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