{"title":"Anterior ischaemic optic neuropathy as an initial manifestation of eosinophilic granulomatosis with polyangiitis: The importance of early and appropriate immunosuppressive treatment for visual recovery.","authors":"Tomoyuki Mutoh, Yusuke Tomiyama, Eiko Yamamori, Hinano Anaba, Masahiro Tsuboi, Hiroshi Fujii","doi":"10.1093/mrcr/rxag076","DOIUrl":"10.1093/mrcr/rxag076","url":null,"abstract":"<p><p>Eosinophilic granulomatosis with polyangiitis (EGPA) is an antineutrophil cytoplasmic antibody-associated vasculitis, affecting various organs. Although ocular involvement occurred in 6.8-11.1% of patients, anterior ischaemic optic neuropathy (AION) is rare. We present the case of a 57-year-old woman with an 11-year history of asthma who developed sudden, painless visual loss in her left eye. On admission (3 days after symptom onset), visual acuity was counting fingers in the left eye and 20/20 in the right eye. Funduscopy showed left optic disc oedema. Magnetic resonance imaging revealed intravitreal protrusion of the left optic nerve head with restricted diffusion and local contrast enhancement of the left intraorbital and retrobulbar fat. Given also peripheral eosinophilia, myeloperoxidase-antineutrophil cytoplasmic antibody positivity, mononeuritis multiplex, nasal polyps, sinusitis, and pulmonary involvement, EGPA with AION was diagnosed. Intravenous methylprednisolone pulse was urgently administered, followed by mepolizumab 2 weeks later. Although eosinophilic inflammation and imaging findings improved, visual acuity remained unchanged over 1 year. A literature review identified 14 cases of EGPA-associated AION. Including our case, 11 cases with available data (14 eyes) were analysed. Overall, visual recovery was observed in 29%. Among 12 eyes with severe visual impairment before treatment, visual recovery occurred only in those treated earlier or with cyclophosphamide except for one, whereas no recovery occurred in those with delayed treatment or without cyclophosphamide. This case highlights the potential of AION as an initial manifestation of EGPA. Both earlier initiation and adequate intensity of immunosuppressive therapy, particularly including cyclophosphamide when clinically appropriate, may be important for visual recovery in EGPA-associated severe AION.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-06-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148681641","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Kota Hyakuna, Shigeru Iwata, Kodai Ueno, Taro Nishikawa, Ryo Matsumiya, Ran Nakashima, Kanako Sagan, Fumiyoshi Kojima, Kohei Hori, Jyunko Taruya, Shoko Yorozu, Takao Fujii
{"title":"Polymyositis with scleroderma-like features and an anti-PM-Scl75 antibody detected by line blot but not by protein array or immunoprecipitation: A case report and literature review.","authors":"Kota Hyakuna, Shigeru Iwata, Kodai Ueno, Taro Nishikawa, Ryo Matsumiya, Ran Nakashima, Kanako Sagan, Fumiyoshi Kojima, Kohei Hori, Jyunko Taruya, Shoko Yorozu, Takao Fujii","doi":"10.1093/mrcr/rxag083","DOIUrl":"10.1093/mrcr/rxag083","url":null,"abstract":"<p><p>Anti-PM-Scl antibodies (Abs) are associated with systemic sclerosis (SSc)-myositis overlap syndrome. However, anti-PM-Scl75/100 reactivity detected by line blot (LB) assays is frequently not confirmed by protein array or immunoprecipitation, indicating that such results should be interpreted with caution in Japan. A 72-year-old woman developed limb fatigue and Raynaud's phenomenon, followed by muscle weakness, elevated serum creatine kinase levels, and gangrenous changes in several fingers. Magnetic resonance imaging and muscle biopsy findings were consistent with myositis. She tested positive for antinuclear Abs with a speckled pattern. LB testing showed strong anti-PM-Scl75 reactivity, and fingertip ulcers raised suspicion of SSc-spectrum manifestations. Treatment with prednisolone (25 mg/day) and high-dose intravenous immunoglobulin improved the muscle enzyme elevation and digital lesions. However, anti-PM-Scl75 Abs were not detected by either protein array or immunoprecipitation. The final clinical diagnosis was polymyositis with SSc-spectrum features. We therefore reviewed previously reported cases in which anti-PM-Scl Abs were detected by LB but were not confirmed by other assays. This case demonstrates that myositis with SSc-like clinical manifestations may occur even when anti-PM-Scl75 reactivity is detected only by LB assay. Although autoantibody testing may aid in the diagnosis of myositis, the results should be interpreted in light of the analytical characteristics of each assay. In particular, anti-PM-Scl75/100 reactivity detected by LB should be interpreted with caution when the antinuclear antibody staining pattern is not nucleolar.