Triple-positive antiphospholipid syndrome (APS) represents a high-risk serologic phenotype associated with recurrent thrombosis and systemic lupus erythematosus (SLE). Direct oral anticoagulants (DOACs), particularly rivaroxaban, have demonstrated inferior outcomes compared with vitamin K antagonists in this population. We report a case of a patient with long-standing SLE and persistently triple-positive APS who developed Libman–Sacks endocarditis following a switch from warfarin to rivaroxaban. The patient had biopsy-proven class V lupus nephritis and had remained clinically stable on long-term warfarin therapy for several years. Transthoracic echocardiography demonstrated underlying rheumatic mitral valve disease with newly developed mobile vegetations measuring 10-15 mm. Blood cultures obtained prior to antibiotic exposure were negative, and inflammatory markers were not suggestive of infection. Rivaroxaban was discontinued, and warfarin was reinstated with bridging therapy. Prednisolone (40 mg daily) was initiated, and follow-up echocardiography demonstrated a reduction in vegetation size with clinical improvement. This case highlights a temporal association between rivaroxaban use and valvular thrombotic lesions in high-risk APS. Improvement was likely multifactorial, reflecting combined effects of anticoagulation, escalation of immunosuppression, and underlying valvular disease. These findings support current recommendations favoring vitamin K antagonists over DOACs in patients with triple-positive APS. Cite this article as: Saleh I, Salman S, Alkady A, Saqabi FA. Libman–Sacks endocarditis in triple-positive antiphospholipid syndrome following switch from warfarin to rivaroxaban: improvement after reinstitution of warfarin and escalation of immunosuppression. Eur J Rheumatol. 2026, 13(2), 0009, doi: 10.5152/ eurjrheum.2026.26009.
Sporadic late-onset nemaline myopathy (SLONM) is a rare muscle disorder that can be treated or may pose a potential threat to life. It usually manifests in the later stages of life and is characterized by the accumulation of nemaline rods within muscle fibers. The pathophysiology of this disease is still not fully understood, with some evidence suggesting that autoimmune responses and hematological neoplasia may be involved. Herein, a case of SLONM associated with systemic lupus erythematosus is presented, which showed a favorable response to immunotherapy. Cite this article as: Cui R, Luan X, Dai S. Nemaline myopathy in systemic lupus erythematosus. Eur J Rheumatol. 2026, 13(2), 0048, doi:10.5152/eurjrheum.2026.25045.
A 74-year-old patient initially presented with rapid onset of bilateral leg swelling, leukocytosis (monocytosis and eosinophilia), hand arthritis, elevated inflammatory markers, and 20% weight loss. Extensive workup, including a bone marrow biopsy, did not show evidence of malignancy. The patient was evaluated many months after initiation of symptoms and found to have deep hardening skin and multiple joint contractures. Pictures of his forearms are shown above. Cite this article as: Combarro AS, Guma J, Jimenez SA, Mendoza FA. The groove sign. Eur J Rheumatol. 2026, 13(2), 0079, doi: 10.5152/eurjrheum.2026.25079.
Large vessel vasculitis (LVV) refers to vasculitis predominantly affecting large arteries, including the aorta and its major branches. It encompasses conditions such as giant cell arteritis and Takayasu arteritis, as well as unclassified or idiopathic forms. Imaging techniques, including computed tomography (CT), are essential for detecting large vessel involvement, particularly when biopsy findings are negative. We report the case of a 56-year-old man admitted with fever, headache, and upper abdominal pain lasting 2 weeks. On examination, he had scalp tenderness and upper abdominal tenderness without temporal artery enlargement. Laboratory tests revealed elevated C-reactive protein (CRP: 19.75 mg/dL) and normal IgG4 (45 mg/dL). Initial CT demonstrated increased attenuation of mesenteric fat (Figure 1), suggestive of misty mesentery. Antibiotic therapy for presumed infectious mesenteritis was ineffective, and CRP increased further to 30.20 mg/dL. Contrast-enhanced CT showed thickening of the aortic wall (Figure 2). Based on fever, headache, systemic inflammation, and aortic wall thickening, LVV was considered the most plausible diagnosis. Cite this article as: Noda S, Kondo S. Misty mesentery as an initial imaging finding suggestive of large vessel vasculitis. Eur J Rheumatol. 13(1), 0084, doi: 10.5152/eurjrheum.2026.25084.
