Amyloidosis is a heterogeneous group of disorder associated with the deposition of protein in an abnormal fibrillar form. Primary Sj？gren’s syndrome (PSS) is a systemic inflammatory disorder that commonly affects the exocrine glands. The reported frequency of pulmonary involvement in PSS varies widely, ranging from 9% to 75%. Pulmonary involvement occurs in light-chain (AL) amyloidosis and is uncommon in the reactive (AA) and hereditary forms. Herein we present a case of PSS associated diffuse multinodular amyloidosis in the lung. We followed up the patient without treatment for three years. There are only minimal lung symptoms related to lung infiltration. In conclusion, pulmonary involvement in SS is an extremely rare clinical manifestation and usually has a good survival rate without treatment. 1. Introduction Amyloidosis is a heterogeneous group of disorders associated with the deposition of protein in an abnormal fibrillar form. The classification of amyloidosis is based on the fibril type [1]. Aggregation of these pathological proteins forms amyloid deposits in various organs eventually leading to organ failure and death. Over 20 amyloidogenic precursor proteins have been documented to form amyloid deposits systemically or localise to specific organs [2]. All amyloid fibrils appear as faintly red on Congo red staining on microscopic examination and show a typical apple-green birefringence under polarised light. The site of deposition relies on the type of amyloidosis, which can be acquired or hereditary, localized, or systemic. At least 25 different precursors of amyloid fibrils are now known [3]. Pulmonary involvement occurs in light-chain (AL) amyloidosis and is uncommon in the reactive (AA) and hereditary forms. The spectrum of pulmonary amyloidosis includes laryngeal, tracheobronchial, parenchymal (localised and diffuse), and mediastinal lymph node disease. Primary Sj？gren’s syndrome (PSS) is a systemic inflammatory disorder that commonly affects the exocrine glands [4]. The reported frequency of pulmonary involvement in PSS varies widely, ranging from 9% to 75% depending on the detection method employed, and consists of various forms of small airway and interstitial lung diseases [5]. The clinical lung manifestations of PSS include pleuritis, interstitial pneumonia, and fibrosis. The common patterns of lung involvement are honeycomb formation, ground-glass attenuation, centrilobular nodules, reticular pattern, and bronchiectasis [6]. We presented a case with PSS associated diffuse multinodular amyloidosis in the lung. 2. Case A 61-year-old
References
[1]
S. Rajagopala, N. Singh, K. Gupta, and D. Gupta, “Pulmonary amyloidosis in Sjogren's syndrome: a case report and systematic review of the literature,” Respirology, vol. 15, no. 5, pp. 860–866, 2010.
[2]
J. D. Sipe, M. D. Benson, J. N. Buxbaum et al., “Amyloid fibril protein nomenclature: 2010 recommendations from the nomenclature committee of the International Society of Amyloidosis,” Amyloid, vol. 17, no. 3-4, pp. 101–104, 2010.
[3]
F. Perfetto, A. Moggi-Pignone, R. Livi, A. Tempestini, F. Bergesio, and M. Matucci-Cerinic, “Systemic amyloidosis: a challenge for the rheumatologist,” Nature Reviews Rheumatology, vol. 6, no. 7, pp. 417–429, 2010.
[4]
S. S. Kassan and H. M. Moutsopoulos, “Clinical manifestations and early diagnosis of Sj？gren syndrome,” Archives of Internal Medicine, vol. 164, no. 12, pp. 1275–1284, 2004.
[5]
R. I. Fox and P. Michelson, “Approaches to the treatment of Sj？gren’s syndrome,” The Journal of Rheumatology, vol. 61, pp. 15–21, 2000.
[6]
I. Ito, S. Nagai, M. Kitaichi et al., “Pulmonary manifestations of primary Sj？gren's syndrome: a clinical, radiologic, and pathologic study,” American Journal of Respiratory and Critical Care Medicine, vol. 171, no. 6, pp. 632–638, 2005.
[7]
B. K. S. Davidson, C. A. Kelly, and I. D. Griffiths, “Ten-year follow up of pulmonary function in patients with primary Sj？gren's syndrome,” Thorax, vol. 59, pp. 709–712, 2000.
[8]
C. V. Strimlan, “Pulmonary involvement in Sj？gren's syndrome,” Chest, vol. 89, no. 6, pp. 901–902, 1986.
[9]
T. Franquet, A. Giménez, J. M. Monill, C. Díaz, and C. Geli, “Primary Sj？gren's syndrome and associated lung disease: CT findings in 50 patients,” American Journal of Roentgenology, vol. 169, no. 3, pp. 655–658, 1997.
[10]
V. Yazisiz, G. Arslan, I. H. Ozbudak et al., “Lung involvement in patients with primary Sj？gren's syndrome: what are the predictors?” Rheumatology International, vol. 30, no. 10, pp. 1317–1324, 2010.
[11]
W. D. Travis, T. V. Colby, and M. N. Koss, Non-Neoplastic Disorders of the Lower Respiratory Tract, Armed Forces Institute of Patholgy, Washington, DC, USA, 2002.
[12]
J. D. Gillmore and P. N. Hawkins, “Amyloidosis and the respiratory tract,” Thorax, vol. 54, no. 5, pp. 444–451, 1999.
[13]
H. Y. Kim, J. G. Im, K. S. Song et al., “Localized amyloidosis of the respiratory system: CT features,” Journal of Computer Assisted Tomography, vol. 23, no. 4, pp. 627–631, 1999.
[14]
J. W. A. dos Santos, A. S. Filho, A. Bertolazzi et al., “Primary tracheobronchial amyloidosis,” Jornal Brasileiro de Pneumologia, vol. 34, no. 10, pp. 881–884, 2008.
[15]
H. A. Pickford, S. J. Swensen, and J. P. Utz, “Thoracic cross-sectional imaging of amyloidosis,” American Journal of Roentgenology, vol. 168, no. 2, pp. 351–355, 1997.
[16]
C. Turesson, L. Jacobsson, and U. Bergstr？m, “Extra-articular rheumatoid arthritis: prevalence and mortality,” Rheumatology, vol. 38, no. 7, pp. 668–674, 1999.
