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Rhupus, in the broad sense, refers to an overlap between rheumatoid arthritis (RA) and lupus. However, there is a paucity of data on the appropriate diagnostic/classification criteria that should be used to define rhupus. Hence, we undertook this narrative review to analyze the clinical characteristics, radiology, and treatment with a focus on diagnostic challenges and defining features of rhupus. The databases of Medline/PubMed, Scopus, and DOAJ were searched for relevant articles using the following keywords: (“Rhupus”), (“lupus” AND “erosive” AND “arthritis”), and (“lupus” AND “rheumatoid arthritis” AND “overlap”). Studies have used a variety of classification criteria for rhupus of which a combination of the latest classification criteria for RA and lupus along with positive anti-cyclic citrullinated peptide, anti-Smith, and anti-dsDNA antibodies seem most relevant. The majority of rhupus cohorts report the onset of the disease as RA (two-thirds of rhupus patients) followed by the development of features of lupus at an average interval of 3–11.3 years. The radiographic features and distribution of erosions are similar to RA. However, ultrasonography and MRI reveal erosions in pure lupus related arthritis as well. This makes the reliability of radiologic tools for the evaluation of rhupus supportive at the most. Extra-articular features in rhupus are mild with major organ involvement in the form of neuropsychiatric lupus and lupus nephritis being rare. We have further discussed the fallacies of the various classification criteria and proposed a theme for classifying rhupus which needs to be tested and validated in future studies. Our current state of understanding supports rhupus as an overlap of SLE and RA with articular disease similar to RA with the extra-articular disease being milder than SLE. Developing standardized classification criteria for rhupus will help in the early diagnosis and prevention of articular damage in patients with rhupus.

Background though considered to have characteristics of systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA) theoretically, Rhupus Syndrome (RS) owns its unique clinical features. In this retrospective cross-sectional study, we included 41 newly diagnosed Rhupus syndrome patients (NRSP). 160 new-diagnosed systemic lupus erythematosus patients (NSLEP) and 709 new-diagnosed rheumatoid arthritis patients (NRAP) were admitted as positive control groups. The clinical, serological, and radiological features among groups were compared, the disease activity of RA and SLE was evaluated, and the proportion of lymphocyte subsets in NRSPs under similar disease activity levels was explored. The initial onset age of RS patients was significantly younger than RA patients ( P  = 0.032), older than SLE patients ( P  = 0.008). 19.5% (8/41) RS patients initially presented with SLE symptom, while 12.2% RS patients presented with symptoms of both diseases. Hematopoietic dysfunction was the most prominent systemic manifestation in RS (61.0%, 25/41), only 9.8% patients experienced renal damage, and neurological disease were even rarer. Moreover, RS exhibited immunological characteristics different from NRAPs and NSLEPs, mainly manifested in decreased CD4 + T cell and NK cell counts, increased ratio of CD8 + T (%) and total B cells, and decreased ratio of NK cells. RS is characterized by a higher incidence of interstitial lung disease and significant hyperglobulinemia besides the typical clinical characteristics of RA and SLE, which may be associated with a re-imbalanced lymphocyte subset. Evaluation of disease activity of RS cannot only rely on either SLEDAI-2 K or DAS28-ESR/-CRP, but more comprehensive assessment tools.

Rhupus syndrome, an overlap of rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE), is a rare condition characterized by heterogeneous clinical manifestations and difficult management. Conventional therapies, including glucocorticoids, disease-modifying anti-rheumatic drugs (DMARDs), immunosuppressants, and biologics, are often limited by drug intolerance and steroid-related complications. Molecular hydrogen (H ) has emerged as a potential adjuvant therapy due to its antioxidant and immunomodulatory properties. This report aimed to evaluate the clinical efficacy of H as an adjunct to rituximab in a patient with refractory Rhupus and multiple drug intolerances. We report a 47-year-old male with a 27-year history of rheumatoid arthritis, who subsequently developed systemic lupus erythematosus, fulfilling criteria for Rhupus syndrome. His disease course was complicated by multiple drug intolerances, including adverse reactions to methotrexate, sulfasalazine, hydroxychloroquine, azathioprine, leflunomide, and rituximab at standard dosing. Despite reduced-dose rituximab and mycophenolic acid, disease activity persisted and lupus nephritis was confirmed. In October 2023, oral molecular hydrogen capsule therapy was initiated as adjuvant treatment, resulting in significant clinical improvement, which was paralleled by characteristic changes in T- and B-cell subsets, normalization of anti-dsDNA antibody, erythrocyte sedimentation rate (ESR), and complement levels, and successful discontinuation of prednisone. Hydrogen therapy was well tolerated without major adverse effects. The patient developed avascular necrosis from prior corticosteroid use but recovered well after hip arthroplasty in January 2025. Disease control was sustained with mycophenolic acid and continued hydrogen therapy. This case suggests molecular hydrogen therapy as a potential adjuvant for refractory Rhupus with multiple drug intolerances, showing immune modulation, reduced inflammation, steroid withdrawal, and sustained control with low-dose rituximab. Further studies are needed to confirm its efficacy and standardize its use.