Objective: Skeletal tuberculosis (TB) arthritis is an uncommon but significant cause of osteoarticular morbidity, representing less than 1% of all TB cases. Delayed diagnosis often leads to persistent joint stiffness and pain. This study aims to identify early magnetic resonance imaging (MRI) features that distinguish TB arthritis from rheumatoid arthritis (RA). Methods: The MRI scans were retrospectively analyzed from 2010 to 2017 of 21 patients diagnosed with TB arthritis and 82 patients with RA. Imaging characteristics including synovial effusion, synovial membrane thickness, abscess wall thickness, bony erosions, rice body formation, tenosynovitis, adjacent soft tissue inflammation, and joint space narrowing were compared between the 2 groups. Results: Compared to RA, TB arthritis patients demonstrated significantly greater abscess wall thickening (P = .001) and rice body formation (P = .008), alongside less pronounced joint space narrowing. Conclusion: In patients presenting with chronic arthritis, the presence of thickened abscess walls, rice bodies, and relatively preserved joint space on MRI serve as early diagnostic markers favoring TB arthritis over RA. Cite this article as: Ting S, Chen J, Yu S, Hsu C, Chen Y. MRI characteristics distinguishing tuberculous arthritis from rheumatoid arthritis: A comparative study. Eur J Rheumatol. 2026, 13(2), 0058, doi: 10.5152/eurjrheum.2026.25058.
Emergency physicians routinely treat acute arthritis with anti-inflammatory medications and discharge patients with the diagnosis of gout. However, when patients return days later with worsening symptoms, the probability of it involving calcium pyrophosphate crystals requiring completely different management prevails. Crystal misdiagnosis has its consequences. Research shows up to 40% of patients diagnosed with gout actually have pseudogout (calcium pyrophosphate crystal arthropathy) when properly analyzed.1 This misidentification leads to treatment failure in over 60% of cases, with patients receiving colchicine for presumed gout when they actually need anti-inflammatory therapy for calcium pyrophosphate arthropathy.
Primary biliary cholangitis (PBC) and immune-mediated necrotizing myositis (IMNM) are both rare autoimmune disorders, and their coexistence is uncommon. Low back pain (LBP) as the initial presentation of this overlap is extremely rare and may delay diagnosis. A case is presented of a 46-year-old woman with recurrent LBP and mild proximal muscle weakness, along with laboratory findings of elevated liver and muscle enzymes, positive anti-mitochondrial antibody subtype M2 (AMA-M2), anti-GP210, and anti-SSA antibodies, and negative myositis-specific antibodies. Imaging revealed paraspinal myositis, and histopathology confirmed PBC (stage 1) and IMNM. The patient responded well to a combination of ursodeoxycholic acid, corticosteroids, hydroxychloroquine, and mycophenolate mofetil, achieving normalization of laboratory parameters and complete symptom resolution after 15 months. This case underscores the need for clinicians to consider systemic autoimmune diseases in patients with atypical LBP and highlights the potential for AMA-M2-positive myositis to involve the paraspinal muscles, providing insight into a rare manifestation of PBC-IMNM overlap. Cite this article as: Zhu L, Yan T, Ye Q. Primary biliary cholangitis presenting with low back pain and complicated by immune-mediated necrotizing myositis: A case report. Eur J Rheumatol. 2026, 13(1), 0050, doi:10.5152/eurjrheum.2026.25050.
A 49-year-old female presented with redness and scaling of scalp of 2 years duration. It was associated with photosensitivity and difficulty getting up from squatting position since 4 months. Written informed consent was obtained from the patient who agreed to take part in the study. Clinical examination revealed diffuse uniform erythema affecting scalp and its margins at forehead, temples and posterior aspect of neck. Cite this article as: Das P., Bhatnagar A, Sharma S, et al. Seborrheic dermatitis-like presentation of dermatomyositis. Eur J Rheumatol. 13(1), 0009, doi: 10.5152/eurjrheum.2026.25009.
