ð57.

19.Erman M, Grunenwald D, Penault-Llorca F, et al. Epidermal growth factor receptor, HER-2/neu and related pathways in lung adenocarcinomas with bronchioloalveolar features. Lung Cancer. 2005;47:315Ð23.

20.Wislez M, Cadranel J, Antoine M, et al. Lymphoma of pulmonary mucosa-associated lymphoid tissue: CT scan Þndings and pathological correlations. Eur Respir J. 1999;14:423Ð9.

21.Borie R, Wislez M, Thabut G, et al. Clinical characteristics and prognostic factors of pulmonary MALT lymphoma. Eur Respir J. 2009;34:1408Ð16.

22.Chintapalli K, Thorsen MK, Olson DL, Goodman LR, Gurney J. Computed tomography of pulmonary thromboembolism and infarction. J Comput Assist Tomogr. 1988;12:553Ð9.

23.Soussan M, Rust E, Pop G, Morere JF, Brillet PY, Eder V. The rim sign: FDG-PET/CT pattern of pulmonary infarction. Insights Imaging. 2012;3:629Ð33.

24.Balakrishnan J, Meziane MA, Siegelman SS, Fishman EK. Pulmonary infarction: CT appearance with pathologic correlation. J Comput Assist Tomogr. 1989;13:941Ð5.

25.Lee KS, Kullnig P, Hartman TE, Muller NL. Cryptogenic organizing pneumonia: CT Þndings in 43 patients. AJR Am J Roentgenol. 1994;162:543Ð6.

26.Logan PM, Primack SL, Miller RR, Muller NL. Invasive aspergillosis of the airways: radiographic, CT, and pathologic Þndings. Radiology. 1994;193:383Ð8.

27.Song KD, Lee KS, Chung MP, et al. Pulmonary cryptococcosis: imaging Þndings in 23 non-AIDS patients. Korean J Radiol. 2010;11:407Ð16.

28.Inoue D, Zen Y, Abo H, et al. Immunoglobulin G4-related lung disease: CT Þndings with pathologic correlations. Radiology. 2009;251:260Ð70.

29.Chung JH, Wu CC, Gilman MD, Palmer EL, Hasserjian RP, Shepard JA. Lymphomatoid granulomatosis: CT and FDG-PET Þndings. Korean J Radiol. 2011;12:671Ð8.

30.Lee KS, Kim TS, Fujimoto K, et al. Thoracic manifestation of WegenerÕs granulomatosis: CT Þndings in 30 patients. Eur Radiol. 2003;13:43Ð51.

31.Chung MP, Yi CA, Lee HY, Han J, Lee KS. Imaging of pulmonary vasculitis. Radiology. 2010;255:322Ð41.

32.Krenke R, Grabczak EM. Tracheobronchial manifestations of Aspergillus infections. Sci World J. 2011;11:2310Ð29.

33.Kim NR, Ha SY, Chung DH, et al. Isolated pulmonary cryptococcosis: report of six cases and review of the Korean cases. Korean J Pathol. 2003;37:193Ð8.

34.Brizendine KD, Baddley JW, Pappas PG. Pulmonary cryptococcosis. Semin Respir Crit Care Med. 2011;32:727Ð34.

35.Lee KH, Chang UI, Kim HW, et al. Acute respiratory failure associated with cryptococcal pneumonia and disseminated cryptococcosis in an AIDS patient. Korean J Intern Med. 2006;

21:39Ð42.

36. Patz Jr EF, Goodman PC. Pulmonary cryptococcosis. J Thorac Imaging. 1992;7:51Ð5.

37.Zinck SE, Leung AN, Frost M, Berry GJ, Muller NL. Pulmonary cryptococcosis: CT and pathologic Þndings. J Comput Assist Tomogr. 2002;26:330Ð4.

38.Limper AH, Knox KS, Sarosi GA, et al. An ofÞcial American Thoracic Society statement: treatment of fungal infections in adult pulmonary and critical care patients. Am J Respir Crit Care Med. 2011;183:96Ð128.

39.Zen Y, Inoue D, Kitao A, et al. IgG4-related lung and pleural disease: a clinicopathologic study of 21 cases. Am J Surg Pathol. 2009;33:1886Ð93.

40.Park HY, Han J, Kang G, Yi CA, Chung MP. IgG4-related lung disease presenting as a consolidative mass: a case report. J Lung Cancer. 2010;9:103Ð5.

41.Ryu JH, Sekiguchi H, Yi ES. Pulmonary manifestations of immunoglobulin G4-related sclerosing disease. Eur Respir J. 2012;39: 180Ð6.

42.Guinee Jr DG, Perkins SL, Travis WD, Holden JA, Tripp SR, Koss MN. Proliferation and cellular phenotype in lymphomatoid granulomatosis: implications of a higher proliferation index in B cells. Am J Surg Pathol. 1998;22:1093Ð100.

43.Roschewski M, Wilson WH. Lymphomatoid granulomatosis. Cancer J. 2012;18:469Ð74.

44.Lee JS, Tuder R, Lynch DA. Lymphomatoid granulomatosis: radiologic features and pathologic correlations. AJR Am J Roentgenol. 2000;175:1335Ð9.