Overlap between rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE) (\"rhupus\") is recognized, but coexistence with a severe eosinophilic asthma syndrome is exceptionally rare. We describe a triple autoimmune overlap of RA, SLE, and hypereosinophilic asthma with systemic manifestations (HASM), initially managed as ANCA-negative eosinophilic granulomatosis with polyangiitis (EGPA) and subsequently re-classified in light of evolving concepts. A 44-year-old woman with a 10-year history of seropositive RA developed alopecia, Coombs-positive hemolytic anemia, hypocomplementemia, and ANA and anti-Sm positivity, fulfilling SLE criteria. While receiving prednisone, hydroxychloroquine and conventional DMARDs, she subsequently developed adult-onset asthma, chronic rhinosinusitis with nasal polyps, and marked hypereosinophilia (>3.5×10 /L). Secondary causes were excluded; bone marrow showed reactive eosinophilia and ANCA (indirect immunofluorescence and ELISA for MPO/PR3) remained negative. She was diagnosed and treated as ANCA-negative EGPA with high-dose glucocorticoids plus methotrexate and hydroxychloroquine, leading to rapid normalization of eosinophils and durable remission of asthma and sinus disease. In retrospect, and according to the ERS/GERM'O'P proposal, this eosinophilic disorder is best classified as HASM within the EGPA-hypereosinophilic spectrum because ANCA and biopsy-proven vasculitis were absent. The case illustrates the evolving boundary between EGPA and hypereosinophilic syndromes and extends the concept of rhupus to include an EGPA-spectrum eosinophilic asthma syndrome. New-onset eosinophilic asthma in patients with established rheumatic disease should prompt evaluation for EGPA-spectrum or hypereosinophilic disorders. Even when the final label is HASM rather than definite EGPA, timely institution of EGPA-type immunosuppression may avert organ damage.

Musculoskeletal involvement is one of the most common manifestations of systemic lupus erythematosus (SLE), with a negative impact on both quality of life and overall prognosis. SLE arthritis can be classified into three different subtypes, with different prevalence and characteristic biomarkers and MRI findings. Identifying the pathogenetic mechanisms underlying musculoskeletal manifestations’ development is crucial to develop therapeutic strategies to suppress synovial inflammation, prevent erosions and deformities, and improve SLE patients’ quality of life. Hence, here we discuss the main pathogenetic mechanisms and therapeutic approaches of musculoskeletal manifestations of SLE from the 2022 International GISEA/OEG Symposium.

Background The aim was to determine the accuracy of high-resolution ultrasonography (US) for detecting erosion in the metacarpophalangeal (MCP) and wrist joints of patients with different subtypes of systemic lupus erythematosus (SLE) arthritis, using computed tomography (CT) as the gold-standard reference method. Method The ulnar head, radiocarpal and second to fifth MCP joints in 26 patients with SLE - 9 classified as having rhupus syndrome, 10 as having Jaccoud’s arthropathy (JA) and 7 as having non-deforming non-erosive (NDNE) arthritis - were subdivided into areas and bilaterally evaluated for the presence of bone erosion by CT and US. On CT, erosion volume was scored according to the outcome measures in rheumatology-rheumatoid arthritis magnetic resonance imaging (OMERACT-RAMRIS) score. On US, erosions were semi-quantitatively scored 0–3 according to scoring by ultrasound structural erosion (ScUSSe) systems. Results Erosions were detected by CT in 92/728 areas (12.6 %) and by US in 43/728 areas (5.9 %). Sensitivity, specificity and accuracy of US overall was 36 %, 98 % and 90 % compared with 57 %, 98 % and 93 % in the dorsal and lateral aspects of the second and fifth MCP, which were identified as areas with the best US reliability. Adding wrist joints would capture a larger number of erosions without affecting the accuracy. US detected 90.0 % of CT erosions with bone volume loss >20 % and 51.2 % of erosions with bone volume loss >10 %. Patients with rhupus had a greater number of larger erosions than those with JA or NDNE arthritis, with prevalent involvement of the MCP joints. Overall reliability of US in detecting bone erosions was moderate for rhupus syndrome (0.55) and JA (0.58), but poor for NDNE arthritis (0.10). Conclusion US had moderate sensitivity and excellent specificity for detection and semi-quantitative assessment of bone erosions in SLE.