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-06-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148820803","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Clinical heterogeneity in paediatric patients with chronic nonbacterial osteomyelitis and chronic recurrent multifocal osteomyelitis.","authors":"Hiroki Shimomura, Kazuki Takahashi, Masahiro Ueki, Kazuma Saito, Haruna Yamahiro, Shinichi Ishikawa, Sho Kato, Akiko Ando, Takashi Suganuma, Makoto Mikawa, Tomonobu Sato","doi":"10.1093/mrcr/rxag049","DOIUrl":"10.1093/mrcr/rxag049","url":null,"abstract":"<p><p>Chronic recurrent multifocal osteomyelitis (CRMO) and chronic nonbacterial osteomyelitis (CNO) are autoinflammatory diseases characterised by sterile bone inflammation. Patients with CNO or CRMO often complain about pain in the extremities, although several unusual manifestations have been reported. We report four paediatric cases of CNO/CRMO, demonstrating marked clinical variability in symptom onset and disease course. All patients presented with bone pain during their disease course, whereas unusual manifestations such as fever of unknown origin prior to the bone pain and cervical pain were confirmed. The diagnosis was established based on clinical features and by ruling out malignancy and infection through biopsy and culture. All patients received nonsteroidal anti-inflammatory drugs as initial therapy, and one required additional treatment with oral methotrexate and adalimumab. Serum cytokine analysis was performed in three cases, revealing elevated levels of both pro- and anti-inflammatory cytokines, such as interleukin-6, interleukin-10, and interleukin-33 in the pretreatment state. These cases underscore the clinical heterogeneity of CNO/CRMO.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-06-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148310978","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Severe cardiac involvement in a young woman with mixed connective tissue disease complicated by macrophage activation syndrome.","authors":"Kosuke Muto, Michiru Nomoto, Yasuto Araki, Yu Miyama, Takahide Arai, Shintaro Nakano","doi":"10.1093/mrcr/rxag061","DOIUrl":"10.1093/mrcr/rxag061","url":null,"abstract":"<p><p>Reports of severe mixed connective tissue disease or macrophage activation syndrome-associated cardiac involvement are limited. A 37-year-old woman with mixed connective tissue disease was transferred to a local hospital with fever and headache. She was diagnosed with meningitis and treated with glucocorticoid pulse therapy (methylprednisolone 1000 mg/day for 3 days), followed by glucocorticoid tapering. Despite a normal left ventricular ejection fraction on admission, her left ventricular ejection fraction abruptly declined to 7%, and she subsequently developed cardiogenic shock. She then experienced a sudden cardiac arrest, which required initiation of veno-arterial extracorporeal membrane oxygenation and the insertion of an Impella CP. Intravenous cyclophosphamide (1000 mg) was administered for suspected myocarditis, although an endomyocardial biopsy showed no specific positive staining. Cardiac function did not improve after immunosuppressive therapy. Therefore, plasma exchange was performed five times considering for macrophage activation syndrome, which resulted in the recovery of the left ventricular ejection fraction to 71%. Veno-arterial extracorporeal membrane oxygenation was successfully ceased, followed by veno-venous extracorporeal membrane oxygenation and mechanical ventilation to facilitate respiratory recovery for 39 days. The aetiology of the myocardial dysfunction remains unclear; however, both cytokine-mediated myocardial dysfunction associated with macrophage activation syndrome and immune-mediated microvascular injury could not be excluded. We successfully managed with a combined immunosuppressive therapy, including glucocorticoid, intravenous cyclophosphamide, and plasma exchange, under mechanical circulatory support.