Rationale: Thrombocytopenia, anasarca, fever, reticulin fibrosis, renal dysfunction, and organomegaly (TAFRO) syndrome are nonmalignant but life-threatening systemic inflammatory disorders. However, many patients are refractory to treatment, resulting in significant morbidity and mortality. Additionally, established treatment options are unavailable. Therefore, we present 2 cases of adults with the iMCD-TAFRO syndrome refractory to initial treatment but responded to Janus kinase (JAK) inhibitors with ruxolitinib. The report reveals that these rare adult cases of the refractory and treatment-resistant iMCD-TAFRO syndrome can be treated using JAK inhibitors. Patient concerns: Case 1 is a 36-year-old previously healthy male patient who presented with fever and general fatigue for 2 weeks. Case 2 is a 42-year-old previously healthy female patient who presented with fever and general fatigue. Diagnosis: The diagnosis met the 2015 criteria for TAFRO syndrome, as determined by All Japan TAFRO Syndrome Research Group in the Research Program for Intractable Disease by the Ministry of Health, Labor and Welfare (MHLW) Japan. Interventions: Treatment with tocilizumab and several immunosuppressants were ineffective. So, we performed ruxolitinib. Outcomes: Each patient received ruxolitinib, the general condition improved, and CRP levels decreased. Lessons: These cases showed that ruxolitinib was effective for treatment-resistant/ refractory TAFRO syndrome. Further prospective studies are needed on using ruxolitinib with a small number of cases.

TAFRO syndrome, first reported in 2010, is a systemic inflammatory disease with a rapid onset and potentially fatal course if not treated promptly and appropriately. The name is derived from the initial letters describing the characteristic symptoms of thrombocytopenia, anasarca, fever, reticulin fibrosis/renal failure, and organomegaly. It is sometimes considered a special subtype of idiopathic multicentric Castleman disease (iMCD) because lymph node biopsies often reveal the pathology findings seen in iMCD. However, its clinical manifestations and prognoses are not well documented. Since the clinical manifestations and prognoses of TAFRO syndrome differ significantly from those of iMCD, it is recognized as an independent disease concept and considered to partially overlap with the pathology of MCD. The pathogenesis of TAFRO syndrome remains largely unknown. Due to the lack of appropriate treatment, it often presents with multiple organ dysfunction and fatality. In this review, we summarized new findings on the pathogenesis of TAFRO syndrome and discussed current effective therapies and future treatment strategies.

Objectives: To evaluate vaccination rates and identify factors associated with vaccine uptake among patients with autoimmune inflammatory rheumatic diseases (AIRD) in Kanagawa Prefecture, Japan. Methods: An anonymous survey was conducted between January and May 2023 using Google Forms™. Adult AIRD patients and rheumatologists were asked about demographics, disease characteristics, vaccination status, and attitudes towards vaccination. Logistic regression and random forest analyses were used to identify factors influencing vaccination rates. Results: A total of 1 637 AIRD patients responded. Vaccination rates were as follows: herpes zoster 8.8%, influenza 86.7%, pneumococcus 29.7%, human papillomavirus 4.3%, hepatitis B virus 8.7%, and severe acute respiratory syndrome corona virus-2 92.2%. Physician recommendations were significantly associated with higher vaccination rates. Although knowledge of vaccine efficacy correlated with increased uptake, it did not fully alleviate vaccine hesitancy. Major barriers included concerns about adverse effects and lack of sufficient information. Conclusions: Physician recommendations and providing comprehensive vaccine information are essential for improving vaccination rates among AIRD patients. Addressing concerns about vaccine safety and efficacy through targeted educational interventions and communication strategies is crucial to enhancing vaccine uptake.

Autoimmune neuromuscular disorders in patients with eosinophilic granulomatosis with polyangiitis (EGPA) are relatively uncommon. Although two cases of myasthenia gravis (MG) comorbid with EGPA have been reported, both patients developed EGPA several years after starting immunosuppressive treatment for MG. We herein report a 75-year-old man with a rare co-occurrence of EGPA and MG that developed simultaneously and was successfully treated with immunosuppressive therapy. Distinguishing the neurological symptoms of EGPA from complications of other neurological autoimmune diseases, such as MG, is crucial, especially in patients with eosinophilia.

Rationale: Anti-melanoma differentiation–associated gene 5 (anti-MDA5) antibody-positive dermatomyositis is frequently complicated by rapidly progressive interstitial lung disease and may present with life-threatening hemophagocytic syndrome, a critical but often under-recognized complication. Prompt diagnosis and intervention are essential to improve patient outcomes. Patient concerns: A 38-year-old Japanese woman presented with fever, arthralgia, erythema, and elevated liver enzymes. Physical examination revealed chilblain-like erythema, Gottron and reverse Gottron signs, and tenderness in the thighs. Diagnoses: Blood tests revealed pancytopenia and elevated soluble interleukin-2 receptor and ferritin levels. Bone marrow aspiration showed macrophage activation and hemophagocytosis. Imaging revealed ground-glass opacities in both lower lung lobes and bilateral thigh myositis. The presence of anti-MDA5 antibodies led to the diagnosis of anti-MDA5-positive dermatomyositis complicated by autoimmune-related hemophagocytic syndrome. Liver biopsy suggested cytokine storm-mediated hepatocyte damage and macrophage infiltration. Interventions: The patient received steroid pulse therapy, tacrolimus, and intravenous cyclophosphamide. Outcomes: Pancytopenia and liver dysfunction improved significantly following treatment. The patient was transitioned to outpatient care. After 4 years of follow-up, during which she received 6 courses of cyclophosphamide, steroids, and tacrolimus were successfully tapered off, and she remained in remission without recurrence. Lessons: This case underscores the importance of early recognition of hemophagocytic syndrome in patients with anti-MDA5-positive dermatomyositis. Prompt bone marrow evaluation and aggressive immunosuppressive therapy may lead to favorable long-term outcomes. Further studies are warranted to establish standardized treatment strategies for autoimmune-related hemophagocytic syndrome.

We present the case of a 75-year-old man diagnosed with myasthenia gravis (MG) based on lower-leg weakness and ptosis for two months before admission to our hospital. The patient was anti-acetylcholine receptor antibody-positive at admission. He was treated with pyridostigmine bromide and prednisolone, which improved the ptosis, but the lower-leg muscle weakness remained. An additional lower-leg magnetic resonance imaging exam suggested myositis. Inclusion body myositis (IBM) was diagnosed after a subsequent muscle biopsy. Although MG is often associated with inflammatory myopathy, IBM is rare. There is no effective treatment for IBM, but various treatment possibilities have recently been proposed. This case emphasises that myositis complications, including IBM, should be considered when elevated creatine kinase levels are observed and conventional treatments do not address chronic muscle weakness.