Modern rheumatology case reports最新文献

Microscopic polyangiitis (MPA) is a subtype of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), a small-vessel vasculitis that can cause organ-threatening complications. Hypertrophic pachymeningitis is a rare, central nervous system manifestation of AAV rarely involving the spine. We herein report a patient with myeloperoxidase-ANCA-positive MPA presenting with progressive cognitive decline and gait disturbance associated with communicating hydrocephalus secondary to spinal hypertrophic pachymeningitis. The patient responded well to high-dose prednisolone and rituximab and showed significant clinical and radiological improvement without surgery. The present case not only demonstrated that spinal hypertrophic pachymeningitis in AAV can cause reversible dementia associated with communicating hydrocephalus but also highlighted the potential of timely immunosuppressive therapy to induce remission.

SAPHO syndrome is a rare inflammatory osteoarticular disorder, which includes autoimmune diseases such as pustulotic arthro-osteitis, inflammatory bowel disease-associated spondyloarthritis, and psoriatic arthritis. There are few reports on the treatment of SAPHO syndrome that presents with bone destruction in the spine. We present a case in which adalimumab (ADA) was administered to treat destruction of the lumbar vertebral endplates caused by SAPHO syndrome. The patient was a woman in her 20s who was referred to Toho University Sakura Medical Center with complaints of low back pain; acne on the face, anterior chest, and back; and sternoclavicular joint pain. Blood tests showed a mild increase in C-reactive protein but negative results for rheumatoid factor and anti-cyclic citrullinated peptide antibody. Radiographs and computed tomography images demonstrated destruction with surrounding bone sclerosis in the cranial endplates of the L4 and L5 vertebrae and the left sternoclavicular joint. The Ankylosing Spondylitis Disease Activity Score was 2.05, and the Bath Ankylosing Spondylitis Functional Index was 3.00. Despite the use of the maximum dose of nonsteroidal anti-inflammatory drugs, her symptoms did not improve, and ADA was administered at a dose of 40 mg every 2 weeks. After ADA administration, both the Ankylosing Spondylitis Disease Activity Score and the Bath Ankylosing Spondylitis Functional Index were immediately reduced and low disease activity or remission was maintained thereafter. After 3 years, the computed tomography images showed no progression of bone destruction in the lumbar vertebrae and sternoclavicular joint, and the patient was completely free from low back pain and was able to run normally.

We describe a rare case of synovial chondromatosis in the subacromial bursa that led to rotator cuff damage and progressive glenohumeral osteoarthritis. A 66-year-old woman initially presented with shoulder pain and limited motion, and imaging revealed a loose body in the subacromial bursa and partial-thickness rotator cuff tear. Conservative treatment was initially effective, but symptoms recurred after migration of the loose body into the rotator cuff. Arthroscopic removal and partial synovectomy were performed, resulting in symptom relief. Histology confirmed benign osteochondroma. This case highlights the importance of early surgical intervention to prevent irreversible rotator cuff damage in similar cases.

We report a fatal case of hepatic portal venous gas (HPVG) following percutaneous endoscopic gastrostomy (PEG) and initiation of enteral nutrition in a 57-year-old Japanese woman with systemic sclerosis-myositis overlap syndrome complicated by pneumatosis cystoides intestinalis (PCI). She had interstitial lung disease with right heart strain, congestive heart failure, and respiratory muscle fatigue, requiring mechanical ventilation. After improvement in ventilatory failure, PEG was performed, and enteral feeding was initiated. Transient abdominal pain and recurrent vomiting developed, and computed tomography (CT) revealed intramural gastric gas and extensive HPVG. Based on the clinical course, absence of peritoneal irritation, and postmortem CT findings, gastrointestinal ischaemia or necrosis was considered unlikely. The abdominal pain was attributed to gastric overdistension with intramural dissection from luminal gas entry, while HPVG was thought to result from gas tracking from the PEG site into the portal system. The presumed mechanism involved delayed wound healing at the PEG site due to systemic sclerosis-related gastric involvement, long-term glucocorticoid therapy, and malnutrition, combined with elevated intragastric pressure from routine feeding advancement and peristalsis, enabling luminal gas to dissect into the wall. The marked reduction of PCI findings when HPVG was detected suggested that gas from PCI may have contributed to its formation. Although the exact cause of death could not be determined, gas embolism was suspected. This case underscores the need for extreme caution when initiating enteral nutrition in similar patients.

