The Journal of Rheumatology
Volume 42, no. 1
Pulmonary Nodular Amyloidosis in Sjögren Syndrome ROBERTO CARBONE, CLAUDIO COSSO and MARCO A. CIMMINO J Rheumatol 2015;42;134 http://www.jrheum.org/content/42/1/134 1. Sign up for our monthly e-table of contents http://www.jrheum.org/cgi/alerts/etoc 2. Information on Subscriptions http://jrheum.com/subscribe.html 3. Have us contact your library about access options [email protected] 4. Information on permissions/orders of reprints http://jrheum.com/reprints.html The Journal of Rheumatology is a monthly international serial edited by Earl D. Silverman featuring research articles on clinical subjects from scientists working in rheumatology and related fields.
Downloaded from www.jrheum.org on January 16, 2015 - Published by The Journal of Rheumatology
Images in Rheumatology
Pulmonary Nodular Amyloidosis in Sjögren Syndrome ROBERTO CARBONE, MD, Respiratory Unit, Department of Internal Medicine, Regional Hospital, Aosta; CLAUDIO COSSO, MD; MARCO A. CIMMINO, MD, Associate Professor, Research Laboratory and Academic Unit of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, Genoa, Italy. Address correspondence to Dr. M.A. Cimmino, Viale Benedetto XV, 6 – 16143, Genoa, Italy. E-mail: [email protected]. J Rheumatol 2015;42:134; doi:10.3899/jrheum.140964
Pulmonary involvement because of amyloidosis is rare in Sjögren syndrome (SS). It can be treated with corticosteroids. A 40-year-old woman presenting with dry cough, dyspnea score Moser 1, chest pain, and fatigue was investigated for suspected interstitial pulmonary disease. Fifteen years earlier, she had been diagnosed with SS on the basis of sicca syndrome, positive salivary glands biopsy, antinuclear antibody > 1:640 (granular pattern), and high titer anti-La and anti-Ro antibodies. Physiological lung function showed an initial restrictive pattern with normal carbon monoxide transfer. High-resolution computed tomography (HRCT) identified multiple pulmonary nodules (Figure 1A), which showed intense uptake on a positron emission tomography/CT scan (Figure 1B). QuantiFERON (Quest Diagnostics) test was negative. To exclude possible lung cancer, a biopsy of one of the nodules was performed. Histopathological evaluation showed peribronchial and perivascular lymphoid nodular hyperplasia, with perivascular and interstitial amyloid deposits seen after use of dye (Figure 1C, arrows). The patient was prescribed prednisone at an initial dose of 25 mg daily for 1 month, which was tapered over 6 months to 5 mg daily with a complete resolution of the symptoms. Pulmonary involvement because of amyloidosis is rare in SS, occurring in only 2/343 of the patients (0.6%) studied by Strimlan, et al1. A biopsy of nodular amyloidosis of the lung has revealed amyloid light-chain type2 or transthyretin amyloidosis3. REFERENCES
1. Strimlan CV, Rosenow EC 3rd, Divertie MB, Harrison EG Jr. Pulmonary manifestations of Sjögren’s syndrome. Chest 1976;70:354-61. 2. Miyagawa T, Mochizuki Y, Nakahara Y, Kawamura T, Sasaki S, Kobashi Y. [A case of Sjögren syndrome with pulmonary nodular amyloidosis and pulmonary multiple cysts]. [Article in Japanese] Nihon Kokyuki Gakkai Zasshi 2009;47:737-41. 3. Sakai T, Tsushima T, Kimura D, Fukuda I, Kamata Y, Hatanaka R, et al. [Multiple nodular pulmonary amyloidosis complicated with Sjögren syndrome]. [Article in Japanese] Kyobu Geka 2010;63:818-21.
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Figure 1. A. High-resolution computed tomography identified multiple pulmonary nodules. B. Nodules showed intense uptake on a positron emission tomography/computed tomography scan. C. Histopathological evaluation showed peribronchial and perivascular lymphoid nodular hyperplasia, with perivascular and interstitial amyloid deposits seen after use of dye (arrows).
Personal non-commercial use only. The Journal of Rheumatology Copyright © 2015. All rights reserved.
