BACKGROUND: Epstein-Barr virus-positive mucocutaneous ulcer is one of the mature B-cell lymphoproliferative diseases occurring in patients with immune dysfunction including those with immunosuppressive treatment such as methotrexate.
METHODS: A Japanese elderly man in his 80s with rheumatoid arthritis on methotrexate was admitted to our hospital complaining persistent pharyngeal pain. Laboratory tests revealed severe pancytopenia, elevated C-reactive protein, and increased creatinine levels. An otolaryngological examination showed ulceration of the right tonsil, from which diagnostic biopsy was performed. The diagnosis of Epstein-Barr virus-positive mucocutaneous ulcer was made and bone marrow aspiration revealed hypocellularity and megaloblastic changes. Pancytopenia was improved after discontinuing methotrexate, and repeated bone marrow aspiration test revealed recovery of normal cellularity and disappearance of dysplasia, confirming the diagnosis of methotrexate intoxication. Tonsil ulcer was improved only with discontinuation of methotrexate, which strongly supported the diagnosis of EBV-MCU.
CONCLUSIONS: Our case suggested that even this best prognosis form of lymphoproliferative disease could lead to fatal complications if not appropriately managed.

A 78-year-old woman with rheumatoid arthritis, who was started on baricitinib five or six months earlier, was referred to our hospital due to a subcutaneous abscess in her right axilla. Contrast-enhanced chest, abdomen, and pelvis computed tomography showed subcutaneous abscesses in her right axilla and lymphadenopathy with calcification. Cultures from the subcutaneous abscess and skin biopsy specimens were positive for Mycobacterium tuberculosis. These findings led to the diagnosis of scrofuloderma associated with tuberculous lymphadenitis. She was started on an antitubercular regimen of isoniazid, rifampicin, pyrazinamide, and ethambutol as the initial phase treatment (first 2 months), followed by isoniazid and rifampicin for 4 months (total 6 months). After 6 months of antitubercular treatment, the abscesses and lymphadenitis disappeared. Although cases of tuberculosis during JAK inhibitor treatment are rare, they are serious adverse events that require caution.

Actinomycosis is a rare chronic granulomatous disease characterized by granuloma formation and tissue fibrosis with sinus tracts, often misdiagnosed due to its similarity to many infectious and non-infectious diseases. This report presents a case of a 60-year-old female with more than 10 years history of rheumatoid arthritis who developed actinomycosis infection after long-term treatment with immunosuppressants and biologics, including methotrexate, leflunomide, and infliximab, leading to recurrent joint pain, poorly controlled rheumatoid arthritis activity, and persistent elevation of white blood cell counts. Abdominal CT revealed a pelvic mass and right ureteral dilation. Pathological examination of cervical tissue showed significant neutrophil infiltration and sulfur granules, indicating actinomycosis. The patient received 18 months of doxycycline treatment for the infection and continued rheumatoid arthritis therapy with leflunomide, hydroxychloroquine sulfate, and tofacitinib, resulting in improved joint symptoms and normalized white blood cell counts. After 2 years of follow-up, the patient remained stable with no recurrence. This case highlights the importance of clinicians being vigilant for infections, particularly chronic, occult infections from rare pathogens, in rheumatoid arthritis patients on potent immunosuppressants and biologics, advocating for early screening and diagnosis.
放线菌病是一种少见的慢性肉芽肿性疾病，以肉芽肿及组织纤维化窦道的形成为特点，其症状与许多传染性和非传染性疾病相似，临床上经常被误诊。现报告1例长期使用免疫抑制剂及生物制剂治疗的类风湿关节炎合并放线菌感染的患者。该患者为60岁女性，类风湿关节炎病程10余年，既往使用氨甲蝶呤、艾拉莫德、英夫利昔单抗等多种免疫抑制剂及生物制剂治疗，关节肿痛反复发作，对类风湿关节炎疾病活动度的控制不佳，白细胞计数持续升高。全腹部CT示盆腔占位，右侧输尿管扩张。对宫颈管刮出组织进行病理检查，可见大量中性粒细胞浸润和硫磺样颗粒，考虑放线菌感染。予多西环素抗感染治疗18个月，以及艾拉莫德、硫酸羟氯喹及托法替布抗风湿治疗后，患者关节肿痛好转，白细胞计数正常。随访2年，患者病情稳定，无复发。类风湿关节炎患者使用较强免疫抑制剂、生物制剂时，应警惕合并发生感染，一些慢性、隐匿部位及少见病原体的感染也应当引起临床医生的重视，并尽早进行相关筛查以明确诊断。.