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-06-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148407567","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Polyarteritis nodosa and sarcoidosis presenting with myalgia and myositis-mimicking magnetic resonance imaging findings: A report of two cases.","authors":"Masayuki Shinojima, Satoshi Kubo, Ippei Miyagawa, Yasuyuki Todoroki, Masanobu Ueno, Yoshiya Tanaka, Shingo Nakayamada","doi":"10.1093/mrcr/rxag070","DOIUrl":"10.1093/mrcr/rxag070","url":null,"abstract":"<p><p>Diseases presenting with muscle symptoms are diverse. We report two cases initially suspected of idiopathic inflammatory myopathies that were ultimately diagnosed as polyarteritis nodosa and sarcoidosis, respectively. Case 1 was a 30-year-old man with fever, limb muscle pain, and elevated inflammatory markers. Muscle magnetic resonance imaging showed high signal intensity on fat-suppressed T2-weighted images, and biopsy revealed vasculitis with fibrinoid necrosis, leading to a diagnosis of polyarteritis nodosa. Case 2 was a 70-year-old man with proximal muscle pain and weakness and elevated muscle enzymes. Magnetic resonance imaging showed high signal intensity in the thigh muscles, and biopsy revealed noncaseating granulomas consistent with sarcoidosis. These cases highlight the diagnostic pitfalls of interpreting magnetic resonance imaging findings and serum creatine kinase levels in isolation when evaluating suspected idiopathic inflammatory myopathies and underscore the critical role of histopathological evaluation, including muscle biopsy, in establishing an accurate diagnosis.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-06-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148427624","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Dearbhail Reid, Benjamin G Faber, Caitlin Cahill, John D Pauling, Fang En Sin
{"title":"Antineutrophil cytoplasmic antibody-positive giant cell arteritis: Small-vessel vasculitis mimicking large-vessel vasculitis.","authors":"Dearbhail Reid, Benjamin G Faber, Caitlin Cahill, John D Pauling, Fang En Sin","doi":"10.1093/mrcr/rxag064","DOIUrl":"10.1093/mrcr/rxag064","url":null,"abstract":"<p><p>Giant cell arteritis is the most common vasculitis in those over 50 years old with a peak incidence between 70 and 80. Symptoms include a new-onset headache, scalp tenderness, jaw/tongue claudication, and visual symptoms, which can be sight-threatening. We report a case of an 82-year-old lady who presented with typical symptoms of giant cell arteritis and bilateral halo sign on an ultrasound scan. Despite initial steroid treatment she deteriorated and developed neurological symptoms including mononeuritis multiplex. Following further investigations, it was found that she was suffering from granulomatosis with polyangiitis and treatment was adjusted accordingly with additional immunomodulating treatment preventing further progression of symptoms. This case highlights how granulomatosis with polyangiitis can mimic giant cell arteritis and should be considered in refractory cases.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-06-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148681583","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Kota Hyakuna, Shigeru Iwata, Taro Nishikawa, Kodai Ueno, Yuta Shimomura, Takeru Sonoda, Takashi Kato, Ryo Matsumiya, Takao Fujii
{"title":"Adult hypophosphatasia with a single heterozygous c.572A > G p.Glu191Gly mutation in the ALPL gene: Case report.","authors":"Kota Hyakuna, Shigeru Iwata, Taro Nishikawa, Kodai Ueno, Yuta Shimomura, Takeru Sonoda, Takashi Kato, Ryo Matsumiya, Takao Fujii","doi":"10.1093/mrcr/rxag082","DOIUrl":"10.1093/mrcr/rxag082","url":null,"abstract":"<p><p>Adult-onset hypophosphatasia manifests after age 18, and is characterised by reduced tissue-nonspecific alkaline phosphatase activity. This can lead to fractures, pseudo-fractures, osteomalacia, decreased bone density (bone loss), muscle weakness, myalgia, arthralgia, headache, dental symptoms (loss of permanent teeth, periodontal disease), and pseudogout. Over 480 distinct genetic mutations have been identified to date, with eight cases reported