Cutaneous Vasculitis in Primary Sjögren Syndrome: Classification and Clinical Significance of 52 Patients

Manuel Ramos-Casals, Juan Manuel Anaya, Mario García-Carrasco, José Rosas, Albert Bové, Gisela Claver, Luis Aurelio Diaz, Carmen Herrero, Josep Font

Resultado de la investigación: Contribución a RevistaArtículo

126 Citas (Scopus)

Resumen

To analyze the different clinical and histologic types of cutaneous vasculitis in patients with primary Sjögren syndrome (SS), we investigated the clinical and immunologic characteristics of 558 consecutive patients with primary SS from our units and selected those with clinical evidence of cutaneous lesions, excluding drug reactions and xeroderma. All patients fulfilled 4 or more of the diagnostic criteria for SS proposed by the European Community Study Group in 1993. A total of 89 (16%) patients presented with cutaneous involvement (88 female patients and 1 male; mean age, 51.8 yr). The main cutaneous involvement was cutaneous vasculitis, present in 52 (58%) patients. There were 51 (98%) female patients and 1 (2%) male, with a mean age at diagnosis of cutaneous vasculitis of 51 years (range, 20-80 yr). Fourteen presented with cryoglobulinemic vasculitis, 11 with urticarial vasculitis, and the remaining 26, with cutaneous purpura not associated with cryoglobulins. A skin biopsy specimen was obtained in 38 patients (73%). Involvement of small-sized vessels was observed in 36 (95%) patients (leukocytoclastic vasculitis), while the remaining 2 (5%) presented with medium-sized vessel vasculitis (necrotizing vasculitis). Patients with cutaneous vasculitis had a higher prevalence of articular involvement (50% vs 29%, p = 0.044), peripheral neuropathy (31% vs 4%, p <0.001), Raynaud phenomenon (40% vs 15%, p = 0.008), renal involvement (10% vs 0%, p = 0.028), antinuclear antibodies (88% vs 60%, p = 0.002), rheumatoid factor (78% vs 48%, p = 0.004), anti-Ro/SS-A antibodies (70% vs 43%, p = 0.011), and hospitalization (25% vs 4%, p = 0.005) compared with SS patients without vasculitis. Six (12%) patients died, all of whom had multisystemic cryoglobulinemia. In conclusion, cutaneous involvement was detected in 16% of patients with primary SS, with cutaneous vasculitis being the most frequent process. The main characteristics of SS-associated cutaneous vasculitis were the overwhelming predominance of small versus medium vessel vasculitis and leukocytoclastic versus mononuclear vasculitis, with a higher prevalence of extraglandular and immunologic SS features. Small vessel vasculitis manifested as palpable purpura, urticarial lesions, or erythematosus maculopapules, with systemic involvement in 44% of patients in association with cryoglobulins in 30%. Life-threatening vasculitis was closely related to cryoglobulinemia.
Idioma originalEnglish (US)
Páginas (desde-hasta)96-106
Número de páginas11
PublicaciónMedicine
DOI
EstadoPublished - mar 1 2004

Huella dactilar

Sjogren's Syndrome
Vasculitis
Skin
Cryoglobulins
Cryoglobulinemia
Purpura
Ichthyosis
Raynaud Disease
Rheumatoid Factor
Antinuclear Antibodies
Peripheral Nervous System Diseases
European Union
Hospitalization