BACKGROUND: Cryptococcosis is an infectious disease caused by encapsulated heterobasidiomycete yeasts. As an opportunistic pathogen, cryptococcal inhalation infection is the most common. While Primary cutaneous cryptococcosis is extremely uncommon.
METHODS: A 61-year-old woman with a history of rheumatoid arthritis on long-term prednisone developed a red plaque on her left thigh. Despite initial antibiotic treatment, the erythema worsened, leading to rupture and fever. Microbiological analysis of the lesion\'s secretion revealed Candida albicans, Pseudomonas aeruginosa, and methicillin-resistant Staphylococcus epidermidis. Skin biopsy showed thick-walled spores, and culture confirmed primary cutaneous infection with Cryptococcus neoformans. Histopathological stains were positive, and mass spectrometry identified serotype A of the pathogen. The patient was treated with oral fluconazole and topical nystatin, resulting in significant improvement and near-complete healing of the skin lesion within 2.5 months.
CONCLUSIONS: Primary cutaneous cryptococcosis was a primary skin infection exclusively located on the skin. It has no typical clinical manifestation of cutaneous infection of Cryptococcus, and culture and histopathology remain the gold standard for diagnosing. The recommended medication for Primary cutaneous cryptococcosis is fluconazole. When patients at risk for opportunistic infections develop skin ulcers that are unresponsive to antibiotic, the possibility of primary cutaneous cryptococcosis needs to be considered.

This report presents a unique case of Caplan syndrome that mimicked accelerated progressive massive fibrosis. The patient, a former coal miner, had been diagnosed with coal worker\'s pneumoconiosis 15 years prior and had been treated for rheumatoid arthritis for over 20 years. Accelerated progressive massive fibrosis and the development of multiple nodules with cavitation in the basal lungs were subsequently observed on serial CT scans. Here, the CT manifestations of Caplan syndrome are highlighted in a case in which Caplan syndrome mimicked accelerated progressive massive fibrosis.
본 증례 보고에서는 가속화된 진행성거대섬유증으로 오인된 카플란 증후군을 소개한다. 환자는 15년 전 탄광부진폐증을 진단받고 20년 이상 류마티스관절염 치료를 받은 자로 연속적인 CT 검사에서 진행거대섬유증의 진행과 함께 폐기저부에 동공을 동반한 다발성 폐결절이 관찰되었다. CT상 가속화된 진행성거대섬유증 소견으로 오인할 수 있는 카플란 증후군 증례를 보고하고자 한다.

Cytoplasmic T Lymphocyte Antigen-4 (CTLA-4) gene encodes for a glycoprotein, expressed on activated T-cells to transfer an inhibitory signal to control T-cell activation and proliferation. Techniques coupled with Real-time Polymerase Chain Reaction (PCR) and High-Resolution Melting Analysis (HRMA) were used to screen a missense signal peptide polymorphism (CTLA-4 + 49 A/G rs231775) in the Indian population to detect its association with Rheumatoid Arthritis (RA). Further, the resulting outcome was confirmed by Sanger\'s sequencing technique, and genotype frequencies were calculated. In eukaryotic cells, the M domain of the Signal Recognition Particle (SRP-54) recognizes the N-terminal region of the Signal Peptide (SP) sequence. SP directs the polypeptide chain into the Sec-61 translocon of the Endoplasmic Reticulum (ER) for further protein modification. As the Single Nucleotide Polymorphism (SNP) rs231775 lies in the signal peptide region of CTLA-4, an in-silico study was also performed to predict the mRNA stability and SP-SRP protein interaction. From the study, it was observed that the genotype frequency of rs231775 SNP G/G homozygous dominant was significantly higher in RA patients than G/A heterozygous dominant and A/A homozygous recessive conditions (Odd Ratio (OR) = 2.0862; 95 % Confidence Interval (C.I) = 1.2584 to 3.4584; Relative Risk (RR) = 1.8507; p = 0.0044). Moreover, the rs231775 SNP G allele frequency was higher in RA than the control group G = 0.407 (40.7 %) vs 0.32 (32 %). In silico approaches of Protein-Protein docking and Molecular Dynamics (MD) simulation reveal CTLA-4 rs231775 SNP (G allele) has destabilized the SP-SRP protein complex, which may affect the translocation of CTLA-4 nascent polypeptide chains into the ER via activating Regulation of Aberrant Protein Production (RAPP) pathway.

