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Glomerulonephritis and Coombs-positive hemolytic anemia mimicking scleroderma renal crisis in an overlap of systemic lupus erythematosus and diffuse systemic sclerosis

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Abstract

Systemic sclerosis (SSc) may overlap frequently with other rheumatic diseases, including systemic lupus erythematosus (SLE), which can require high-dose steroids depending on the clinical presentation. We present a case involving this overlap in which the patient concomitantly developed lupus nephritis and Coombs (+) hemolytic anemia, which was confused with scleroderma renal crisis. In this condition, assessing for lupus nephritis with timely renal biopsy and lupus serology can aid in guiding the appropriate treatment. We discuss the clinical features and challenging management of this case with a review of the English-language literature for SSc and SLE overlap with glomerulonephritis.

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References

  1. Cervera R, Khamashta MA, Hughes GR (1990) 'Overlap' syndromes. Ann Rheum Dis 49(11):947–948. https://doi.org/10.1136/ard.49.11.947

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  2. Alharbi S, Ahmad Z, Bookman AA, Touma Z, Sanchez-Guerrero J et al (2018) Epidemiology and survival of systemic sclerosis-systemic lupus erythematosus overlap syndrome. J Rheumatol. 45(10):1406–1410. https://doi.org/10.3899/jrheum.170953(Epub 2018 Jul 15)

    Article  PubMed  Google Scholar 

  3. Pakozdi A, Nihtyanova S, Moinzadeh P, Ong VH, Black CM et al (2011) Clinical and serological hallmarks of systemic sclerosis overlap syndromes. J Rheumatol 38(11):2406–2409. https://doi.org/10.3899/jrheum.101248(Epub 2011 Aug 15)

    Article  PubMed  Google Scholar 

  4. Moinzadeh P, Aberer E, Ahmadi-Simab K, Blank N, Distler JH et al (2015) Disease progression in systemic sclerosis-overlap syndrome is significantly different from limited and diffuse cutaneous systemic sclerosis. Ann Rheum Dis 74(4):730–737. https://doi.org/10.1136/annrheumdis-2013-204487(Epub 2014 Jan 3)

    Article  CAS  PubMed  Google Scholar 

  5. Sharp GC, Irvin WS, Tan EM, Gould RG, Holman HR (1972) Mixed connective tissue disease–an apparently distinct rheumatic disease syndrome associated with a specific antibody to an extractable nuclear antigen (ENA). Am J Med. 52(2):148–159

    Article  CAS  Google Scholar 

  6. van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A et al (2013) 2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative. Ann Rheum Dis. 72(11):1747–1755. https://doi.org/10.1136/annrheumdis-2013-204424

    Article  PubMed  Google Scholar 

  7. Petri M, Orbai AM, Alarcón GS, Gordon C, Merrill JT et al (2012) Derivation and validation of the systemic lupus international collaborating clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum. 64(8):2677–2686. https://doi.org/10.1002/art.34473

    Article  PubMed  PubMed Central  Google Scholar 

  8. Walker JG, Ahern MJ, Smith MD, Coleman M, Pile K et al (2003) Scleroderma renal crisis: poor outcome despite aggressive antihypertensive treatment. Intern Med J 33(5–6):216–220

    Article  CAS  Google Scholar 

  9. Denton CP, Lapadula G, Mouthon L, Müller-Ladner U (2009) Renal complications and scleroderma renal crisis. Rheumatology (Oxford) 48(Suppl 3):iii32–iii35. https://doi.org/10.1093/rheumatology/ken483

    Article  Google Scholar 

  10. Hudson M (2015) Scleroderma renal crisis. Curr Opin Rheumatol 27(6):549–554. https://doi.org/10.1097/BOR.0000000000000221

    Article  CAS  PubMed  Google Scholar 

  11. Shimizu T, Iwamoto N, Okamoto M, Endo Y, Tsuji S et al (2019) Scleroderma renal crisis complicated with thrombotic microangiopathy triggered by influenza B virus infection. Intern Med. 58(3):441–445. https://doi.org/10.2169/internalmedicine.1441-18(Epub 2018 Aug 24)

    Article  PubMed  Google Scholar 

  12. Mouthon L, Bérezné A, Bussone G, Noël LH, Villiger PM et al (2011) Scleroderma renal crisis: a rare but severe complication of systemic sclerosis. Clin Rev Allergy Immunol 40(2):84–91. https://doi.org/10.1007/s12016-009-8191-5