Objective: Systemic-onset juvenile idiopathic arthritis (sJIA) follows a variable and unpredictable course, with long-term outcomes remaining inconsistent worldwide despite therapeutic advancements. This study aimed to evaluate the disease course and clinical outcomes of the sJIA cohort after 5-year follow-up. Methods: The study retrospectively analyzed 100 sJIA patients with at least 5 years of follow-up. Ten patients were excluded due to insufficient data. Data on demographics, clinical features, treatments, outcomes, and complications were collected. Results: The mean age at disease onset was 6.75 ± 3.64 years, with a median follow-up of 7 years. Common clinical manifestations included fever (100%), arthritis (96%), rash (55%), and hepatosplenomegaly (32%). Polyarticular arthritis was observed in 70.83% of cases. Treatment modalities included Non-steroidal anti-inflammatory drugs (100%), steroids (89%), methotrexate (86%), thalidomide (31%), lenalidomide (21%), and tocilizumab (13%). While 11% of patients responded to NSAIDs alone,46% achieved remission with glucocorticoids and methotrexate. A total of 46% of the patients exhibited a monocyclic disease course, and 27% of the patients had polycyclic and persistent courses. On multivariate analysis, diagnostic delay (odds ration (OR) = 1.02, 95% CI: 1.01-1.03) and symmetric arthritis (OR = 2.36, 95% CI: 1.65-3.32) were identified as key predictors of persistent disease. Common complications included infections (20%), joint damage (14%), and macrophage activation syndrome (11%). At 5 years, 62% achieved drug-free remission, 16% remained in remission on medication, and 21% had active disease. Mortality was noted in 1 patient due to a suspected cerebrovascular accident or meningitis. Conclusion: Delayed diagnosis and symmetric arthritis at onset increased the risk of persistent disease, highlighting the need for early and aggressive treatment for better outcomes. Cite this article as: Rao AP, B A, Kumar A, et al. Five-year outcomes of systemic-onset juvenile idiopathic arthritis in India: insights into disease course and predictive factors. Eur J Rheumatol. 2026, 13(1), 0068, doi: 10.5152/eurjrheum.2026.25068
Eosinophilic Granulomatosis with Polyangiitis (EGPA) is a rare Anti-neutrophil cytoplasm antibodies (ANCA)-associated vasculitis with multi-organ involvement and eosinophilia. We present a 61-year-old male with a history of eosinophilic asthma who developed progressive weakness and muscle aches six weeks after his second COVID-19 booster. Four to six weeks post vaccination, he developed myal gias and weakness, especially in proximal muscles, leading to a severe decline in function. Lab results showed leukocytosis (29.54 cells/mm³); 54% eosinophils; and elevated erythrocyte sedimentation rate (ESR), C-reactive protein, and creatine kinase levels at 13 736 u/L. Anti-myeloperoxidase antibodies were positive, while PR-3, C-ANCA, and P-ANCA were negative. Magnetic resonance imaging of the lower extremities showed intramuscular edema. Muscle biopsies confirmed EGPA. Diagnosed per American College of Rheumatology (ACR)/ European Alliance of Associations for Rheumatology (EULAR) criteria, he received pulse dose IV methylprednisolone with significant improvement. He was discharged on 60 mg prednisone and started on mepolizumab (300 mg subcutaneous once every 4 weeks). Eosinophilic granulomatosis with polyangiitis progresses through stages: asthma, eosinophilia with organ involvement, and vasculitis. This case highlights a unique presentation of rapid myositis without significant involvement of any other organs and vasculitis post-mRNA vaccination. While environmental factors may trigger EGPA, the role of COVID-19 vaccines remains hypothesis-generating. This case underscores the importance of post-market surveillance for rare events, contributing to the understanding of vaccine-related rheumatic disease triggers Cite this article as: Memdani A, Busa V, Avula S, Ford A, Nesheiwat J. Unmasking Eosinophilic granulomatosis with polyangiitis: a case of rapid-onset myositis following COVID-19 booster in an eosinophilic asthma patient. Eur J Rheumatol. 2025, 13(1), 0016, doi:10.5152/eurjrheum.2026.25016.
Avacopan, a complement component 5a (C5a) receptor antagonist, is an emerging therapeutic agent for anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis. However, its intrinsic efficacy remains unclear. A 74-year-old woman with granulomatosis with polyangiitis (GPA) presenting as a pulmonary mass, sinusitis, otitis media with hearing loss, and mononeuritis multiplex is reported. Initial remission was achieved with prednisolone and cyclophosphamide followed by maintenance with low-dose prednisolone and azathioprine. After 3 years, she relapsed with recurrent blood-stained sputum and enlargement of the pulmonary lesion despite negative ANCA titers. Infection and malignancy were excluded. Avacopan (60 mg/day) was introduced with low-dose prednisolone and azathioprine. Over 12 months, the pulmonary mass regressed, and blood-stained sputum improved, enabling complete withdrawal of prednisolone after 20 months. This case suggests a potential direct effect of avacopan on pulmonary inflammatory lesions in GPA and highlights the importance of long-term treatment evaluation. Cite this article as: Nakagomi D, Kubota S, Kobayashi Y, Hanai S. Regression of a refractory pulmonary lesion in granulomatosis with polyangiitis following the addition of avacopan. Eur J Rheumatol. 2026, 13, 0062, doi:10.5152/eurjrheum.2026.25062.