Autoimmune connective tissue diseases are a group of immune disorders, characterized by different clinical features, which affects not only the skin but also different organs and systems. Such diseases include: rheumatoid arthritis, systemic lupus erythematosus, Sjögren’s syndrome, systemic sclerosis, dermatomyositis and polymyositis. However, there are patients that fulfil the diagnostic criteria of more than one disorder, thus supporting the concept of the so called “overlap syndromes”. The aim of this review is to present the history, clinical and immunologic hallmarks of these overlap syndromes. Such conditions are scleromyositis, lupus erythematosus/lichen planus overlap, Sharp’s syndrome, Rhupus syndrome, Rowell’s syndrome, Reynolds syndrome and Senear-Usher syndromes. Patients with these syndromes usually do not meet most of the diagnostic criteria of “classic” connective tissue diseases and this usually causes diagnostic difficulties. Overlap syndromes are commonly treated with corticosteroids, hydroxychloroquine and immunosuppressant drugs as a first-line treatment. The new therapeutic molecules that precisely interact with immune mechanisms will require accurate diagnosis and a better understanding of the pathogenesis of the overlap syndromes.

Systemic lupus erythematosus (SLE) is an autoimmune disease involving multiple organs and organ systems. Musculoskeletal (MSK) involvement is one of the most frequent and the earliest locations of disease. This disease affects joints and periarticular soft tissues, tendon sheaths and tendons, bones, and muscles. Multimodality imaging, including radiography, ultrasound (US), and magnetic resonance imaging (MRI), plays a significant role in the initial evaluation and treatment follow up of MSK manifestations of the SLE. In this paper, we illustrate MSK imaging features in three clinical forms of SLE, including nondeforming nonerosive arthritis, deforming nonerosive arthropathy, and erosive arthropathy, as well as the other complications and features of SLE within the MSK system in adults and juveniles. Advances in imaging are included. Conventional radiography primarily shows late skeletal lesions, whereas the US and MRI are valuable in the diagnosis of the early inflammatory changes of the soft tissues and bone marrow, as well as late skeletal manifestations. In nondeforming nonerosive arthritis, US and MRI show effusions, synovial and/or tenosynovial hypertrophy, and vascularity, whereas radiographs are normal. Deforming arthritis clinically resembles that observed in rheumatoid arthritis, but it is reversible, and US and MRI show features of inflammation of periarticular soft tissues (capsule, ligaments, and tendons) without the pannus and destruction classically observed in RA. Erosions are rarely seen, and this form of disease is called rhupus syndrome.

CD5 + primary hepatosplenic large B-cell lymphoma (LBCL) is an infrequent disease (Shi et al. in J Int Med Res 48(8):1220737725, 2020; Yamaguchi et al. in Blood 99(3):815–821, 2002; Durani et al. in Leuk Lymphoma 62(13):3078–3086, 2021). Rhupus syndrome is defined as an overlap between systemic lupus erythematosus and rheumatoid arthritis. Here, we presented a rare case of CD5-positive primary hepatosplenic LBCL with rhupus syndrome. The 58-year-old female achieved complete remission after 6 cycles of R-COP and 2 cycles of R-CHOP. Besides, we reviewed case reports of CD5 + primary hepatosplenic LBCL and speculated the connections between rhupus syndrome and lymphomas.

Coronary artery aneurysms (CAA) are an infrequent cause of coronary artery disease in both systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA), most occurring as a result of acute coronary syndromes (ACS). Until now, no cases of CAA have been described in a patient with rhupus syndrome (RhS). Differentiating whether CAA stem from primary vasculitis, atherosclerosis, or a combination of both continues to pose a significant challenge. We present the first described clinical case of a 43-year-old patient with RhS and multiple CAA identified by the presentation of an acute myocardial infarction. The presence of multiple cardiovascular risk factors and the absence of inflammatory findings, both in PET-CT and arterial biopsy, favored an atherosclerotic versus a vasculitic etiology of the CAA. At the time of the aneurysms diagnosis, the patient showed no signs of SLE activity and only moderate RA activity, which underscores the importance of screening for silent coronary aneurysms in these patients, even in subjects exhibiting little apparent activity from their underlying disease. This case also exemplifies the severe impact of atherosclerotic burdens on such patients, demanding vigilance and aggressiveness in its prevention, early diagnosis, and treatment. We hypothesize that RhS could engender an even greater risk of presenting CAA than either SLE or RA on their own, which therefore warrants more careful follow-up in these patients.