Rhupus syndromeM32.9

"Rhupus" or "Rhupus syndrome" is a poorly described and under-diagnosed disease in which features of both rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE) occur in the same patient, usually one after the other.

SLE-related involvement is usually mild and dominated by hematologic abnormalities as well as skin, serosal and renal involvement. The natural history of rhupus arthritis follows an RA-like pattern and can lead to typical inflammatory erosions, deformities and disability. Although there is no consensus on the definition of rhupus and its place in the spectrum of autoimmunity, an increasing number of studies suggest a true overlap between RA and SLE.

The inclusion criteria used in the literature of the last four decades are heterogeneous, which makes it difficult to analyze the already rare cohorts and case reports. Due to this heterogeneity and the rarity of the disease, the prevalence, pathophysiology and natural history as well as the radiologic and immunologic profiles of Rhupus are poorly characterized. Moreover, in the absence of a validated therapeutic strategy, treatment is based on the experience of physicians and the results of a few studies.

The prognosis is further complicated by neurological and hematological complications, which drastically worsen patients' perception of their quality of life in relation to their health.

1. C.A. Brand et al. (1992) Coexistent rheumatoid arthritis and systemic lupus erythematosus: clinical, serological, and phenotypic features. Ann Rheum Dis 51(2):173-176
2. L. Andrade-Ortega et al. (2013) Efficacy and tolerability of rituximab in patients with rhupus Reumatol Clin 9(4):201-205
3. Tani C et al. (2013) Rhupus syndrome: assessment of its prevalence and its clinical and instrumental characteristics in a prospective cohort of 103 SLE patients. Autoimmune Rev 12:537-541
4. Upadhyaya S et al. (2022) Rhupus Syndrome: A Diagnostic Dilemma. Cureus. 2022 Sep 11;14(9):e29018.