Tocilizumab (TCZ) is effective for inducing remission in adult-onset Still's disease (AOSD), but its use may occasionally trigger macrophage activation syndrome (MAS). The rationale for re-introducing TCZ in patients with a history of MAS is not well established. Here, we report a case of successful re-administration of TCZ for an AOSD relapse in a patient with a prior history of MAS during TCZ therapy. A 67-year-old woman, initially treated with TCZ for polyarthritis, developed MAS associated with AOSD. MAS was resolved with glucocorticoid pulse therapy, high-dose glucocorticoids, and cyclosporine A. However, AOSD relapsed during glucocorticoid tapering. Methotrexate, cyclosporine A, and repeated glucocorticoid pulses failed to control the disease. Following another glucocorticoid pulse, TCZ (8 mg/kg weekly) was re-introduced intravenously. This approach allowed successful glucocorticoid tapering and long-term remission. This case highlights the complexities of managing AOSD: while the initial TCZ therapy may have contributed to the onset of MAS, the subsequent re-introduction of TCZ enabled effective disease control and sustained remission.

Systemic lupus erythematosus (SLE) can present with various symptoms, including rare manifestations such as gangrene. This report describes a 12-year-old girl with SLE who presented with intermittent claudication and gangrene. Although intermittent claudication is rare in paediatric cases, it is essential to consider vascular diseases including those associated with SLE as a potential cause. The patient initially experienced pain, redness, and cold sensations in the right great toe accompanied by intermittent claudication, with symptoms worsening over time. Diagnostic imaging, including contrast-enhanced computed tomography and magnetic resonance imaging, revealed occlusion of the right popliteal artery with associated vasculitis and thrombosis. The diagnosis of SLE and antiphospholipid syndrome was confirmed based on clinical criteria. Treatment included prednisone, methylprednisolone pulse therapy, mycophenolate mofetil, hydroxychloroquine, and anticoagulants. The patient showed significant improvement, with resolution of claudication and effective management of her gangrene through immunosuppressive therapy and careful wound care. This case highlights the importance of considering vascular complications in paediatric SLE and underscores the need for early diagnosis and comprehensive treatment.

IgG4-related disease (IgG4-RD) is an under-recognised multisystem inflammatory disorder that has several typical manifestations. Cardiac manifestations of IgG4-RD are well documented; however, they do not feature in the definition or diagnosis of IgG4-RD according to a recent consensus statement. The most well-recognised cardiac manifestation of IgG4-RD, pericardial disease, is outlined in this case report as the initial presenting pathology. In the present case, the diagnosis was delayed due to the relative obscurity of cardiac manifestations of IgG4-RD and exposed the patient to the risks of pericardiectomy.

Polymyalgia rheumatica (PMR) is a common inflammatory disorder characterized by myalgia/stiffness in proximal hip and shoulder girdle, elevated C reactive protein, and erythrocyte sedimentation rate, but its pathogenesis is not fully elucidated. We report three cases of PMR who do not respond adequately to standard treatment. Those patients had typical symptoms of myalgia and muscle weakness, with elevated C reactive protein in absence of creatine kinase elevation. Muscle specimen showed the findings of vasculitis in all cases; therefore, muscular-limited vasculitis may be an underlying pathology in PMR in those refractory cases.

Anti-tumour necrosis factor (TNF) agents like adalimumab are safe and effective for rheumatologic disorders, but they have been reported to cause demyelinating diseases like multiple sclerosis. A 47-year-old woman with weakness in the left hand was evaluated. She had a previous medical history of intermediate uveitis and rheumatoid arthritis. Anti-TNF-α treatment was initiated with adalimumab 2 years before this visit. Magenetic resonance imaging showed numerous T2-hyperintense lesions in bilateral periventricular, juxtacortical, and subcortical areas, which were absent in her scan before anti-TNF therapy. She was diagnosed with multiple sclerosis, and adalimumab was discontinued. IV glucocorticoids were administered with marked improvement. She was then started on anti-CD20 therapy with rituximab. A year later, she was symptom-free, and her follow-up brain magnetic resonance imaging showed no new lesions. The link between multiple sclerosis and TNF-α inhibitors is poorly understood, but several hypotheses have been proposed. Discontinuing anti-TNF therapy alone may not be enough to prevent further demyelinating disease activity, and it is essential to consider the necessity of starting a disease-modifying treatment. Autoimmunity plays a significant role in rheumatologic and neurological diseases, and as personalised medicine advances, understanding genetic risk is crucial for selecting appropriate therapeutic targets. A thorough evaluation of a patient's family background is recommended before a therapeutic decision-making, especially in patients with multiple autoimmune disorders, and the question of whether TNF-α is a suitable therapeutic target in patients with multiple autoimmune disorders is raised.