Citar esto

Ramos-Casals, Manuel ; Anaya, Juan Manuel ; García-Carrasco, Mario ; Rosas, José ; Bové, Albert ; Claver, Gisela ; Diaz, Luis Aurelio ; Herrero, Carmen ; Font, Josep. / Cutaneous Vasculitis in Primary Sjögren Syndrome: Classification and Clinical Significance of 52 Patients. En: Medicine. 2004 ; pp. 96-106.
@article{4fc8274afb7d41009d8b374af9ef2c4f,
title = "Cutaneous Vasculitis in Primary Sj{\"o}gren Syndrome: Classification and Clinical Significance of 52 Patients",
abstract = "To analyze the different clinical and histologic types of cutaneous vasculitis in patients with primary Sj{\"o}gren syndrome (SS), we investigated the clinical and immunologic characteristics of 558 consecutive patients with primary SS from our units and selected those with clinical evidence of cutaneous lesions, excluding drug reactions and xeroderma. All patients fulfilled 4 or more of the diagnostic criteria for SS proposed by the European Community Study Group in 1993. A total of 89 (16{\%}) patients presented with cutaneous involvement (88 female patients and 1 male; mean age, 51.8 yr). The main cutaneous involvement was cutaneous vasculitis, present in 52 (58{\%}) patients. There were 51 (98{\%}) female patients and 1 (2{\%}) male, with a mean age at diagnosis of cutaneous vasculitis of 51 years (range, 20-80 yr). Fourteen presented with cryoglobulinemic vasculitis, 11 with urticarial vasculitis, and the remaining 26, with cutaneous purpura not associated with cryoglobulins. A skin biopsy specimen was obtained in 38 patients (73{\%}). Involvement of small-sized vessels was observed in 36 (95{\%}) patients (leukocytoclastic vasculitis), while the remaining 2 (5{\%}) presented with medium-sized vessel vasculitis (necrotizing vasculitis). Patients with cutaneous vasculitis had a higher prevalence of articular involvement (50{\%} vs 29{\%}, p = 0.044), peripheral neuropathy (31{\%} vs 4{\%}, p <0.001), Raynaud phenomenon (40{\%} vs 15{\%}, p = 0.008), renal involvement (10{\%} vs 0{\%}, p = 0.028), antinuclear antibodies (88{\%} vs 60{\%}, p = 0.002), rheumatoid factor (78{\%} vs 48{\%}, p = 0.004), anti-Ro/SS-A antibodies (70{\%} vs 43{\%}, p = 0.011), and hospitalization (25{\%} vs 4{\%}, p = 0.005) compared with SS patients without vasculitis. Six (12{\%}) patients died, all of whom had multisystemic cryoglobulinemia. In conclusion, cutaneous involvement was detected in 16{\%} of patients with primary SS, with cutaneous vasculitis being the most frequent process. The main characteristics of SS-associated cutaneous vasculitis were the overwhelming predominance of small versus medium vessel vasculitis and leukocytoclastic versus mononuclear vasculitis, with a higher prevalence of extraglandular and immunologic SS features. Small vessel vasculitis manifested as palpable purpura, urticarial lesions, or erythematosus maculopapules, with systemic involvement in 44{\%} of patients in association with cryoglobulins in 30{\%}. Life-threatening vasculitis was closely related to cryoglobulinemia.",
author = "Manuel Ramos-Casals and Anaya, {Juan Manuel} and Mario Garc{\'i}a-Carrasco and Jos{\'e} Rosas and Albert Bov{\'e} and Gisela Claver and Diaz, {Luis Aurelio} and Carmen Herrero and Josep Font",
year = "2004",
month = "3",
day = "1",
doi = "10.1097/01.md.0000119465.24818.98",
language = "English (US)",
pages = "96--106",
journal = "Medicine (United States)",
issn = "0025-7974",
publisher = "Lippincott Williams and Wilkins",

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Ramos-Casals, M, Anaya, JM, García-Carrasco, M, Rosas, J, Bové, A, Claver, G, Diaz, LA, Herrero, C & Font, J 2004, 'Cutaneous Vasculitis in Primary Sjögren Syndrome: Classification and Clinical Significance of 52 Patients', Medicine, pp. 96-106. https://doi.org/10.1097/01.md.0000119465.24818.98

Cutaneous Vasculitis in Primary Sjögren Syndrome: Classification and Clinical Significance of 52 Patients. / Ramos-Casals, Manuel; Anaya, Juan Manuel; García-Carrasco, Mario; Rosas, José; Bové, Albert; Claver, Gisela; Diaz, Luis Aurelio; Herrero, Carmen; Font, Josep.

En: Medicine, 01.03.2004, p. 96-106.

Resultado de la investigación: Contribución a RevistaArtículo

TY - JOUR

T1 - Cutaneous Vasculitis in Primary Sjögren Syndrome: Classification and Clinical Significance of 52 Patients