    Article  PubMed  Google Scholar 

  13. Trang G, Steele R, Baron M, Hudson M (2012) Corticosteroids and the risk of scleroderma renal crisis: a systematic review. Rheumatol Int 32(3):645–653. https://doi.org/10.1007/s00296-010-1697-6(Epub 2010 Dec 4)

    Article  CAS  PubMed  Google Scholar 

  14. Pokroy-Shapira E, Gelernter I, Molad Y (2014) Evolution of chronic kidney disease in patients with systemic lupus erythematosus over a long-period follow-up: a single-center inception cohort study. Clin Rheumatol. 33(5):649–657. https://doi.org/10.1007/s10067-014-2527-0(Epub 2014 Feb 19)

    Article  PubMed  Google Scholar 

  15. Ruiz-Irastorza G, Ugarte A, Saint-Pastou Terrier C, Lazaro E, Iza A et al (2017) Repeated pulses of methyl-prednisolone with reduced doses of prednisone improve the outcome of class III, IV and V lupus nephritis: an observational comparative study of the Lupus-Cruces and lupus-Bordeaux cohorts. Autoimmun Rev. 16(8):826–832. https://doi.org/10.1016/j.autrev.2017.05.017(Epub 2017 May 28)

    Article  CAS  PubMed  Google Scholar 

  16. Satoh T, Ishikawa O, Ihn H, Endo H, Kawaguchi Y et al (2009) Clinical usefulness of anti-RNA polymerase III antibody measurement by enzyme-linked immunosorbent assay. Rheumatology (Oxford) 48(12):1570–1574. https://doi.org/10.1093/rheumatology/kep290(Epub 2009 Oct 5)

    Article  CAS  Google Scholar 

  17. Alayoud A, Qamouss O, Hamzi MA, Benyahia M, Oualim Z (2012) Scleroderma renal crisis precipitated by steroid treatment in systemic lupus erythematosus and scleroderma overlap syndrome. Arab J Nephrol Transpl 5(3):153–157

    Google Scholar 

  18. Tewari R, Badwal S, Kumar A, Subramaniam S, Nijhawan SV, Srinivas V (2016) Anti-neutrophil cytoplasmic antibody negative crescentic paucimmune glomerulonephritis in a case of scleroderma with systemic lupus erythematosus overlap. Saudi J Kidney Dis Transpl. 27(3):602–605

    Article  Google Scholar 

  19. McConville E, Smith D, Bélanger E, Ivory C (2018) Development of lupus nephritis in a patient with previous scleroderma renal crisis. Lupus. 27(10):1732–1734. https://doi.org/10.1177/0961203318785776(Epub 2018 Jul 20)

    Article  CAS  PubMed  Google Scholar 

  20. Fraticelli P, Fischetti C, Salaffi F, Carotti M, Mattioli M et al (2018) Combination therapy with rituximab and mycophenolate mofetil in systemic sclerosis. A single-centre case series study. Clin Exp Rheumatol 113(4):142–145 (Epub 2018 Sep 30)

    Google Scholar 

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Authors and Affiliations

  1. Department of Rheumatology, Izmir Tepecik Training and Research Hospital, Saglik Bilimleri University, Yenisehir, Izmir, Turkey

    Ali Taylan

  2. Department of Pathology, Izmir Tepecik Training and Research Hospital, Saglik Bilimleri University, Izmir, Turkey

    Emel Tekin

  3. Department of Internal Medicine, Izmir Tepecik Training and Research Hospital, Saglik Bilimleri University, Izmir, Turkey

    Bahar Engin

Authors
  1. Ali Taylan
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  2. Emel Tekin
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  3. Bahar Engin
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Contributions

Conception and design of this study: all authors. Interpretation of results and drafting the manuscript: T.A, E.B Acquisition of data: E.B, Y.E Critical revision of manuscript: T.A Manuscript approval: all authors.

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Correspondence to Ali Taylan.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

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Informed consent was obtained from the individual participant included in the study.

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Taylan, A., Tekin, E. & Engin, B. Glomerulonephritis and Coombs-positive hemolytic anemia mimicking scleroderma renal crisis in an overlap of systemic lupus erythematosus and diffuse systemic sclerosis. Rheumatol Int 40, 785–789 (2020). https://doi.org/10.1007/s00296-019-04471-w

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  • DOI: https://doi.org/10.1007/s00296-019-04471-w

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