Modern rheumatology case reports最新文献

Gout is the most prevalent form of inflammatory arthritis in males as a result of high uric acid levels that cause deposition of monosodium urate crystals in soft tissues, bones and joints. It commonly presents as swollen, erythematous and painful joint. Necrotizing fasciitis is rapidly progressing aggressive soft tissue infection that spreads along the fascial planes sparing the skin; it is very uncommon for necrotizing fasciitis to occur as a complication of gouty arthritis. We present here a case of a 69 year old male with known history of untreated gouty arthritis and tophi over the right foot that complicated into necrtotizing fasciits of the leg with extension to the upper thigh that was surgically debrided but also necessitated above knee amputation for infection control, survival and best possible functional outcome of the patient.

Pyoderma gangrenosum (PG) is a rare chronic skin disease characterized by painful skin ulcers. There are no treatment guidelines for PG; however, systemic treatment is often administered. Recently, adalimumab (ADA), a fully human monoclonal antibody against tumor necrosis factor, was approved for the treatment of refractory PG in Japan. However, data are limited, and it is not clear whether ADA has the same effect on the treatment of PG in patients with systemic rheumatic disease (SRD), including rheumatoid arthritis (RA). In addition, the glucocorticoid-sparing effect of ADA in SRD patients with PG has not yet been clarified. Herein, we present two successful cases of RA with glucocorticoid-refractory PG on ADA treatment. Our report suggests that ADA may have a glucocorticoid-sparing effect on refractory PG in patients with RA.

This report describes the case of an 85-year-old woman who was found to have seronegative rheumatoid arthritis during preoperative investigations for knee replacement surgery for osteoarthritis. Follow-up for knee osteoarthritis was continued for many years without any symptoms involving other joints. Before total knee arthroplasty for osteoarthritis, preoperative investigations revealed a highly inflammatory state. After differential diagnosis, seronegative rheumatoid arthritis was diagnosed. Even in preoperative investigations before total knee arthroplasty for very old patients with osteoarthritis, the possibility of rheumatoid arthritis developing should always be kept in mind.

Polymyalgia rheumatica (PMR) is a common inflammatory disorder characterized by myalgia/stiffness in proximal hip and shoulder girdle, elevated C reactive protein (CRP) and erythrocyte sedimentation rate (ESR), 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 CRP in absence of creatine kinase elevation. Muscle specimen showed the findings of vasculitis in all cases, therefore muscular limited vasculitis (MLV) may be an underlying pathology in PMR in those refractory cases.

Patients with rheumatoid arthritis (RA) receiving immunosuppressive therapy including methotrexate (MTX), are at risk of developing lymphoproliferative disorder (LPD). Herein, we report the case of a 61-year-old man who has been treated with MTX and sulfasalazine for seropositive RA since the age of 52. He underwent diffusion-weighted whole-body imaging with background signal suppression (DWIBS), which revealed high-intensity lesions in the affected lymph nodes of the cervical, clavicular, and axillary regions. Follow-up DWIBS after MTX withdrawal showed the suppression or disappearance of the high-intensity lesions. This case demonstrates the potential of DWIBS as a new standard imaging modality for MTX-LPD in patients with RA in clinical practice.

Pyoderma gangrenosum (PG) is a rare inflammatory skin disorder, categorized under neutrophilic dermatoses. It can be idiopathic or associated with underlying conditions like inflammatory bowel disease, autoimmune diseases, and certain cancers. Some medications, including TNF-alpha inhibitors like adalimumab, can also induce this paradoxical reaction. We describe the case of a 19 year old male with adalimumab induced PG which was successfully treated with methotrexate, contributing to the understanding of drug-induced PG and alternative treatment strategies.

The patient was a 48-year-old man who had developed acute myocardial infarction 3 years earlier. He started experiencing recurrent attacks of abdominal pain 2 years earlier. One month before the presentation, he developed perforative peritonitis, which was treated with right hemicolectomy. Preoperative computed tomography revealed systemic thrombotic aneurysms and fibrinoid necrotizing vasculitis was detected in the vessels in the serosa of the resected intestinal specimen. These findings led to a diagnosis of polyarteritis nodosa. Despite the start of remission induction therapy with high-dose glucocorticoid and intermittent intravenous cyclophosphamide, the effect of immunosuppressive therapy was limited. Approximately 1 month after treatment initiation, he died from small intestinal perforation. Polyarteritis nodosa often exhibits nonspecific clinical symptoms, which make an early diagnosis difficult in some cases. Although the prognosis depends on the presence of ischemic lesions due to a ruptured aneurysm or intra-aneurysmal thrombi, it is not rare for the diagnosis to be made following acute myocardial infarction or acute abdominal pain. In young patients with ischemic organ dysfunction without any arteriosclerotic lesions at low risk of developing cardiovascular events, early diagnosis can be made by performing a whole-body examination with a differential diagnosis of polyarteritis nodosa.

A woman in her 60s presented with erythema on both elbows, dyspnea on exertion, discomfort, and swelling of the left upper extremity, followed by swelling and myalgia of the right upper and lower extremities. She was diagnosed with anti-TIF1γ antibody-positive dermatomyositis with interstitial pneumonia. The initial screening tests for malignant diseases including contrast-enhanced CT, upper and lower endoscopy, and gynecological examination did not reveal any obvious abnormalities. The patient experienced two recurrent episodes of muscle weakness and dysphagia during treatment with intravenous glucocorticoids and cyclophosphamide. Five months after diagnosis, a bone marrow biopsy and positron emission tomography- computed tomography (PET-CT) scan revealed a coronary malignant lymphoma with suspected systemic metastasis. Although chemotherapy was initiated, the patient ultimately succumbed to alveolar hemorrhage. Coronary lymphoma is very rare and there has been no report of a cases associated with myositis. PET-CT may be useful for searching malignancy in anti-TIF1γ antibody-positive dermatomyositis cases of recurrent relapse.

Drug-induced liver injury (DILI) is a major concern associated with the use of avacopan, a new therapeutic agent for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Here, we report a rare instance of liver biopsy performed for persistent liver injury following discontinuation of avacopan in an elderly woman with microscopic polyangiitis (MPA). The patient developed cholestatic liver injury after 2 months of avacopan therapy, which led to drug cessation. Despite the discontinuation of avacopan followed by supportive care, the liver enzyme levels remained elevated, necessitating liver biopsy. Histological examination revealed residual inflammation and focal necrosis around the portal vein, indicating ongoing liver injury despite avacopan withdrawal. This case report highlights the hepatotoxic potential of avacopan, the importance of vigilant liver function monitoring, and the value of liver biopsy in the chronic phase of avacopan-induced liver injury. Further research is required to elucidate avacopan hepatotoxicity mechanisms and identify potential risk factors for DILI in patients with AAV.