AU - Ramos-Casals, Manuel

AU - Anaya, Juan Manuel

AU - García-Carrasco, Mario

AU - Rosas, José

AU - Bové, Albert

AU - Claver, Gisela

AU - Diaz, Luis Aurelio

AU - Herrero, Carmen

AU - Font, Josep

PY - 2004/3/1

Y1 - 2004/3/1

N2 - To analyze the different clinical and histologic types of cutaneous vasculitis in patients with primary Sjögren syndrome (SS), we investigated the clinical and immunologic characteristics of 558 consecutive patients with primary SS from our units and selected those with clinical evidence of cutaneous lesions, excluding drug reactions and xeroderma. All patients fulfilled 4 or more of the diagnostic criteria for SS proposed by the European Community Study Group in 1993. A total of 89 (16%) patients presented with cutaneous involvement (88 female patients and 1 male; mean age, 51.8 yr). The main cutaneous involvement was cutaneous vasculitis, present in 52 (58%) patients. There were 51 (98%) female patients and 1 (2%) male, with a mean age at diagnosis of cutaneous vasculitis of 51 years (range, 20-80 yr). Fourteen presented with cryoglobulinemic vasculitis, 11 with urticarial vasculitis, and the remaining 26, with cutaneous purpura not associated with cryoglobulins. A skin biopsy specimen was obtained in 38 patients (73%). Involvement of small-sized vessels was observed in 36 (95%) patients (leukocytoclastic vasculitis), while the remaining 2 (5%) presented with medium-sized vessel vasculitis (necrotizing vasculitis). Patients with cutaneous vasculitis had a higher prevalence of articular involvement (50% vs 29%, p = 0.044), peripheral neuropathy (31% vs 4%, p <0.001), Raynaud phenomenon (40% vs 15%, p = 0.008), renal involvement (10% vs 0%, p = 0.028), antinuclear antibodies (88% vs 60%, p = 0.002), rheumatoid factor (78% vs 48%, p = 0.004), anti-Ro/SS-A antibodies (70% vs 43%, p = 0.011), and hospitalization (25% vs 4%, p = 0.005) compared with SS patients without vasculitis. Six (12%) patients died, all of whom had multisystemic cryoglobulinemia. In conclusion, cutaneous involvement was detected in 16% of patients with primary SS, with cutaneous vasculitis being the most frequent process. The main characteristics of SS-associated cutaneous vasculitis were the overwhelming predominance of small versus medium vessel vasculitis and leukocytoclastic versus mononuclear vasculitis, with a higher prevalence of extraglandular and immunologic SS features. Small vessel vasculitis manifested as palpable purpura, urticarial lesions, or erythematosus maculopapules, with systemic involvement in 44% of patients in association with cryoglobulins in 30%. Life-threatening vasculitis was closely related to cryoglobulinemia.

AB - To analyze the different clinical and histologic types of cutaneous vasculitis in patients with primary Sjögren syndrome (SS), we investigated the clinical and immunologic characteristics of 558 consecutive patients with primary SS from our units and selected those with clinical evidence of cutaneous lesions, excluding drug reactions and xeroderma. All patients fulfilled 4 or more of the diagnostic criteria for SS proposed by the European Community Study Group in 1993. A total of 89 (16%) patients presented with cutaneous involvement (88 female patients and 1 male; mean age, 51.8 yr). The main cutaneous involvement was cutaneous vasculitis, present in 52 (58%) patients. There were 51 (98%) female patients and 1 (2%) male, with a mean age at diagnosis of cutaneous vasculitis of 51 years (range, 20-80 yr). Fourteen presented with cryoglobulinemic vasculitis, 11 with urticarial vasculitis, and the remaining 26, with cutaneous purpura not associated with cryoglobulins. A skin biopsy specimen was obtained in 38 patients (73%). Involvement of small-sized vessels was observed in 36 (95%) patients (leukocytoclastic vasculitis), while the remaining 2 (5%) presented with medium-sized vessel vasculitis (necrotizing vasculitis). Patients with cutaneous vasculitis had a higher prevalence of articular involvement (50% vs 29%, p = 0.044), peripheral neuropathy (31% vs 4%, p <0.001), Raynaud phenomenon (40% vs 15%, p = 0.008), renal involvement (10% vs 0%, p = 0.028), antinuclear antibodies (88% vs 60%, p = 0.002), rheumatoid factor (78% vs 48%, p = 0.004), anti-Ro/SS-A antibodies (70% vs 43%, p = 0.011), and hospitalization (25% vs 4%, p = 0.005) compared with SS patients without vasculitis. Six (12%) patients died, all of whom had multisystemic cryoglobulinemia. In conclusion, cutaneous involvement was detected in 16% of patients with primary SS, with cutaneous vasculitis being the most frequent process. The main characteristics of SS-associated cutaneous vasculitis were the overwhelming predominance of small versus medium vessel vasculitis and leukocytoclastic versus mononuclear vasculitis, with a higher prevalence of extraglandular and immunologic SS features. Small vessel vasculitis manifested as palpable purpura, urticarial lesions, or erythematosus maculopapules, with systemic involvement in 44% of patients in association with cryoglobulins in 30%. Life-threatening vasculitis was closely related to cryoglobulinemia.

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DO - 10.1097/01.md.0000119465.24818.98

M3 - Article

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EP - 106

JO - Medicine (United States)

JF - Medicine (United States)

SN - 0025-7974

ER -