1887
Volume 2025, Issue 3
  • ISSN: 0253-8253
  • EISSN: 2227-0426

Abstract

Systemic lupus erythematosus (SLE) and Sjögren’s syndrome (SS) are chronic autoimmune diseases that can coexist, complicating the diagnostic process due to overlapping clinical and serological features. Hypergammaglobulinemia, often associated with these conditions, can mimic hematological malignancies, posing a significant diagnostic challenge.

We report a case of a 57-year-old woman presenting with progressive fatigue, dry cough, and vasculitic rashes for several weeks. Initial laboratory results revealed pancytopenia, hypergammaglobulinemia, and elevated inflammatory markers (erythrocyte sedimentation rate (ESR) >100 mm/hr, C-reactive protein (CRP) 137.7 mg/L), raising suspicion for multiple myeloma. However, protein electrophoresis (which showed polyclonal rather than monoclonal globulinemia) and bone marrow biopsy excluded malignancy. Further autoimmune screening confirmed coexisting SLE and SS, with positive ANA (≥1:1280), anti-dsDNA, anti-Ro, and anti-La antibodies. Polyclonal hypergammaglobulinemia was attributed to these autoimmune conditions. The patient responded well to azathioprine and hydroxychloroquine, with significant clinical and biochemical improvement.

This case highlights the importance of distinguishing polyclonal hypergammaglobulinemia due to autoimmune diseases from monoclonal gammopathies such as multiple myeloma. Early autoimmune screening and multidisciplinary collaboration were essential in achieving an accurate diagnosis and appropriate management.

Polyclonal hypergammaglobulinemia in the presence of elevated inflammatory markers warrants a thorough differential diagnosis to distinguish autoimmune conditions from hematological malignancies. This case underscores the critical role of comprehensive autoimmune screening in guiding timely and accurate diagnosis.

Loading

Article metrics loading...

/content/journals/10.5339/qmj.2025.91
2025-09-09
2025-12-16

Metrics

Loading full text...

Full text loading...

/deliver/fulltext/qmj/2025/3/qmj.2025.91.html?itemId=/content/journals/10.5339/qmj.2025.91&mimeType=html&fmt=ahah

