SESSION TITLE: ILD Case Report Posters I
SESSION TYPE: Case Report Poster
PRESENTED ON: Sunday, March 23, 2014 at 01:15 PM - 02:15 PM
INTRODUCTION: Amyloidosis is the abnormal formation of proteins, amyloid fibrins. It is characterised by extracellular deposition in various organs. Pulmonary amyloidosis is rare and can present in the forms of laryngeal, tracheobronchial, pulmonary and mediastinal lymph node disease (1). We present a case of nodular pulmonary amyloidosis in a patient with MCTD.
CASE PRESENTATION: Ms X is a 47 year-old Chinese female with history of MCTD (systemic lupus erythematosus, Sjogren's syndrome and scleroderma) and ocular myasthenia gravis. CT thorax in 2005 as part of the work-up for myasthenia gravis, revealed multiple cavities and nodules with parenchymal fibrosis and calcification. Patient declined further investigation. She presented to our institution again in October 2012 for dyspnea and signs and symptoms suggestive of an active MCTD (Raynaud’s phenomenon, sicca symptoms and leucopenia). A repeat CT thorax showed an increase in size and number of the multiple pulmonary nodules and multiple thin walled cysts and areas of parenchymal scarring and calcification (See Figure 1 and Figure 2). Histology from CT guided biopsy revealed cores of eosinophilic amorphous appearing tissue with scattered histiocytes and occasional foci of chondroid metaplasia. Congo Red stain is positive with apple green birefringence using polarized light consistent with pulmonary amyloidosis. Immunostain for AA amyloid was negative. Patient was started on plaquenil for the manifestations of MCTD.
DISCUSSION: Amyloidosis is associated with an abnormal deposition of proteins in an abnormal beta-sheet fibrillar form. The two common forms are AL and AA. The latter is associated with chronic inflammation. It is rare to see the diffuse nodular radiological pattern in patients with connective tissue disease (2). The top differentials for pulmonary nodules in patients with connective tissue diseases remain malignancy, infections and amyloidosis. Histological confirmation is the key to diagnosis. 3 main forms of pulmonary amyloidosis are tracheobronchial amyloidosis, diffuse interstitial amyloidosis and nodular pulmonary amyloidosis (3). Prognosis is the best in the nodular form.
CONCLUSIONS: Pulmonary amyloidosis is a rare complication of MCTD. Though radiological features can be distinguishing, tissue diagnosis is still the gold standard.
Reference #1: Rajagola S, Singh N, Gupta K et al. Pulmonary amyloidosis in Sjogren's Syndrome: A case report and a systemic review of the literature. Respirology (2010) 15, 860-868
Reference #2: Ito I, Nagai S, Kitaichi M et al. Pulmonary manifestations of primary Sjogren’s syndrome: a clinical, radiologic, and pathologic study. Am. J. Respir. Crit. Care Med. 2005; 171: 632-8.
Reference #3: Aylwin AC, Gishen P, Copley SJ. Imaging appearance of thoracic amyloidosis. J Thorac Imaging 2005;20(1):41-46
DISCLOSURE: The following authors have nothing to disclose: Chuen Peng Lee, Ser Hon Puah, Wenyuan Sim, Mark Tien, Jessica Pau, Grace Tan, Ruth Chang
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