for the c.572A > G (p.Glu191Gly) mutation. However, all reported cases involved compound heterozygous mutations, and there have been no previous reports of cases of a single heterozygous mutation. We report the case of a 40-year-old woman with persistent hypophosphatasia, elevated urinary phosphoethanolamine levels suggesting adult-onset hypophosphatasia, and a heterozygous missense mutation (c.572A > G p.Glu191Gly) in the ALPL gene according to genetic testing. Treatment with subcutaneous injections of aspartate phosphatase alpha was initiated, which was followed by significant improvement of the pain, enhanced range of motion in both upper limbs, and improved gait. This patient's case differed from previously reported cases in that it involved a heterozygous c.572A > G (p.Glu191Gly) mutation alone in adult-onset hypophosphatasia. Adult-onset hypophosphatasia is an extremely rare disorder. However, its symptoms resemble those of systemic rheumatic diseases such as polymyalgia rheumatica and fibromyalgia, and rheumatologists may encounter this condition in routine clinical practice. Importantly, it can be detected based on low serum ALP levels.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-06-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148820782","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Systemic polyarteritis nodosa diagnosed in adulthood after childhood-onset recurrent inflammatory disease with atypical Still-like features.","authors":"Daisuke Kobayashi, Shunsuke Sakai, Sayuri Takamura, Yoko Wada, Satoshi Ito, Eriko Hasegawa, Ayako Wakamatsu, Yukiko Nozawa, Hiroe Sato, Takeshi Kuroda, Suguru Yamamoto","doi":"10.1093/mrcr/rxag080","DOIUrl":"10.1093/mrcr/rxag080","url":null,"abstract":"<p><p>We report a rare long-term case in which systemic polyarteritis nodosa was ultimately diagnosed in adulthood after childhood-onset recurrent inflammatory disease with atypical Still-like features. A Japanese man had recurrent febrile episodes from infancy and was diagnosed with juvenile idiopathic arthritis at 9 years of age. In early adulthood, he developed recurrent fever, rash, polyarthralgia, splenomegaly, neutrophilia, and elevated inflammatory markers, and was considered to have Still-spectrum disease. However, ferritin elevation remained modest despite marked systemic inflammation, suggesting that the early phenotype may not have represented typical Still's disease and may instead have reflected a broader autoinflammatory process. At 25 years of age, he developed digital ischaemia with necrosis, palpable nodules, and erectile dysfunction. Angiography demonstrated a narrowing of the ulnar and palmar arteries, and skin biopsy showed necrotising vasculitis involving medium-sized vessels, leading to the diagnosis of systemic polyarteritis nodosa. He subsequently developed fever, rash, inner-ear involvement, hypertrophic pachymeningitis, and tortuosity/dilatation of a right intercostal artery. A subtle suspected lesion of the right testicular artery later became a definitely enlarging aneurysmal lesion on serial imaging. Systemic inflammatory manifestations were subsequently controlled during treatment that included glucocorticoids, azathioprine, and tocilizumab. However, serial imaging demonstrated the gradual enlargement of the right testicular artery aneurysmal lesion despite the absence of fever, rash, or C-reactive protein elevation for 5 years. This clinical course suggests that vascular progression may not always be adequately reflected by C-reactive protein levels during interleukin-6 blockade. This case illustrates the diagnostic difficulty of a childhood-onset recurrent inflammatory disease with atypical Still-like features that was ultimately recognised as systemic polyarteritis nodosa in adulthood. Occult or under-recognised medium-vessel vasculitis may have been present earlier in the disease course. Long-term diagnostic reappraisal, vascular imaging when indicated, and continued follow-up across paediatric and adult rheumatology care may therefore be important in similar patients, particularly in the current biologic era.