Emerging evidence suggests that somatic mutations in genes associated with innate immunity can trigger adult-onset autoinflammatory diseases. Notably, loss-of-function variants in the TET2 gene have been linked to both hematological malignancies and immune-mediated disorders. A case is presented of a 73-year-old woman with chronic myelomonocytic leukemia who developed severe pericardial effusion secondary to inflammatory serositis, associated with a pathogenic TET2 variant. Despite initial treatment with corticosteroids and diuretics, her condition worsened, which led to the need to implement treatment with colchicine and anakinra. This regimen led to significant clinical improvement and resolution of the effusion. This case reflects the importance of searching for pathogenic variants in TET2 in patients with hematological disorders, with the aim of early recognition of inflammatory manifestations associated with this genetic alteration. Treatment with colchicine and anti-interleukin-1 should be considered in these cases, as they are effective and avoid the unnecessary use of other immunosuppressants. Cite this article as: Porto Fuentes Ó, de Paz Arias R, Álvarez Troncoso J. Pathogenic TET2 variants and autoinflammatory manifestations in myeloid hematologic malignancies: case report and literature review. Eur J Rheumatol. 2026, 13(1), 0119, doi:10.5152/eurjrheum.2026.24119.
Omalizumab is an anti-immunoglobulin E (IgE) monoclonal antibody used to treat severe allergic asthma. Although most of the reported cases of omalizumab-associated eosinophilic granulomatous polyangiitis (EGPA) are attributed to the accompanying glucocorticoid reduction, a patient who met the 2022 American College of Rheumatology diagnostic criteria for EGPA yet had no history of systemic glucocorticoid treatment is described. This individual developed limb numbness accompanied by eosinophilia (14.24 × 109/L) within 22 days after initiating omalizumab therapy. Critical warning: In asthmatic patients treated with omalizumab, if persistent eosinophilia or new neurological symptoms occur, the possibility of EGPA should be highly vigilant and evaluated. Cite this article as: Wang Z, Lin S, Yang J, Cheng M, Cai M. Eosinophilic granulomatous polyangiitis emerging after omalizumab administration: an asthma patient without systemic corticosteroids develops mononeuritis multiplex after 22 days. Eur J Rheumatol. 2026, 13(1), 0048, doi: 10.5152/eurjrheum.2026.25048.
Rowell syndrome is a distinct subtype of systemic lupus erythematosus presenting with features of lupus as well as erythema multiforme. It has been well characterized by a different set of systemic signs and symptoms and a unique serological profile. Lupus podocytopathy (LP) is a rare pattern of lupus nephritis with a slightly different clinical and serological profile, natural course of the disease, as well as prognosis. A case of Rowell syndrome is presented, which was later diagnosed with LP on the basis of global effacement of podocytes on electron microscopy Cite this article as: Gera V, Kashif AW, Koshy V, et al. Rowell syndrome with lupus podocytopathy: A diagnostic challenge. Eur J Rheumatol. 2026, 13(1), 0007, doi:10.5152/eurjrheum.2026.25007.
Objective: Limited information exists regarding the impact of axial spondyloarthritis (axSpA) on pregnancy compared to other chronic inflammatory disorders. Axial spondyloarthritis’s unique anatomical and inflammatory impact on pregnancy presents challenges. This study aims to assess axSpA’s progression during pregnancy. Methods: A retrospective observational study at a private rheumatology clinic assessed axSpA’s progression during pregnancy in 80 women previously diagnosed with the condition. Results: Onset of axSpA occurred at an average age of 23.2 years. Symptoms worsened in 29 (36.25%) patients during pregnancy, while 51 (63.7%) experienced improvement or remission. Twelve (15%) women faced conception difficulties, with a delay of over a year. Cesarean sections were performed in 42 (62.6%) cases, while 25 (37.3%) had full-term vaginal deliveries. Conclusion: During pregnancy, there is a noticeable tendency for alterations in disease activity and symptoms experienced. This study highlights the need for better understanding and awareness of axSpA’s impact on pregnancy outcomes. Cite this article as: Gore S, Tambe R, Raut A, Patil P. Disease course of axial spondyloarthritis in pregnancy. Eur J Rheumatol. 2026, 13, 0107, doi:10.5152/eurjrheum.2026.24107.