A 53-year-old woman with recurrent stomatitis, genital ulcers, and folliculitis was admitted to Tokyo Medical University Hospital after experiencing visual disturbances for the past 2 weeks and a nonthrobbing headache for the past 3 days. She had also developed numbness in her left extremities. An ophthalmological examination revealed inflammatory changes in the eye. Cerebrospinal fluid analysis showed increased cell counts, protein, and interleukin-6 levels. Brain magnetic resonance imaging revealed multiple high signal intensities on T2-weighted/fluid-attenuated inversion recovery images of the pons and occipital and parietal lobes. The T2-weighted/fluid-attenuated inversion recovery high-signal-intensity lesion in the pons was hyperintense on diffusion-weighted imaging and hypointense on apparent diffusion coefficient mapping, suggesting cytotoxic oedema. Another high-signal-intensity lesion on T2-weighted/fluid-attenuated inversion recovery was isointense to hyperintense on diffusion-weighted imaging and hyperintense on apparent diffusion coefficient, indicating vasogenic oedema. The vasogenic oedema in the left occipital lobe contained a small core that was hyperintense on diffusion-weighted imaging and hypointense on apparent diffusion coefficient, suggesting cytotoxic oedema. The patient was diagnosed with acute neuro-Behçet's disease and responded well to high-dose glucocorticoid and colchicine treatment. The present report emphasises that patients with acute neuro-Behçet's disease may present with cytotoxic oedema in the pons and cerebral spheres. Further reports of similar cases would contribute to a better understanding of the role of cytotoxic oedema in the pathophysiology of neuro-Behçet's disease and help elucidate the mechanisms underlying a unique presentation characterised by a central cytotoxic oedema core within vasogenic oedema.

Synovitis-acne-pustulosis-hyperostosis-osteomyelitis (SAPHO) syndrome is characterised by aseptic osteitis and is often complicated by pustular dermatitis, such as palmoplantar pustulosis or acne. Although bone lesions are most found in the anterior thoracic region or spine, femoral lesions are not well documented in the literature. There is no established treatment for this condition, and few reports have described its long-term course. Here, we describe two cases of SAPHO syndrome involving the femur and discuss their long-term follow-up. A 40-year-old man (Case 1) presented with right thigh pain. Fifteen years after the initial diagnosis, the pain could be controlled with minomycin, salazosulfapyridine, and methotrexate. X-rays of the femur showed gradual cortical thickening. Although there were waves of pain, it gradually improved with the adjustment of drugs 25 years following the initial diagnosis. A 35-year-old man (Case 2) with right thigh pain was prescribed salazosulfapyridine and methotrexate; however, these were ineffective. Alendronate and guselkumab also proved ineffective. Ultimately, infliximab was started 9 years following disease onset, and pain became manageable. X-rays of the femur showed cortical thickening. SAPHO syndrome can be managed with drug therapies, such as nonsteroidal anti-inflammatory drugs, methotrexate, and conventional synthetic disease-modifying antirheumatic drugs; however, there are occasional treatment-resistant cases.

Sternocostoclavicular hyperostosis (SCCH) has been reported in patients with pustulotic arthro-osteitis, but there are few reports of marked ossification of the lateral part of the clavicle. Here, we report a case of stress fracture in a patient with SCCH with marked ossification of the lateral part of the clavicle. In this case, the clavicular fracture was proximal and no dislocation. Conservative treatment with a clavicle band and the administration of corticosteroids resulted in rapid symptom improvement. Eight months later, the patient had no difficulty in daily life, but X-rays showed that bone fusion was not complete. Therefore, it is necessary to carefully follow-up such patients for any recurrence of symptoms and radiographic changes.

We report a case of IgG4-related disease (IgG4-RD) with marked eosinophilia. A 79-year-old woman was admitted due to diarrhoea and weight loss. Cervical lymphadenopathy, bilateral submandibular glands swelling, anaemia (Hb8.5 g/dl), hypereosinophilia (9750/μl), elevated serum creatinine (1.57 mg/dl), pancreatic amylase (191 IU/l), and IgG4 (3380 mg/dl) were found. Diffusion-weighted image on magnetic resonance imaging showed high-intensity signals inside both the pancreas and the kidneys. The echogram of submandibular glands revealed cobblestone pattern. Kidney biopsy revealed acute tubulointerstitial nephritis. Biopsies of lip, gastrointestinal tract, and bone marrow showed infiltration of lymphoplasmacytic cells and IgG4-positive plasma cells (30-67/HPF). Gastrointestinal and bone marrow biopsies also showed eosinophilic infiltration. Adrenal insufficiency, rheumatic disease, tuberculosis, parasite infection, drug-induced eosinophilia, and eosinophilic leukaemia were all ruled out. We started treatment with 40 mg of prednisolone (PSL) and her general condition rapidly improved. The eosinophil count, serum IgG4, and serum creatinine decreased. We gradually tapered PSL and maintained 5 mg/day. During the 5 years of treatment, she had no recurrence of the symptom. According to the 2019 American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-RD, eosinophils >3000/μl is one of the exclusion criteria. If we comply with this criterion, the diagnosis of IgG4-RD should be avoided. However, our case fit the diagnostic criteria of type I autoimmune pancreatitis, IgG4-related sialadenitis, and global diagnosis of IgG4-RD. We finally diagnosed our case as IgG4-RD with secondary hypereosinophilic syndrome. This case suggests that IgG4-RD with eosinophils >3000/μl does exist in the real world.

This study evaluated a reverse V-shaped osteotomy for ankylosing rocker-bottom foot deformity in patients with rheumatoid arthritis. Three feet were presented in this study: rheumatoid rocker-bottom deformities with painful and/or infectious bony prominence towards the bottom of the foot, treated with a reverse V-shaped osteotomy in the mid-hindfoot. In all three cases, significant correction was achieved with restoration of the medial longitudinal arch, and improvement in clinical scores was confirmed. Reverse V-shaped osteotomy has the potential to be a useful and definitive procedure for ankylosing rocker-bottom deformity in patients with rheumatoid arthritis.