Glomerulonephritis and Coombs-positive hemolytic anemia mimicking scleroderma renal crisis in an overlap of systemic lupus erythematosus and diffuse systemic sclerosis
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.
KeywordsSystemic sclerosis and systemic lupus erythematosus overlap Glomerulonephritis Hemolytic anemia Scleroderma renal crisis
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.
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 was obtained from the individual participant included in the study.
- 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) CrossRefPubMedGoogle 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 CrossRefPubMedGoogle 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 CrossRefPubMedPubMedCentralGoogle 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) CrossRefPubMedGoogle 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) CrossRefPubMedGoogle 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):153–157Google 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