Cite this article as: Shao Q. Simulated rheumatoid arthritis induced by immune checkpoint inhibitor. Eur J Rheumatol.2025, 12(4), 0067, doi:10.5152/ eurjrheum.2025.24067.
Methotrexate (MTX) remains a cornerstone in the management of rheumatoid arthritis (RA). Extensive basic pharmacological research has consistently demonstrated the e cacy of MTX in treating RA, while there is still insu cient understanding about the potential mechanisms. In this review, current knowledge on the mechanisms by which MTX acts in bone erosion and synovitis is summarized, and MTX-based combination therapies are discussed. Additionally, the bone-protective and anti-in%am matory roles of adenosine receptors are described, and biomarkers associated with MTX responders in RA are explored. This review sheds new light on future research directions for RA, as well as o ers evidence-based recommendations to enhance the clinical utilization of MTX in RA treatment. Cite this article as: Teng Y, Jiang L, Yin H, Deng G. The role and mechanistic e ects of methotrexate in the treatment of rheumatoid arthritis. Eur J Rheumatol. 2025:12(4), -0086, 10.5152/ eurjrheum.2025.24086.
Cite this article as: Prieto-González S, Marco-Hernández J, Jordà-Sánchez R, et al. Is there still a place for computed tomography angiography in assessing inflammatory large-vessel involvement in large vessel vasculitis? comment of the article of nakagomi et al.. Eur J Rheumatol. 2025, 12(4), 0041, doi: 10.5152/ eurjrheum.2025.25041.
Catastrophic antiphospholipid syndrome (CAPS), also known as Asherson’s syndrome, is a rare and life-threatening condition that represents the most severe clinical manifestation of antiphospho lipid syndrome. Due to its rapid and aggressive course, CAPS is characterized by the development of widespread micro- and macrothrombosis, which results in multi-organ ischemia and failure. Antiphospholipid syndrome, an autoimmune disorder, is characterized by thrombotic and/or obstet ric events, accompanied by persistent antiphospholipid antibodies. The case of a 36-year-old woman was described, who initially presented with low-effort dyspnea and abdominal pain, with subsequent imaging revealing pulmonary embolism and aortic thrombosis, alongside acute ischemic cerebellar infarcts. The eventual confirmation of a positive lupus anticoagulant solidified the diagnosis of CAPS. This case underscores the diagnostic challenge posed by CAPS, particularly when presenting with prominent pulmonary embolism, and highlights the critical need for prompt recognition and mul tidisciplinary management. Furthermore, the patient’s positive outcome demonstrates the potential for recovery even in such severe presentations. Cite this article as: Maradiaga FA, Diaz VS, Rubio MA, Arias JP, Alas-Pineda C. A case of catastrophic antiphospholipid syndrome presenting as pulmonary embolism and renal thrombosis: a case report and literature review. Eur J Rheumatol. 2025, 12(4), 0018, doi:10.5152/eurjrheum.2025.25018.
Immunoglobulin G4–related disease (IgG4-RD) is an immune-mediated fibroinflammatory disorder characterized by mass forming lesions that can result in irreversible organ damage and mortality if ignored. Mixed connective tissue disease (MCTD) is characterized as an overlap of systemic lupus erythematosus (SLE), systemic sclerosis, inflammatory myopathy, and rheumatoid arthritis (RA). While cases of IgG4-RD linked to SLE and RA have been documented, to the authors’ knowledge, there has not been a recorded instance of a patient with IgG4-RD related with MCTD. A patient diagnosed with both MCTD and IgG4-RD is presented. Cite this article as: Kocaayan H, Ediboglu ED, Aydin I, Erdogan N, Akar S. Coincidence of mixed connective tissue disease with immunoglobulin –related disease: A case report. Eur J Rheumatol. 2025, 12 (4), 0043, doi: 10.5152/ eurjrheum.2025.25043.
