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Primary Amyloidosis Presenting as Sjögren's Syndrome

Primary Amyloidosis Presenting as Sjögren's Syndrome Abstract • A patient had severe sicca complex. Histopathologic and immunologic examination of the salivary glands showed amyloid fibril infiltration, type AλVI. To our knowledge, this is the first documentation of immunologically characterized primary amyloidosis causing the sicca complex. The sicca complex is usually associated with Sjögren's syndrome and the presence of autoantibodies to SSA and SSB. These antibodies were absent in our patient, despite the severity of the sicca syndrome. The clinician should consider more unusual causes of the sicca complex, eg, amyloidosis, particularly if the serologic markers of antibodies to SSA and SSB are absent. Tissue typing the amyloid protein diagnosed primary amyloidosis, obviating the need to search for underlying disease. (Arch Intern Med 1983;143:2325-2326) References 1. Bloch KJ, Buchanan WW, Wohl MJ, et al: Sjögren syndrome: A clinical, pathological and serological study of 62 cases. Medicine 1965;44:187-231.Crossref 2. Martine-Lavin M, Vaughan JH, Tan EM: Autoantibodies and the spectrum of Sjögren's syndrome. Ann Intern Med 1979;91:185-190.Crossref 3. Cornwell GG III, Husby G, Westermark P, et al: Identification and characterization of different amyloid fibril proteins in tissue sections. Scand J Immunol 1977;6:1071-1080.Crossref 4. Cornwell GG III, Westermark JB, Murdock N, et al: Senile cardiac amyloid: Evidence that fibrils contain a protein immunologically related to prealbumin. Immunology 1981;44:447-452. 5. Talal N: Sjögren's syndrome and connective tissue disease with other immunologic disorders , in McCarty DJ (ed): Arthritis and Allied Conditions , ed 9. Philadelphia, Lea & Febiger, 1979, chap 54. 6. Golding PL, Brown R, Mason AMS, et al: `Sicca complex' in liver disease. Br Med J 1970;4:340-342.Crossref 7. Mason AMS, Golding PL: Hyperglobulinemic renal tubular acidosis: A report of nine cases. Br Med J 1970;3:143-146.Crossref 8. Simon BG, Moutsopoulos HM: Primary amyloidosis resembling sicca syndrome. Arthritis Rheum 1979;22:932-934.Crossref 9. Kuczynski A, Evans RJC, Mitchinson MJ: Sicca syndrome due to primary amyloidosis. Br Med J 1971;2:506.Crossref 10. Gardner DL: Pathology of Connective Tissue Diseases . London, Arnold Ltd, 1965, p 86. 11. Cataleno MA, Vaughan JH: Secondary amyloidosis and sicca syndrome. Arthritis Rheum 1979;22:1067. 12. Solomon A, Frangione B, Franklin EC: Bence Jones proteins and light chains—preferential association of the VλVI subgroup of human light chains with amyloidosis AL(λ). J Clin Invest 1982;70:453-460.Crossref 13. Glenner GG: Amyloid deposits and amyloidosis: The β-fibrilloses. N Engl J Med 1980;302:1283-1292, 1333-1343.Crossref 14. Kyle RA, Bayrd ED: Amyloidosis: Review of 236 cases. Medicine 1975;54:271-299.Crossref 15. Benson MD, Brandt KD, Cohen AS, et al: Neuropathy, M components, and amyloid. Lancet 1975;1:10-12.Crossref 16. Trotter JL, Engel WK, Ignaczak TF: Amyloidosis with plasma cell dyscrasia: An overlooked cause of adult onset sensorimotor neuropathy. Arch Neurol 1977;34:209-214.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Internal Medicine American Medical Association

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Publisher
American Medical Association
Copyright
Copyright © 1983 American Medical Association. All Rights Reserved.
ISSN
0003-9926
eISSN
1538-3679
DOI
10.1001/archinte.1983.00350120123027
Publisher site
See Article on Publisher Site

Abstract

Abstract • A patient had severe sicca complex. Histopathologic and immunologic examination of the salivary glands showed amyloid fibril infiltration, type AλVI. To our knowledge, this is the first documentation of immunologically characterized primary amyloidosis causing the sicca complex. The sicca complex is usually associated with Sjögren's syndrome and the presence of autoantibodies to SSA and SSB. These antibodies were absent in our patient, despite the severity of the sicca syndrome. The clinician should consider more unusual causes of the sicca complex, eg, amyloidosis, particularly if the serologic markers of antibodies to SSA and SSB are absent. Tissue typing the amyloid protein diagnosed primary amyloidosis, obviating the need to search for underlying disease. (Arch Intern Med 1983;143:2325-2326) References 1. Bloch KJ, Buchanan WW, Wohl MJ, et al: Sjögren syndrome: A clinical, pathological and serological study of 62 cases. Medicine 1965;44:187-231.Crossref 2. Martine-Lavin M, Vaughan JH, Tan EM: Autoantibodies and the spectrum of Sjögren's syndrome. Ann Intern Med 1979;91:185-190.Crossref 3. Cornwell GG III, Husby G, Westermark P, et al: Identification and characterization of different amyloid fibril proteins in tissue sections. Scand J Immunol 1977;6:1071-1080.Crossref 4. Cornwell GG III, Westermark JB, Murdock N, et al: Senile cardiac amyloid: Evidence that fibrils contain a protein immunologically related to prealbumin. Immunology 1981;44:447-452. 5. Talal N: Sjögren's syndrome and connective tissue disease with other immunologic disorders , in McCarty DJ (ed): Arthritis and Allied Conditions , ed 9. Philadelphia, Lea & Febiger, 1979, chap 54. 6. Golding PL, Brown R, Mason AMS, et al: `Sicca complex' in liver disease. Br Med J 1970;4:340-342.Crossref 7. Mason AMS, Golding PL: Hyperglobulinemic renal tubular acidosis: A report of nine cases. Br Med J 1970;3:143-146.Crossref 8. Simon BG, Moutsopoulos HM: Primary amyloidosis resembling sicca syndrome. Arthritis Rheum 1979;22:932-934.Crossref 9. Kuczynski A, Evans RJC, Mitchinson MJ: Sicca syndrome due to primary amyloidosis. Br Med J 1971;2:506.Crossref 10. Gardner DL: Pathology of Connective Tissue Diseases . London, Arnold Ltd, 1965, p 86. 11. Cataleno MA, Vaughan JH: Secondary amyloidosis and sicca syndrome. Arthritis Rheum 1979;22:1067. 12. Solomon A, Frangione B, Franklin EC: Bence Jones proteins and light chains—preferential association of the VλVI subgroup of human light chains with amyloidosis AL(λ). J Clin Invest 1982;70:453-460.Crossref 13. Glenner GG: Amyloid deposits and amyloidosis: The β-fibrilloses. N Engl J Med 1980;302:1283-1292, 1333-1343.Crossref 14. Kyle RA, Bayrd ED: Amyloidosis: Review of 236 cases. Medicine 1975;54:271-299.Crossref 15. Benson MD, Brandt KD, Cohen AS, et al: Neuropathy, M components, and amyloid. Lancet 1975;1:10-12.Crossref 16. Trotter JL, Engel WK, Ignaczak TF: Amyloidosis with plasma cell dyscrasia: An overlooked cause of adult onset sensorimotor neuropathy. Arch Neurol 1977;34:209-214.Crossref

Journal

Archives of Internal MedicineAmerican Medical Association

Published: Dec 1, 1983

References