BACKGROUND: Rheumatoid arthritis (RA) is a systemic inflammatory disease characterized by joint inflammation and various extra-articular manifestations, including rheumatoid nodules (RNs). This case study aims to explore the effectiveness of alternative treatments for RNs, particularly highlighting the therapeutic potential of sulfasalazine.
METHODS: A 52-year-old male with established RA presented with worsening joint pain and firm nodules on his elbows, feet, and fingers.
METHODS: The patient fulfilled the diagnostic criteria for RA and was diagnosed with methotrexate-induced RNs based on their temporal association with methotrexate initiation.
METHODS: Methotrexate was discontinued and a combination of leflunomide and sulfasalazine was initiated. Sulfasalazine led to improvement in both joint pain and nodule size. However, due to cost concerns, the patient discontinued sulfasalazine, resulting in a resurgence of both symptoms and nodule enlargement. Reintroduction of methotrexate resulted in significant improvement in joint inflammation, and notably, no new nodules developed at 6 months follow-up.
RESULTS: Sulfasalazine demonstrated efficacy in managing RA nodules, suggesting a potential alternative therapy.
CONCLUSIONS: The case highlights the complex etiology of nodules in RA and emphasizes the importance of individualized treatment approaches and close monitoring for optimal management.

A patient with longstanding rheumatoid arthritis (RA) complained of spinal cord symptoms after RA relapse. Contrast MRI demonstrated neuromyelitis in the upper thoracic spinal cord, and anti-aquaporin-4 (anti-AQP4) antibody was positive in the serum and cerebrospinal fluid (CSF). Neuromyelitis optica spectrum disorder (NMOSD) was diagnosed after excluding central nervous system (CNS) infection and tumor, and spinal cord symptoms were relieved after high dose of glucocorticoid and immunosuppressant were initiated for treatment.

The Receptor Activator Nuclear of κB Ligand (RANKL) plays an important function in immune responses, activating osteoclast cells and unchanged bone resorption, which in turn leads to bone erosion and inflammation. Genetic variants in the promoter region of the RANKL gene could lead to a higher risk of rheumatoid arthritis (RA).
OBJECTIVE: To assess the association of rs9533155 (-693C>G) and rs9533156 (-643T>C) genetic variants with RA risk.
METHODS: A case-control study was carried out. A total of 94 patients with RA (RA group) and 134 subjects without any rheumatologic disease (control group) were included. Genetic DNA was extracted from peripheral white blood cells (leukocytes). Genetic variant rs9533155 (-693C>G) was screened by an approach based on Polymerase Chain Reaction-Restriction Fragment Length Polymorphism (PCR-RFLP), while rs9533156 (-643T>C) was screened using quantitative polymerase chain reaction (qPCR) with TaqMan probes. RANKL serum levels were measured by ELISA.
RESULTS: For rs9533155 (-693C>G), the polymorphic homozygous genotype frequencies (CC) were higher in the RA group (p = 0.006). Individuals carrying the risk genotype presented higher levels of serum RANKL. Carriers of the polymorphic homozygous genotype in the dominant model (CC vs. CG + GG) had an increased risk of developing RA (OR: 1.8, 95% CI 1.04 to 3.1). No association between rs9533156 (-643T>C) and the haplotypes with RA risk was observed.
CONCLUSIONS: The rs9533155 (-693C>G) genetic variant exhibits a potential role in RA risk. The studied population had no association with the rs9533156 (-643T>C) genetic variant.

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