Sjögren's syndrome and localized nodular cutaneous amyloidosis

Coincidence or a distinct clinical entity?

Jiska M. Meijer, Stefan O. Schonland, Giovanni Palladini, Giampaolo Merlini, Ute Hegenbart, Olga Ciocca, Vittorio Perfetti, Martha K. Leijsma, Hendrika Bootsma, Bouke P C Hazenberg

Research output: Contribution to journalArticle

42 Citations (Scopus)

Abstract

Objective. To report 8 patients with Sjögren's syndrome (SS) and localized nodular cutaneous amyloidosis and to examine serologic and immunohistologic findings that may link the 2 diseases. Methods. The databases for 3 amyloidosis centers were searched for patients with localized nodular cutaneous amyloidosis and SS. Eight patients with this combination were identified, and clinical, serologic, and histologic parameters were retrospectively evaluated. Results. Among the 8 patients with a clinical diagnosis of SS, 6 fulfilled the American-European Consensus Group criteria for SS. All of the patients were women in whom SS had been diagnosed at a median age of 47 years (range 30-61 years) and amyloid had been diagnosed at a median age of 60 years (range 42-79 years). The presence of the immunoglobulin light chain type of amyloid (AL amyloid) was confirmed in 4 patients. In 3 of these 4 patients as well as 2 other patients, a light chain-restricted plasma cell population was observed near the amyloid deposits. Progression to systemic amyloidosis was not observed in any patient during a median followup of 3.5 years. Conclusion. SS should be considered in patients with cutaneous amyloidosis. The combination of cutaneous amyloidosis and SS appears to be a distinct disease entity reflecting a particular and benign part of the polymorphic spectrum of lymphoproliferative diseases related to SS.

Original languageEnglish
Pages (from-to)1992-1999
Number of pages8
JournalArthritis and Rheumatism
Volume58
Issue number7
DOIs
Publication statusPublished - Jul 2008

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Amyloidosis
Skin
Amyloid
Immunoglobulin Light Chains
Amyloid Plaques
Plasma Cells
Databases
Light

ASJC Scopus subject areas

  • Immunology
  • Rheumatology

Cite this

Sjögren's syndrome and localized nodular cutaneous amyloidosis : Coincidence or a distinct clinical entity? / Meijer, Jiska M.; Schonland, Stefan O.; Palladini, Giovanni; Merlini, Giampaolo; Hegenbart, Ute; Ciocca, Olga; Perfetti, Vittorio; Leijsma, Martha K.; Bootsma, Hendrika; Hazenberg, Bouke P C.

In: Arthritis and Rheumatism, Vol. 58, No. 7, 07.2008, p. 1992-1999.

Research output: Contribution to journalArticle

Meijer, JM, Schonland, SO, Palladini, G, Merlini, G, Hegenbart, U, Ciocca, O, Perfetti, V, Leijsma, MK, Bootsma, H & Hazenberg, BPC 2008, 'Sjögren's syndrome and localized nodular cutaneous amyloidosis: Coincidence or a distinct clinical entity?', Arthritis and Rheumatism, vol. 58, no. 7, pp. 1992-1999. https://doi.org/10.1002/art.23617
Meijer, Jiska M. ; Schonland, Stefan O. ; Palladini, Giovanni ; Merlini, Giampaolo ; Hegenbart, Ute ; Ciocca, Olga ; Perfetti, Vittorio ; Leijsma, Martha K. ; Bootsma, Hendrika ; Hazenberg, Bouke P C. / Sjögren's syndrome and localized nodular cutaneous amyloidosis : Coincidence or a distinct clinical entity?. In: Arthritis and Rheumatism. 2008 ; Vol. 58, No. 7. pp. 1992-1999.
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abstract = "Objective. To report 8 patients with Sj{\"o}gren's syndrome (SS) and localized nodular cutaneous amyloidosis and to examine serologic and immunohistologic findings that may link the 2 diseases. Methods. The databases for 3 amyloidosis centers were searched for patients with localized nodular cutaneous amyloidosis and SS. Eight patients with this combination were identified, and clinical, serologic, and histologic parameters were retrospectively evaluated. Results. Among the 8 patients with a clinical diagnosis of SS, 6 fulfilled the American-European Consensus Group criteria for SS. All of the patients were women in whom SS had been diagnosed at a median age of 47 years (range 30-61 years) and amyloid had been diagnosed at a median age of 60 years (range 42-79 years). The presence of the immunoglobulin light chain type of amyloid (AL amyloid) was confirmed in 4 patients. In 3 of these 4 patients as well as 2 other patients, a light chain-restricted plasma cell population was observed near the amyloid deposits. Progression to systemic amyloidosis was not observed in any patient during a median followup of 3.5 years. Conclusion. SS should be considered in patients with cutaneous amyloidosis. The combination of cutaneous amyloidosis and SS appears to be a distinct disease entity reflecting a particular and benign part of the polymorphic spectrum of lymphoproliferative diseases related to SS.",
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