Modern Rheumatology Case Reports最新文献

The current report presents two cases with leg ulcers related to Behçet's disease (BD) resistant to conventional immunosuppressive therapy (CIST) but successfully treated with adalimumab (ADA). BD, which can affect vessels of any size and type, is a systemic vasculitis. In the vascular system, veins are the most predominantly affected blood vessels, with deep vein thrombosis and recurrent superficial vein thrombophlebitis being the most common vascular signs of the disease in the lower extremities. Leg ulcers, commonly associated with vasculitis or deep vein thrombosis, are rare in patients with BD. Conventional immunosuppressive therapy is very critical to prevent relapses and diminish the risk of post-thrombotic syndrome. In patients with BD-associated venous thrombosis (deep vein thrombosis or superficial vein thrombophlebitis) resistant to these treatments, tumour necrosis factor-α inhibitors can be used alone or in combination with traditional disease-modifying antirheumatic drugs. In view of such information, add-on adalimumab treatment was considered appropriate for both patients. Response to this intervention was highly satisfying for the patients at the end of the 6-month treatment. Nonetheless, it warrants further studies directly evaluating the efficacy of tumour necrosis factor-α inhibitors alone in leg ulcers in BD.

Autoimmune diseases, including dermatomyositis, can be complicated by an acquired autoimmune coagulation factor XIII deficiency, which sometimes results in fatal bleeding. Here, we report the case of a young woman with anti-NPX-2 antibody-positive dermatomyositis who developed massive haemothorax with acquired factor XIII deficiency during treatment, including plasma exchange therapy. Emergency transcatheter arterial embolisation was performed and coagulation factor XIII concentrates (Fibrogammin P® 240 U/day for 5 days) were supplemented. Subsequently, the patient was discharged and managed with oral prednisolone and tacrolimus. Coagulation system test results were followed up regularly and remained within normal limits and the patient progressed without recurrence of bleeding symptoms. Coagulation factor XIII deficiency cannot be assessed without measuring coagulation factor XIII activity because common coagulation-fibrinolytic system test results are not abnormal. The measurement of factor XIII activity should be performed when autoimmune diseases are complicated by unexplained bleeding.

This case report describes a 52-year-old immunocompromised man diagnosed with disseminated Mycobacterium abscessus complex (MABC) infection. The patient had a history of malignant lymphoma and presented with fever and polyarthritis that lasted 3 weeks. Upon initial evaluation, blood and synovial fluid cultures from the swollen joints were negative. Reactive arthritis or rheumatoid arthritis was suspected as the cause of inflammatory synovitis in multiple joints. Administration of prednisolone followed by an interleukin-6 inhibitor improved the fever, but polyarthritis persisted, and destruction of the left hip joint was observed. Two months later, M. abscessus was detected in a blood culture and right shoulder joint synovium, leading to a final diagnosis of disseminated MABC infection. The joint symptoms resolved with combined antimicrobial therapy using amikacin, azithromycin, and imipenem/cilastatin. To date, 12 cases of disseminated MABC infection with osteoarticular manifestations have been reported. A total of 13 cases, including the present case, were reviewed. Seven patients had bone involvements, five had joint involvement, and the remaining one had bursa involvement. All the cases with joint involvement, except for our case, presented with monoarthritis. MABC infection is diagnosed based on the demonstration of MABC itself. Clinicians should keep disseminated MABC infection in mind as a possible cause of persistent arthritis. As demonstrated in our case, multiple replicate cultures of blood or specimens from the affected sites may be needed to detect it.

According to previous reports, most cases of inflammatory myopathy following messenger RNA (mRNA) vaccination can be classified as idiopathic inflammatory myopathy, particularly dermatomyositis, owing to their similar clinical features and courses. However, some patients have different clinical features and courses. We report a rare case of transient inflammatory myopathy involving the masseter muscle following the third dose of coronavirus disease 2019 (COVID-19) mRNA vaccination. An 80-year-old woman presented with a history of fever and fatigue for 3 months soon after receiving the third COVID-19 mRNA vaccination. Her symptoms progressed to jaw pain and inability to open her mouth. She also experienced mild proximal muscle weakness in the lower limbs but no skin manifestations or daily difficulties. Fat-saturated T2-weighted magnetic resonance imaging showed bilateral high-intensity signals for the masseter and quadriceps muscles. The patient experienced spontaneous resolution of fever and improvement of symptoms 5 months after onset. The timing of the onset of symptoms, the lack of detectable autoantibodies, and the atypical presentation of myopathy in the masseter muscles, in addition to the spontaneous mild course of the disease, all indicate the substantial role of mRNA vaccination in this myopathy. Since then, the patient has been followed up for 4 months without any recurrence of symptoms or any additional treatment. It is important to recognise that the course of myopathy after COVID-19 mRNA vaccination could be different from that of typical idiopathic inflammatory myopathies.