The Journal of Rheumatology 2015; 42:1; doi:10.3899/jrheum.140964
Downloaded from www.jrheum.org on January 16, 2015 - Published by The Journal of Rheumatology
Volume 42, no. 1
Pulmonary Nodular Amyloidosis in Sjögren Syndrome ROBERTO CARBONE, CLAUDIO COSSO and MARCO A. CIMMINO J Rheumatol 2015;42;134 http://www.jrheum.org/content/42/1/134 1. Sign up for our monthly e-table of contents http://www.jrheum.org/cgi/alerts/etoc 2. Information on Subscriptions http://jrheum.com/subscribe.html 3. Have us contact your library about access options [email protected] 4. Information on permissions/orders of reprints http://jrheum.com/reprints.html The Journal of Rheumatology is a monthly international serial edited by Earl D. Silverman featuring research articles on clinical subjects from scientists working in rheumatology and related fields.
Downloaded from www.jrheum.org on January 16, 2015 - Published by The Journal of Rheumatology
Images in Rheumatology
Pulmonary Nodular Amyloidosis in Sjögren Syndrome ROBERTO CARBONE, MD, Respiratory Unit, Department of Internal Medicine, Regional Hospital, Aosta; CLAUDIO COSSO, MD; MARCO A. CIMMINO, MD, Associate Professor, Research Laboratory and Academic Unit of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, Genoa, Italy. Address correspondence to Dr. M.A. Cimmino, Viale Benedetto XV, 6 – 16143, Genoa, Italy. E-mail: [email protected]. J Rheumatol 2015;42:134; doi:10.3899/jrheum.140964
Pulmonary involvement because of amyloidosis is rare in Sjögren syndrome (SS). It can be treated with corticosteroids. A 40-year-old woman presenting with dry cough, dyspnea score Moser 1, chest pain, and fatigue was investigated for suspected interstitial pulmonary disease. Fifteen years earlier, she had been diagnosed with SS on the basis of sicca syndrome, positive salivary glands biopsy, antinuclear antibody > 1:640 (granular pattern), and high titer anti-La and anti-Ro antibodies. Physiological lung function showed an initial restrictive pattern with normal carbon monoxide transfer. High-resolution computed tomography (HRCT) identified multiple pulmonary nodules (Figure 1A), which showed intense uptake on a positron emission tomography/CT scan (Figure 1B). QuantiFERON (Quest Diagnostics) test was negative. To exclude possible lung cancer, a biopsy of one of the nodules was performed. Histopathological evaluation showed peribronchial and perivascular lymphoid nodular hyperplasia, with perivascular and interstitial amyloid deposits seen after use of dye (Figure 1C, arrows). The patient was prescribed prednisone at an initial dose of 25 mg daily for 1 month, which was tapered over 6 months to 5 mg daily with a complete resolution of the symptoms. Pulmonary involvement because of amyloidosis is rare in SS, occurring in only 2/343 of the patients (0.6%) studied by Strimlan, et al1. A biopsy of nodular amyloidosis of the lung has revealed amyloid light-chain type2 or transthyretin amyloidosis3. REFERENCES
1. Strimlan CV, Rosenow EC 3rd, Divertie MB, Harrison EG Jr. Pulmonary manifestations of Sjögren’s syndrome. Chest 1976;70:354-61. 2. Miyagawa T, Mochizuki Y, Nakahara Y, Kawamura T, Sasaki S, Kobashi Y. [A case of Sjögren syndrome with pulmonary nodular amyloidosis and pulmonary multiple cysts]. [Article in Japanese] Nihon Kokyuki Gakkai Zasshi 2009;47:737-41. 3. Sakai T, Tsushima T, Kimura D, Fukuda I, Kamata Y, Hatanaka R, et al. [Multiple nodular pulmonary amyloidosis complicated with Sjögren syndrome]. [Article in Japanese] Kyobu Geka 2010;63:818-21.
134
Figure 1. A. High-resolution computed tomography identified multiple pulmonary nodules. B. Nodules showed intense uptake on a positron emission tomography/computed tomography scan. C. Histopathological evaluation showed peribronchial and perivascular lymphoid nodular hyperplasia, with perivascular and interstitial amyloid deposits seen after use of dye (arrows).
Personal non-commercial use only. The Journal of Rheumatology Copyright © 2015. All rights reserved.
The Journal of Rheumatology 2015; 42:1; doi:10.3899/jrheum.140964
Downloaded from www.jrheum.org on January 16, 2015 - Published by The Journal of Rheumatology
