Abstract
Background
The coexistence of systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA) named Rhupus is an unusual clinical condition. Previous reports mentioned that Rhupus patients have prominent RA-associated clinical manifestations and only mild organic damage related to SLE. Progressive or life-threatening manifestations are rare in Rhupus patients.
Methods
Three patients with Rhupus are described in this article. Two of them presented antiphospholipid syndrome (APS) in addition to Rhupus. Also, we searched for similar cases in published literature.
Results
We present three patients with Rhupus syndrome. One of the patients has only Rhupus, the second patient has Rhupus and APS, and the third patient has Rhupus accompanied by severe Raynaud’s syndrome with digital ulcers, APS, pulmonary hypertension and two malignancies.
Several studies have shown that Rhupus patients have an increased prevalence of positive antiphospholipid antibodies that resembles SLE. However, the presence of these antibodies is not associated with APS. There is only one case of Rhupus with secondary APS in which the patient presented headache and papilloedema due to cerebral venous thrombosis. Secondary Raynaud’s syndrome is rare in Rhupus patients, and to the best of our knowledge, only three cases of this are mentioned in literature. Secondary pulmonary hypertension and malignancies were never reported before in Rhupus patients.
Conclusions
Rheumatologists should be aware of the possibility that Rhupus may be accompanied by progressive or life-threatening conditions such as APS, severe Raynaud’s syndrome with digital ulcers, pulmonary hypertension, or malignancies.
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References
Schur PH (1971) Systemic lupus erythematosus. In: Beeson PB, McDermott W (eds) Cecil-Loeb texbook of medicine. Sanders, Philadelphia, p 821
Simón JA, Granados J, Cabiedes J, Morales JR, Varela JA (2002) Clinical and immunogenetic characterization of Mexican patients with ‘rhupus’. Lupus 11:287–292
Fernández A, Quintana G, Matteson EL, Restrepo JF, Rondón F, Sánchez A et al (2004) Lupus arthropathy: historical evolution from deforming arthritis to rhupus. Clin Rheumatol 23:523–526
Prete M, Racanelli V, Digiglio L, Vacca A, Dammacco F, Perosa F (2011) Extra-articularmanifestations of rheumatoid arthritis: an update. Autoimmun Rev 11:123–131
Iaccarino L, Gatto M, Bettio S, Caso F, Rampudda M, Zen M et al (2013) Overlap connective tissue disease syndromes. Autoimmun Rev 12:363–373
Panush RS, Edwards NL, Longley S, Webster E (1988) ‘Rhupus’ syndrome. Arch Intern Med 148:1633–1636
Cohen MG, Webb J (1987) Concurrence of rheumatoid arthritis and systemic lupus erythematosus: report of 11 cases. Ann Rheum Dis 46:853–858
Brand CA, Rowley MJ, Tait BD, Muirden KD, Whittingham SF (1992) Coexistent rheumatoid arthritis and systemic lupus erythematosus; clinical, serological, and phenotypic features. Ann Rheum Dis 51:173–176
Li J, Wu H, Huang X, Xu D, Zheng W, Zhao Y et al (2014) Clinical analysis of 56 patients with rhupus syndrome: manifestations and comparisons with systemic lupus erythematosus: a retrospective case-control study. Medicine (Baltimore) 93
Hayakawa S, Komine-Aizawa S, Osaka S, Iida T, Hayakawa J, Nishinarita S et al (2007) Rembrandt’s Maria Bockenolle has a butterfly rash and digital deformities: overlapping syndrome of rheumatoid arthritis and systemic lupus erythematosus. Med Hypotheses 68(4):906–909
Liu T, Li G, Mu R, Ye H, Li W, Li Z (2014) Clinical and laboratory profiles of rhupus syndrome in a Chinese population: a single-centre study of 51 patients. Lupus 23:958–963
