Frequency of systemic manifestations in patients with primary Sjögren's syndrome in Argentina

Zazzetti, Federico; Adolfo Rivero, Mariano; Duartes Noé, Damián Elvio; Gallacher, Alberto; Schiel, Amalia; Claudia Khoury, Marina; Armando Laborde, Hugo; Carlos Barreira, Juan

doi:10.1016/S2173-5743(10)70070-8

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Abstract

Twenty to 71% of patients with Sjögren's syndrome (SS) will develop systemic manifestations.

Objetive

To characterize the clinical-serological presentation and the frequency of systemic manifestations in patients with primary SS.

Methods

Retrospective study including patients with SS visited in “Hospital Británico de Buenos Aires” during the period from January 2000 to August 2008.

Results

Forty-one patients fulfilled the 2002 American-European classification criteria for SS. All patients were women. Mean age at enrollment was 57,85±12,42 years (range 26–79). Mean duration of the disease was 9,28 years (range 0,08–24). Thirty-three (80.49%) developed systemic manifestations. The most frequent were arthritis, cutaneous vasculitis and polyneuropathy. This group featured more frequently ANA titles ≥1/640 and hypocomplementemia; although no statistical significance was found. The frequency of systemic manifestations found was greater than reported in the literature.

Conclusions

A multidisciplinary approach focusing also on systemic manifestations should be the new standard for management of SS.

Keywords:

Sjögren's Syndrome

Autoimmunity

Connective Tissue Diseases

Xerostomia

Resumen

Del 20–71% de los pacientes con síndrome de Sjögren (SS) desarrolla manifestaciones sistémicas.

Objetivos

El objetivo fue evaluar las características clinicoserológicas y frecuencia de manifestaciones sistémicas en pacientes con SS primario.

Material y métodos

Material y métodos: Estudio retrospectivo con revisión de historias clínicas de pacientes con Sd de Sjögren primario visitados en el Hospital Británico de Buenos Aires en el período desde Enero de 2000 a Agosto de 2008.

Resultados

Se incluyeron 41 pacientes que cumplian criterios de clasificacion Europeoamericanos 2002 para SS, todos de sexo femenino. La edad media fue 57,85±12,42 anos (rango 26–79). El tiempo de evolucion fue de 9,28 anos (rango 0,08–24). Treinta y tres (80,49%) presentaron manifestaciones sistemicas. Las mas frecuentes fueron artritis, vasculitis cutanea y polineuropatia. Este grupo presento mas frecuentemente titulos de AAN ≥1/640 e hipocomplementemia; aunque no estadisticamente significativas. La frecuencia de manifestaciones sistemicas halladas fue mayor a la reportada en otras series.

Conclusiones

Un abordaje multidisciplinario enfocado en las manifestaciones sistémicas debería ser el nuevo estándar para el manejo del SS.

Palabras clave:

Síndrome de Sjögren

Autoinmunidad

Enfermedades del tejido conectivo

Xerostomía

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References

[1.]

A. Hansen, P.E. Lipsky, T. Dorner.

Immunopathogenesis of primary Sjögren's syndrome: implications for disease management and therapy (review).

Curr Opin Rheumatol, 17 (2005), pp. 558-565

Medline

[2.]

R. Gerli, C. Muscat, M. Giansanti, M.G. Danieli, M. Sciuto, A. Gabrielli, et al.

Quantitative assessment of salivary gland inflammatory infiltration in primary Sjögren's syndrome: its relationship to different demographic, clinical and serological features of the disorder.

Br J Rheumatol, 36 (1997), pp. 969-975

Medline

[3.]

M. Garcia-Carrasco, M. Ramos-Casals, J. Rosas, L. Pallarés, J. Calvo-Alen, R. Cervera, et al.

Primary Sjogren syndrome: clinical and immunologic disease patterns in a cohort of 400 patients.

Medicine (Baltimore), 81 (2002), pp. 270-280

[4.]

R.I. Fox.

Sjögren's syndrome (review).

Lancet, 366 (2005), pp. 321-331

http://dx.doi.org/10.1016/S0140-6736(05)66990-5 | Medline

[5.]

E. Theander, S.I. Andersson, R. Manthorpe, L.H. Jacobsson.

Proposed core set of outcome measures in patients with primary Sjögren's syndrome: 5 year followup.

J Rheumatol, 32 (2005), pp. 109-116

[6.]

M. Ramos-Casals, A.G. Tzioufas, J. Font.

Primary Sjögren's syndrome: new clinical and therapeutic concepts (review).

Ann Rheum Dis, 64 (2005), pp. 347-354

http://dx.doi.org/10.1136/ard.2004.025676 | Medline

[7.]

N. Talal.

What is Sjögren's syndrome and why is it important?.

J Rheumatol, 27 (2000), pp. 1-3

Medline

[8.]

N.A. Pavlidis, J. Karsh, H.M. Moutsopoulos.

The clinical picture of primary Sjögren's syndrome: a retrospective study.

J Rheumatol, 9 (1982), pp. 685-690

Medline

[9.]

C. Vitali, S. Bombardieri, R. Jonsson, H.M. Moutsopoulos, E.L. Alexander, S.E. Carsons, European Study Group on classification criteria for Sjögren's syndrome, et al.

Classification criteria for Sjögren's syndrome: a revised version of the European criteria proposed by the American-European Consensus Group.

Ann Rheum Dis, 61 (2002), pp. 554-558

Medline

[10.]

D.I. Mitsias, E.K. Kapsogeorgou, H.M. Moutsopoulos.

Sjögren's syndrome: why autoimmune epithelitis?.

Oral Dis, 12 (2006), pp. 523-532

http://dx.doi.org/10.1111/j.1601-0825.2006.01292.x | Medline

[11.]

J.P. Ioannidis, V.A. Vassiliou, H.M. Moutsopoulos.

Long term risk of mortality and lymphoproliferative diseases and predictive classification of primary Sjögren's syndrome.

Arthritis Rheum, 46 (2002), pp. 741-747

http://dx.doi.org/10.1002/art.10221 | Medline

[12.]

E. Theander, L.T.H. Jacobsson.

Relationship of Sjögren's syndrome to other connective tissue and autoimmune disorders.

Rheum Dis Clin N Am, 34 (2008), pp. 935-947

[13.]

M. Ramos-Casals, R. Solans, J. Rosas, M.T. Camps, A. Gil, J. Del Pino-Montes, et al.

Primary Sjogren syndrome in Spain: clinical and immunologic expression in 1010 patients.

Medicine (Baltimore), 87 (2008), pp. 210-219

[14.]

K. Asmussen, V. Anderson, G. Bendixen, M. Schiødt, P. Oxholm.

A new model for classification of disease manifestations in primary Sjögren's syndrome: evaluation in a retrospective long-term study.

J Intern Med, 239 (1996), pp. 475-482

Medline

[15.]

J.M. Anaya, P.A. Correa, R.D. Mantilla.

Síndrome de Sjogren primario. Manifestaciones clínicas e inmunogenéticas.

Acta Med Colomb, 24 (1999), pp. 127-136

[16.]

F. Shah, R.P. Rapini, F.C. Arnett, N.B. Warner, C.A. Smith.

Association of labial salivary gland histopathology with clinical and serologic features of connective tissue diseases.

Arthritis Rheum, 33 (1990), pp. 1682-1687

Medline

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