Journal of Diagnostics Concepts & Practice >

2021 , Vol. 20 >Issue 02: 155 - 160

DOI: https://doi.org/10.16150/j.1671-2870.2021.02.007

Original articles

Clinical features of primary Sjögren syndrome with purpura

Expand
  • a. Department of Dermatology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200025, China
    b. Department of Pulmonary and Critical Care Medicine, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200025, China

Received date: 2021-04-07

  Online published: 2022-06-28

Fold

Abstract

Objective: To study the clinical characteristics of patients with primary Sjögren syndrome(pSS) with purpura. Methods: A total of 101 patients with pSS were enrolled from January 2017 through January 2020 in Department of Dermatology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine. The patients were divided into 2 groups: pSS with purpura (purpura group, n=15) and without purpura (non-purpura group, n=86). The clinical presentations and laboratory data were compared between the 2 groups. Results: Of the 15 patients with manifestation of purpura (14.9%), 12(80%) were diagnosed as hypergammaglobulinemia purpura and the other 3(20%) were cryoglobulinemia purpura, thrombocytopenic purpura, and pigmented purpuric dermatosis, respectively. Four cases developed purpura prior to the glandular symptoms of dry mouth and eyes, and 11 cases had purpura after presentations of glandular symptoms. Com-paring with the non-purpura group, the pSS patients with purpura group had earlier onset age [(41.6±13.5) years vs. (51.7±11.4) years, P=0.003], higher incidence of lymphoma (2/15 vs. 0, P=0.011); higher serum levels of rheumatoid factor (RF) (P=0.002), erythrocyte sedimentation rate (ESR) (P=0.019), immunoglobulin (Ig) G (P<0.001), γ-globulin (P=0.001), free Ig light (κ) chains (P<0.001), free Ig heavy (λ) chains (P=0.015), and lower levels of compliment components C3 (P=0.011) and C4 (P=0.021). Conclusions: As a common and heterogeneous dermatological manifestation of pSS patients revealed in this study, the diagnosis of pSS should be considered in patients presenting purpura. The pSS patients with purpura with early onset and abnormal laboratory parameters have increased risk of lymphoma and demanded further monitoring.

Key words： Primary Sjögren syndrome; Cutaneous manifestation; Purpura; Lymphoma

Cite this article

ZHAO Qian, ZHAO Xiaoqing, DIAO Licheng, SUN Fei, ZHENG Jie, ZHU Xuemei, CAO Hua . Clinical features of primary Sjögren syndrome with purpura[J]. Journal of Diagnostics Concepts & Practice, 2021 , 20(02) : 155 -160 . DOI: 10.16150/j.1671-2870.2021.02.007

References

[1] 张乃峥, 曾庆馀, 张凤山, 等. 中国风湿性疾病流行情况的调查研究[J]. 中华风湿病学杂志, 1997, 1(1):34-38.
[2] 宣磊, 屈洋, 董振华. 原发性干燥综合征皮肤黏膜损害[J]. 中华临床免疫和变态反应杂志, 2015, 9(3):218-221.
[3] Theisen E, Lee DE, Pei S, et al. Hypergammaglobulinemic purpura of Waldenström in children[J]. Pediatr Dermatol, 2020, 37(3):467-475.
[4] Kittridge A, Routhouska SB, Korman NJ. Dermatologic manifestations of Sjögren syndrome[J]. J Cutan Med Surg, 2011, 15(1):8-14.
[5] 唐倩, 李志勇. 原发性干燥综合征误诊原因分析[J]. 现代医药卫生, 2015, 31(z2):1-3.
[6] Shiboski CH, Shiboski SC, Seror R, et al. 2016 American College of Rheumatology/European League Against Rheumatism Classification Criteria for Primary Sjögren’s Syndrome: a consensus and data-driven methodology involving three international patient cohorts[J]. Arthritis Rheumatol, 2017, 69(1):35-45.
[7] Franceschini F, Cavazzana I, Andreoli L, et al. The 2016 classification criteria for primary Sjögren’s syndrome: what’s new?[J]. BMC Med, 2017, 15(1):69.
[8] Orgeolet L, Foulquier N, Misery L, et al. Can artificial intelligence replace manual search for systematic literature? Review on cutaneous manifestations in primary Sjögren’s syndrome[J]. Rheumatology(Oxford), 2020, 59(4):811-819.
[9] Piette WW. The differential diagnosis of purpura from a morphologic perspective[J]. Adv Dermatol, 1994, 9:3-23
[10] Micheletti RG. Cutaneous vasculitis in rheumatologic disease: current concepts of skin and systemic manifestations[J]. Clin Dermatol, 2018, 36(4):561-566.
[11] Paravar T. Less common rheumatologic disorders: current concepts of skin and systemic manifestations[J]. Clin Dermatol, 2018, 36(4):525-532.
[12] Lamadrid-Zertuche AC, Garza-Rodríguez V, Ocampo-Candiani JJ. Pigmented purpura and cutaneous vascular occlusion syndromes[J]. An Bras Dermatol, 2018, 93(3):397-404.
[13] Anquetil C, Hachulla E, Machuron F, et al. Is early-onset primary Sjögren’s syndrome a worse prognosis form of the disease?[J]. Rheumatology (Oxford), 2019, 58(7):1163-1167.
[14] Yayla ME, Karaman Z, Torgutalp M, et al. Early onset primary Sjögren syndrome, clinical and laboratory characteristics[J]. Clin Rheumatol, 2020, 39(9):2689-2696.
[15] Nakano T, Matsui M, Inoue I, et al. Free immunoglobulin light chain: its biology and implications in diseases[J]. Clin Chim Acta, 2011, 412(11-12):843-849.
[16] Nocturne G, Pontarini E, Bombardieri M, et al. Lymphomas complicating primary Sjögren’s syndrome: from autoimmunity to lymphoma[J]. Clin Chim Acta, 2011, 412(11-12):843-849.
[17] Quartuccio L, Baldini C, Priori R, et al. Cryoglobulinemia in Sjögren syndrome: a disease subset that links higher systemic disease activity, autoimmunity, and local B cell proliferation in mucosa-associated lymphoid tissue[J]. J Rheumatol, 2017, 44(8):1179-1183.
Options
Outlines

/

〈 〉