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-06-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148803868","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Pustulotic arthro-osteitis complicated by polyarteritis nodosa.","authors":"Moe Asada, Masaki Matsubara, Yoshinori Uenishi, Yuki Hatta, Nobushiro Nishimura, Hiromasa Kawashima, Ryo Yoneima, Hidetoshi Matsuoka, Shiro Ono, Noritaka Yada, Hiroto Nishimura, Ryota Hara, Takayoshi Morita, Yasuhiro Akai, Kenji Nishio, Kiyomi Yoshimoto","doi":"10.1093/mrcr/rxag088","DOIUrl":"10.1093/mrcr/rxag088","url":null,"abstract":"<p><p>Pustulotic arthro-osteitis (PAO) is a chronic inflammatory disorder associated with palmoplantar pustulosis (PPP) and characterised by osteoarticular manifestations. Although several immune-mediated comorbidities have been reported in association with PPP/PAO, polyarteritis nodosa occurring in patients with PPP/PAO has not been described previously. We report a case of a 56-year-old woman with a long-standing history of PPP and PAO who developed worsening osteoarticular symptoms in close temporal proximity to purpura, lower-extremity pain, and sensory disturbance, followed by paraesthesia in the fingers. Neurological examination revealed mild weakness of the left tibialis anterior muscle. Laboratory testing showed elevated inflammatory markers, whereas myeloperoxidase- and proteinase 3-antineutrophil cytoplasmic antibodies were negative. Musculoskeletal imaging revealed bilateral sternoclavicular joint synovitis, inflammatory changes adjacent to the right patellar tendon insertion with infrapatellar bursitis, and right peroneal tenosynovitis. Nerve conduction studies in the lower extremities suggested mononeuritis multiplex. A skin biopsy of a purpuric lesion demonstrated fibrinoid necrosis and inflammatory infiltration in medium-sized arteries within the subcutaneous tissue. The patient was diagnosed with an exacerbation of PAO complicated by polyarteritis nodosa and was treated with oral prednisolone followed by methotrexate, resulting in rapid normalisation of inflammatory markers and improvement in osteoarticular symptoms, purpura, and finger paraesthesia, although lower-extremity neurological symptoms persisted at discharge. When patients with PAO develop atypical features such as purpura or neuropathic symptoms suggestive of mononeuritis multiplex, concomitant vasculitis, including polyarteritis nodosa, should be considered in the differential diagnosis.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-06-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148901710","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Tocilizumab for refractory cerebral arteritis in a patient with systemic lupus erythematosus.","authors":"Yasuto Minegishi, Takehisa Ogura, Chihiro Imaizumi, Reina Maezawa, Takafumi Uchi, Hideto Kameda","doi":"10.1093/mrcr/rxag094","DOIUrl":"10.1093/mrcr/rxag094","url":null,"abstract":"<p><p>Cerebral arteritis is a rare (<1%) but life-threatening manifestation of systemic lupus erythematosus (SLE). The optimal treatment for cerebral arteritis in refractory cases remains unclear; conventional immunosuppressive therapy results in an improvement in arterial stenosis in only 29% of reported cases. We report the case of a 51-year-old woman with a 20-year history of SLE and rheumatoid arthritis who developed aphasia and right hemiparesis due to a haemodynamic cerebral infarction caused by cerebral arteritis. Despite treatment with high-dose glucocorticoids, intravenous cyclophosphamide, mycophenolate mofetil, and anifrolumab, the disease remained progressive with persistently elevated C-reactive protein levels and worsening arterial stenosis on magnetic resonance angiography. Cerebrospinal fluid interleukin-6 level was markedly elevated (1590 pg/ml). After switching from anifrolumab to tocilizumab, C-reactive protein levels normalised, the patient's neurological condition stabilised, and follow-up magnetic resonance angiography suggested an improvement in arterial stenosis. Tocilizumab might be a viable treatment option for refractory cerebral arteritis in patients with SLE, particularly in cases with elevated cerebrospinal fluid interleukin-6 levels. To our knowledge, this is the first reported case suggesting the potential efficacy of tocilizumab in SLE-related cerebral arteritis.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-06-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149083385","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}