References

  1. Rider V, Abdou NI, Kimler BF, Lu N, Brown S, Fridley BL. Gender bias in human systemic lupus erythematosus: A problem of steroid receptor action? Front Immunol. 2018Mar28: 9:611. http://www.doi.org/10.3389/fimmu.2018.00611
    [Google Scholar]
  2. Peri Y, Agmon-Levin N, Theodor E, Shoenfeld Y. Sjögren’s syndrome, the old and the new. Best Pract Res Clin Rheumatol. 2012Feb; 26:(1):105–17. https://doi.org/10.1016/j.berh.2012.01.012
    [Google Scholar]
  3. Mok CC, Lau CS. Pathogenesis of systemic lupus erythematosus. J Clin Pathol. 2003Jul; 56:(7):481–90. http://www.doi.org/10.1136/jcp.56.7.481
    [Google Scholar]
  4. Yeo AL, Kandane-Rathnayake R, Koelmeyer R, Golder V, Louthrenoo W, Chen Y-H, et al.. SMART-SLE: Serology monitoring and repeat testing in systemic lupus erythematosus—an analysis of anti-double-stranded DNA monitoring. Rheumatology (Oxford). 2024Feb1; 63:(2):525–33. http://www.doi.org/10.1093/rheumatology/kead231
    [Google Scholar]
  5. Eastham AB, Vleugels RA. Cutaneous lupus erythematosus. JAMA Dermatol. 2014Mar; 150:(3):344. http://www.doi.org/10.1001/jamader matol.2013.10393
    [Google Scholar]
  6. Jiang J, Zhao J, Liu D, Zhang M. Different roles of urinary light chains and serum light chains as potential biomarkers for monitoring disease activity in systemic lupus erythematosus. PeerJ. 2022May17; 10:e13385. http://www.doi.org/10.7717/peerj. 13385
    [Google Scholar]
  7. Brebner JA, Stockley RA. Polyclonal free light chains: A biomarker of inflammatory disease or treatment target? F1000 Med Rep. 2013: 5:4. https://doi.org/10.3410/M5-4
    [Google Scholar]
  8. Smeele HTW, Schreurs MWJ, Costedoat-Chalumeau N, Cornette JMJ, Dolhain R. Low prevalence of anti-SSA (anti-Ro) and anti-SSB (anti-La) autoantibodies in female patients with rheumatoid arthritis with a wish to conceive. RMD Open. 2021Jul; 7:(2):e001727. 10.1136/rmdopen-2021-001727
    [Google Scholar]
  9. Stefanski AL, Tomiak C, Pleyer U, Dietrich T, Burmester GR, Dörner T. The diagnosis and treatment of Sjögren’s syndrome. Dtsch Arztebl Int. 2017May;26; 114:(20):354–61. http://www.doi.org/10. 3238/arztebl.2017.0354
    [Google Scholar]
  10. Błochowiak K, Olewicz-Gawlik A, Polańska A, Nowak-Gabryel M, Kocięcki J, Witmanowski H, et al.. Oral mucosal manifestations in primary and secondary Sjögren syndrome and dry mouth syndrome. Postepy Dermatol Alergol. 2016Feb; 33:(1):23–7. http://www.doi.org/10.5114/pdia. 2016.57764
    [Google Scholar]
  11. Akpek EK, Bunya VY, Saldanha IJ. Sjögren’s syndrome: More than just dry eye. Cornea. 2019May; 38:(5):658–61. 10.1097/ICO. 0000000000001865
    [Google Scholar]
  12. Karp DR, Bacalao M. Differential diagnosis and disease associations. Dubois’ lupus erythematosus and related syndromes. Elsevier;2025. p. 693–702. http://www.doi.org/10.1016/B978-0-323-93232-5.00058-7
    [Google Scholar]
  13. Björk A, Mofors J, Wahren-Herlenius M. Environmental factors in the pathogenesis of primary Sjögren’s syndrome. J Intern Med. 2020May; 287:(5):475–92. 10.1111/joim.13032
    [Google Scholar]
  14. Durán-Barragán S, Bátiz-Andrade JP, Valenzuela-Marrufo R, Alarcón GS. Influence of the environment, gender, and hormones on systemic lupus erythematosus: A narrative review. Rev Colomb Reumatol. 2021; 28:177–90. 10.1016/j.rcreu.2021.02.00
    [Google Scholar]
  15. Rasel M, Zahra F. Hypergammaglobulinemia (Polyclonal Gammopathy). Treasure Island (FL):StatPearls Publishing;2024.
    [Google Scholar]
  16. Lian L, Wang K, Xu S. Systemic lupus erythematosus associated with multiple myeloma: Two case reports and a literature review. Immun Inflamm Dis. 2023Jan; 11:(1):e755. http://www.doi.org/10.1002/iid3.755
    [Google Scholar]
  17. Desai R, Devaragudi S, Kaur L, Singh K, Bawa J, Theik NWY, et al.. SLE and multiple myeloma: An underlooked link? A review of case reports from the last decade. J Med Life. 2024Feb; 17:(2):141–6. https://doi.org/10.25122/jml-2023-0314
    [Google Scholar]
  18. Han JY, Kim H, Jung SY, Jang EJ, Cho SK, Sung YK. Increased risk of malignancy in patients with systemic lupus erythematosus: Population-based cohort study in Korea. Arthritis Res Ther. 2021Oct27; 23:(1):270. http://www.doi.org/10.1186/s13075-021-02648-y
    [Google Scholar]
  19. Xue K, Niu WQ, Cui Y. Association of HLA-DR3 and HLA-DR15 polymorphisms with risk of systemic lupus erythematosus. Chin Med J (Engl). 2018Dec;5; 131:(23):2844–51. http://www.doi.org/10.4103/0366-6999.246058
    [Google Scholar]
/content/journals/10.5339/qmj.2025.91
Loading
/content/journals/10.5339/qmj.2025.91
Loading

Data & Media loading...

This is a required field
Please enter a valid email address
Approval was a Success
Invalid data
An Error Occurred
Approval was partially successful, following selected items could not be processed due to error