A 71-year-old male with hypertrophic osteoarthropathy was referred to our hospital because of a nocturnal fever and tenderness stretching from the left parotid region to the left front neck, in which antibacterials were ineffective. He was diagnosed with Takayasu's arteritis following findings of contrast-enhanced computed tomography and neck ultrasound. This is the first report to describe the development of Takayasu's arteritis after a prolonged course of typical hypertrophic osteoarthropathy, and the proposed hypertrophic osteoarthropathy and Takayasu's arteritis have common underlying pathophysiological factors.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a small- to medium-vessel necrotising vasculitis and eosinophilic inflammation. Mepolizumab, an anti-interleukin-5 (IL-5) monoclonal antibody has been approved in Japan since 2018 for refractory EGPA treatment. Benralizumab, an anti-IL-5 receptor monoclonal antibody, also has been reported to reduce the glucocorticoid dose in patients with refractory EGPA. On the other hand, several investigators have demonstrated new-onset EGPA under biologics, and it is unclear whether this treatment for severe allergic diseases can prevent the development of EGPA. Herein, we report a case of new-onset EGPA under benralizumab treatment. The patient had fever, weight loss, muscle pain, and paraesthesia, the serum eosinophil count was 0/μL, and the biopsy showed necrotizing vasculitis without eosinophilic infiltration. She was diagnosed as having EGPA and treated with high-dose glucocorticoid and intravenous cyclophosphamide, with a good response. Our case report indicates that anti-IL-5 agents may mask the development of EGPA and clinicians should be aware of the development of EGPA during anti-IL-5 agents.

A 38-year-old female was referred with a history of fever, polyarthralgia, and bone pain. She was diagnosed with chronic recurrent multifocal osteomyelitis based on imaging and biopsy findings. Non-steroidal anti-inflammatory drugs and bisphosphonate caused no improvement. Then, she developed recurrent diarrhoea and abdominal pain. Genetic testing revealed MEFV mutation. Based on the symptoms and genetic mutation results that emerged during the course of these events, she was diagnosed with familial Mediterranean fever. All symptoms, including bone pain, improved with daily colchicine administration. This case was considered familial Mediterranean fever complicated with a clinical diagnosis of chronic recurrent multifocal osteomyelitis, which is included in the spectrum of pyrine autoinflammatory diseases. Considering this case, patients with chronic recurrent multifocal osteomyelitis with MEFV gene variants may respond to colchicine.

Systemic lupus erythematosus (SLE) is an autoimmune disease that causes damage to multiple organs. Various factors, including vaccination, have been associated with SLE development. Vaccination for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) began in 2020, and there are a few reports on the exacerbation of SLE after SARS-CoV-2 vaccination. The influence of SARS-CoV-2 vaccination on SLE development remains unclear. We present the case of a 53-year-old man who developed peritonitis and was subsequently diagnosed with SLE on Day 9 after receiving a third dose of the messenger ribonucleic acid-1273 SARS-CoV-2 vaccine. This case and previous reports have shown that patients who developed SLE after SARS-CoV-2 vaccination are more likely to develop it within 2 weeks of vaccination, especially when they have a higher rate of immunological abnormalities or a family history of autoimmune diseases. Furthermore, these features suggest that type I interferon is involved in the pathogenesis of SLE after SARS-CoV-2 vaccination.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a complex multifactorial disease that results in multisystemic inflammation of the small- and medium-sized arteries. The exact pathogenesis of this syndrome is poorly understood, but it is postulated to result from a combination of eosinophilic dysfunction, genetic predisposition, and the development of autoantibodies after exposure to an unknown stimulus. We describe a case of new-onset EGPA following the third dose of the Pfizer-BioNTech mRNA vaccine in an infection-naive middle-aged man with a background history of allergic respiratory symptoms. The patient developed acute onset of mononeuritis multiplex, pauci-immune glomerulonephritis, and leucocytoclastic vasculitis 10 days after receiving the booster dose. His laboratory markers including eosinophil count, antineutrophil cytoplasmic antibodies, and renal function tests improved markedly after the initiation of pulse steroid therapy and rituximab infusion. However, his peripheral muscle weakness and neuropathic pain did not respond to the initial therapy but improved later with intravenous cyclophosphamide and intravenous immunoglobulin. To the best of our knowledge, this is the fourth case report of post-coronavirus disease 2019 vaccination precipitation of EGPA. All reported cases including our report were in patients with previous allergic manifestations who received mRNA-based coronavirus disease 2019 vaccines, and all the patients developed mononeuritis multiplex at presentation. Despite the few reported cases of post-vaccination autoimmune phenomena, the temporal association between vaccination administration and disease onset does not indicate causality, given the mass vaccination programmes employed. However, the novel use of the mRNA platform in vaccine delivery necessitates vigilant monitoring by the scientific committee.

Total wrist arthroplasty (TWA) is a challenging procedure for wrist joints severely destroyed by rheumatoid arthritis. The most common postoperative complication of TWAs is the loosening of the carpal component. Revision surgeries for failed TWAs can be complicated owing to severe bone loss and concomitant soft-tissue problems. Here, we report a case (68-year-old woman with a history of rheumatoid arthritis for 24 years) of severe aseptic loosening of semi-constrained TWA and its salvage surgery. During the primary arthroplasty procedure, severe instability at the second through fifth carpometacarpal joints was observed and arthrodesis of these joints was required. The radiographs obtained 9 months after surgery showed loosening of the carpal component. Subsequently, she suffered a stroke, resulting in a loss of follow-up, and higher stress was loaded on her upper extremities during standing and walking. In the radiograph taken at her revisit 25 months after the primary surgery, the subsidence of the carpal component progressed and loosening of the radial component was observed. Total wrist arthrodesis was performed using a bulk bone allograft of the femoral head, combined with a penetrating Wrist Fusion Rod®. Rapid bone union was achieved without soft-tissue irritation. We conclude that wrist arthrodesis with a bulk bone allograft combined with an intramedullary nail is a reasonable option for failed total wrist arthroplasty.