Tani C, D’Aniello D, Delle Sedie A, Carli L, Cagnoni M, Possemato N et al (2013) Rhupus syndrome: assessment of its prevalence and its clinical and instrumental characteristics in a prospective cohort of 103 SLE patients. Autoimmun Rev 12:537–541
Cohen S, Stastny P, Sontheimer RD (1986) Concurrence of subacute cutaneous lupus erythematosus and rheumatoid arthritis. Arthritis Rheum 29:421–425
Mosca M, Tani C, Carli L, Bombardieri S (2011) Glucocorticoids in systemic lupus erythematosus. Clin Exp Rheumatol 29:S126–S129
Agmon-Levin N, Mosca M, Petri M (2012) Systemic lupus erythematosus one disease or many? Autoimmun Rev 11:593–595
Satoh M, Ajmani AK, Akizuki M (1994) What is the definition for coexistent rheumatoid arthritis and systemic lupus erythematosus? Lupus 3:137–138
Piga M, Gabba A, Cauli A, Garau P, Vacca A, Mathieu A (2013) Rituximab treatment for ‘rhupus syndrome’: clinical and power-Doppler ultrasonographic monitoring of response. A longitudinal pilot study. Lupus 22:624–628
Ostendorf B, Scherer A, Specker C (2003) Jaccoud’s arthropathy in systemic lupus erythematosus: differentiation of deforming and erosive patterns by magnetic resonance imaging. Arthritis Rheum 48:157–165
Fernández A, Quintana G, Rondón F, Restrepo JF, Sánchez A, Matteson EL et al (2006) Lupus arthropathy: a case series of patients with rhupus. Clin Rheumatol 25:164–167
Sarkar S, Saha K (2012) Bilateral acute lupus pneumonitis in a case of rhupus syndrome. Lung India 29:280–282
Wang JG, Tang HH, Tan CY, Liu Y, Lin H, Chen YT (2010) Diffuse lupus encephalopathy in a case of rhupus syndrome. Rheumatol Int 30:961–963
Chowdhury SR, Majumder S, Chakraborty PP, Mandal SK, Bandyopadhyay R, Bandyopadhyay D et al (2008) Headache in a case of Rhupus syndrome. Headache 48:155–158
Alarcón-Segovia D, Cardiel MH (1989) Comparison between 3 diagnostic criteria for mixed connective tissue disease. Study of 593 patients. J Rheumatol 16:328–334
Turesson C, Matteson EL (2013) Malignancy as a comorbidity in rheumatic diseases. Rheumatology (Oxford) 52:5–14
Skare TL, Nisihara R, Barbosa BB, da Luz A, Utiyama S, Picceli V (2013) Anti-CCP in systemic lupus erythematosus patients: a cross sectional study in Brazilian patients. Clin Rheumatol 32:1065–1070
Liao KP, Kurreeman F, Li G, Duclos G, Murphy S, Guzman R et al (2013) Associations of autoantibodies, autoimmune risk alleles, and clinical diagnoses from the electronic medical records in rheumatoid arthritis cases and non-rheumatoid arthritis controls. Arthritis Rheum 65:571–581
Mawson AR (1985) Are rheumatoid arthritis and systemic lupus erythematosus inversely related diseases? Med Hypotheses 18:377–386
Sundaramurthy SG, Karsevar MP, van Vollenhoven RF (1999) Influence of hormonal events on disease expression in patients with the combination of systemic lupus erythematosus and rheumatoid arthritis. J Clin Rheumatol 5:9–16
Pipili C, Sfritzeri A, Cholongitas E (2008) Deforming arthropathy in systemic lupus erythematosus. Eur J Intern Med 19:482–487
Aringer M, Smolen JS (2012) Therapeutic blockade of TNF in patients with SLE-promising or crazy? Autoimmun Rev 11:321–325
Andrade-Ortega L, Irazoque-Palazuelos F, Muñóz-López S, Rosales-Don Pablo VM (2013) Efficacy and tolerability of rituximab in patients with rhupus. Reumatol Clin 9:201–205
Ikeda K, Sanayama Y, Makita S, Hosokawa J, Yamagata M, Nakagomi D et al (2013) Efficacy of abatacept for arthritis in patients with an overlap syndrome between rheumatoid arthritis and systemic lupus erythematosus. Clin Dev Immunol. 2013: Article ID 697525
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Shovman, O., Langevitz, P. & Shoenfeld, Y. Rhupus; unusual presentations. Clin Rheumatol 34, 2041–2046 (2015). https://doi.org/10.1007/s10067-015-2978-y
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DOI: https://doi.org/10.1007/s10067-015-2978-y