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 necrotising vasculitis was detected in the vessels of 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 ischaemic 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 iscahemic 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.

Polyarteritis nodosa (PAN) is a rare systemic necrotising vasculitis that can lead to the formation of refractory lower limb ulcers requiring amputation. The standard treatment for severe PAN involves combination therapy with steroids and cyclophosphamide; however, some cases prove to be challenging. Recently, case reports have described the use of biological agents for PAN treatment. We present the case of a 61-year-old Japanese man with cutaneous PAN and refractory recurrent lower limb ulcers. In 2017, the patient was admitted to the hospital because of exacerbation of a right lower limb ulcer. Despite combination therapy with corticosteroids, cyclophosphamide, and endovascular therapy, the gangrene in the right lower leg progressed, and amputation was performed. The patient was temporarily stabilised with prednisolone monotherapy. In 2019, new ulcers were observed on the left lower limb. Owing to steroid resistance, subcutaneous tocilizumab (162 mg/week) was initiated. Over a few months, the ulcer healed completely, and left lower limb amputation was avoided. Therefore, tocilizumab could potentially be one of the treatment options for severe cases in the future.

Pyoderma gangrenosum (PG) is a rare inflammatory skin disorder, categorised under neutrophilic dermatoses. It can be idiopathic or associated with underlying conditions like inflammatory bowel disease, autoimmune diseases, and certain cancers. Some medications, including tumour necrosis factor-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 coexistence of Takayasu arteritis (TA) and chronic myeloid leukaemia (CML) is extremely rare, with most reported cases occurring either concurrently or during active disease. We report a unique case of TA developing during a deep molecular response (MR) of CML. A 28-year-old female was diagnosed with CML in December 2020 and achieved a deep MR with bosutinib treatment. After 3.5 years of successful CML treatment, she developed symptoms including fatigue, low-grade fever, and chest pain. Imaging revealed wall thickening of multiple large vessels characteristic of TA. The patient responded well to prednisolone therapy while maintaining the MR of CML. A review of published cases identified eight previous reports of TA associated with myeloid disorders. Our case is distinctive for the development of TA during sustained deep MR of CML, contrasting with previous reports where vasculitis typically occurred during active disease or at initial presentation. This case highlights the importance of monitoring for vasculitic complications in CML patients, even during MR. The temporal relationship between these conditions suggests that inflammatory mechanisms leading to vasculitis might persist or develop independently of CML disease activity, challenging the conventional understanding of their association as a purely paraneoplastic phenomenon.

A patient in his late seventies presented with dizziness, loss of appetite, weight loss and iron deficiency anaemia. Computed tomography of the chest, abdomen, and pelvis found a descending colon adenocarcinoma. Further assessment with whole body fluorodeoxyglucose positron emission tomography/computed tomography incidentally identified extensive large vessel arteritis. The patient opted for no immunosuppressive or steroid treatments for his vasculitis to minimise risk associated with planned left hemicolectomy and en bloc resection of the small bowel. Following surgical resection of the tumour, the large vessel vasculitis was seen to resolve with improvement of inflammatory markers and minimal vessel wall fluorodeoxyglucose uptake on subsequent postoperative positron emission tomography/computed tomography.

We report a case of a 64-year-old Japanese man who developed IgG4-related pericoronary arteritis following mRNA-based COVID-19 vaccination. The patient presented with anterior chest pain and imaging revealed perivascular soft tissue thickening around the coronary arteries, along with pancreatic enlargement, enlarged prostate, and periaortitis. Laboratory tests showed markedly elevated serum IgG4 levels (1740 mg/dl). Histopathological findings from prostate biopsy were consistent with IgG4-related disease. Notably, retrospective analysis of preserved serum samples demonstrated a sharp increase in IgG4 levels following the third BNT162b2 vaccine dose, suggesting a possible link between vaccination and disease onset. The patient responded to prednisolone, with significant clinical and radiological improvement. However, IgG4 levels rebounded during tapering, requiring additional immunosuppressive therapy with azathioprine and planned rituximab treatment. This case represents the first documented instance tracking longitudinal changes in serum IgG4 levels from prevaccination through disease onset. While a definitive causal relationship between COVID-19 vaccination and IgG4-related disease remains unproven, this case highlights the need for further investigation into the potential immunopathogenic mechanisms involved.