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

  • Ali TaylanEmail author
  • Emel Tekin
  • Bahar Engin
Case Based Review


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.


Systemic sclerosis and systemic lupus erythematosus overlap Glomerulonephritis Hemolytic anemia Scleroderma renal crisis 


Author 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.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

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.

Informed consent

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


  1. 1.
    Cervera R, Khamashta MA, Hughes GR (1990) 'Overlap' syndromes. Ann Rheum Dis 49(11):947–948. CrossRefPubMedPubMedCentralGoogle Scholar
  2. 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. 2018 Jul 15) CrossRefPubMedGoogle Scholar
  3. 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. 2011 Aug 15) CrossRefPubMedGoogle Scholar
  4. 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. 2014 Jan 3) CrossRefPubMedGoogle Scholar
  5. 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):148–159CrossRefGoogle Scholar
  6. 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. CrossRefPubMedGoogle Scholar
  7. 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. CrossRefPubMedPubMedCentralGoogle Scholar
  8. 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):216–220CrossRefGoogle Scholar
  9. 9.
    Denton CP, Lapadula G, Mouthon L, Müller-Ladner U (2009) Renal complications and scleroderma renal crisis. Rheumatology (Oxford) 48(Suppl 3):iii32–iii35. CrossRefGoogle Scholar
  10. 10.
    Hudson M (2015) Scleroderma renal crisis. Curr Opin Rheumatol 27(6):549–554. CrossRefPubMedGoogle Scholar
  11. 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. 2018 Aug 24) CrossRefPubMedGoogle Scholar
  12. 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. CrossRefPubMedGoogle Scholar
  13. 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. 2010 Dec 4) CrossRefPubMedGoogle Scholar
  14. 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. 2014 Feb 19) CrossRefPubMedGoogle Scholar
  15. 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. 2017 May 28) CrossRefPubMedGoogle Scholar
  16. 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. 2009 Oct 5) CrossRefGoogle Scholar
  17. 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):153–157Google Scholar
  18. 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):602–605CrossRefGoogle Scholar
  19. 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. 2018 Jul 20) CrossRefPubMedGoogle Scholar
  20. 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

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of Rheumatology, Izmir Tepecik Training and Research HospitalSaglik Bilimleri UniversityIzmirTurkey
  2. 2.Department of Pathology, Izmir Tepecik Training and Research HospitalSaglik Bilimleri UniversityIzmirTurkey
  3. 3.Department of Internal Medicine, Izmir Tepecik Training and Research HospitalSaglik Bilimleri UniversityIzmirTurkey

Personalised recommendations