{"title":"Paradoxical pyoderma gangrenosum secondary to adalimumab successfully treated with methotrexate: a case report.","authors":"Kristy El Morr, Mohamad Ali Rida","doi":"10.1093/mrcr/rxae073","DOIUrl":"https://doi.org/10.1093/mrcr/rxae073","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2024-12-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142808382","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"An autopsy of a patient with polyarteritis nodosa who developed fatal intestinal perforation accompanied by systemic multiple aneurysms and arterial thrombosis.","authors":"Nao Tsugita, Ippei Miyagawa, Masanobu Ueno, Miyabi Takahashi, Shumpei Kosaka, Shingo Nakayamada, Yoshiya Tanaka","doi":"10.1093/mrcr/rxae079","DOIUrl":"https://doi.org/10.1093/mrcr/rxae079","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2024-12-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142808998","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Macrophage migration inhibitory factor as a potential 'missing important factor' driving inflammatory arthritis in adrenocorticotropic hormone deficiency.","authors":"Lisa Wang, Akihiro Nakamura","doi":"10.1093/mrcr/rxae070","DOIUrl":"10.1093/mrcr/rxae070","url":null,"abstract":"","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2024-11-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142831592","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Taiki Ando, Yoshiyuki Abe, Ken Yamaji, Ryuta Nishikomori, Naoto Tamura
{"title":"A case of cryopyrin-associated periodic syndrome due to somatic mosaic mutation complicated with recurrent circinate erythematous psoriasis.","authors":"Taiki Ando, Yoshiyuki Abe, Ken Yamaji, Ryuta Nishikomori, Naoto Tamura","doi":"10.1093/mrcr/rxad067","DOIUrl":"10.1093/mrcr/rxad067","url":null,"abstract":"<p><p>Cryopyrin-associated periotic syndrome (CAPS) is a rare autoinflammatory disease caused by genetic variants in innate immunity genes. Autoinflammatory diseases, including CAPS, mediate proinflammatory cytokines such as interleukin (IL)-1 and IL-18 and result in severe systemic inflammation. A gain-of-function mutation in the NLR family pyrin domain-containing 3 (NLRP3) gene, which encodes the protein cryopyrin, was identified to be responsible for CAPS in 2001, and since then several additional pathogenic mutations have been found. Moreover, other phenotypes have been identified based on severity and symptomatology, including familial cold autoinflammatory syndrome, Muckle-Wells syndrome, and neonatal-onset multisystem inflammatory disease/chronic neurologic cutaneous articular syndrome. Prompt diagnosis of CAPS remains challenging, however, due to unspecific, extensive clinical signs, and delayed diagnosis and treatment targeting IL-1 lead to multiorgan damage. Another factor complicating diagnosis is the existence of somatic mosaic mutations in the NLRP3 gene in some cases, resulting in symptoms and clinical courses that are atypical. The frequency of somatic mosaic mutations in CAPS was estimated to be 19% in a systematic review. Psoriasis is a chronic inflammatory skin disease that affects ∼3% of the global population. Although no reports have shown complication between CAPS and psoriasis, these diseases have several similarities and potential relationships, for instance activation of T helper 17 cells in the dermis and increased NLRP3 gene expression in psoriatic skin compared with normal skin. Here, we report a case of CAPS due to a somatic mosaic mutation with recurrent circinate erythematous psoriasis.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":"368-372"},"PeriodicalIF":0.9,"publicationDate":"2024-07-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138465246","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Neurofascialvascular training for the treatment of Raynaud's phenomenon: A case report.","authors":"Paolo Bertacchini","doi":"10.1093/mrcr/rxae026","DOIUrl":"10.1093/mrcr/rxae026","url":null,"abstract":"<p><p>Primary Raynaud's phenomenon (PRP) is characterised by episodic, reversible, and disabling vasospasms of the peripheral arteries. In the most severe cases, it can lead to ulceration of the fingers and toes. Neuro fascial VascularTraining (NFVT) is a novel therapeutic approach for treating PRP. NFVT aims to enhance peripheral circulation and stimulate the autonomic nervous system (ANS) by engaging multiple physiological mechanisms simultaneously. This integrated approach works to reduce vasospasms and alleviate associated symptoms through neurodynamic and myofascial interventions. A 54-year-old woman, who has experienced pain and hypoesthesia in her hands for 9 years, received a diagnosis of PRP without systemic sclerosis in 2014. The patient reported daily colour changes in her fingers, along with pain and a temporary decrease in tactile sensitivity. The patient engaged in ten 30-minute exercise sessions, and the clinical outcomes were assessed based on several parameters. These included the frequency and duration of vasospastic attacks, evaluated using the Raynaud Condition Score, as well as pain and tingling, measured through the daily Numeric Rating Scale. The Composite Autonomic Symptom Score (COMPASS 31) was utilised to assess dysautonomia, while the frequency of medication use and the Disabilities of the Arm, Shoulder, and Hand questionnaire were also considered. The results indicated a significant improvement in symptoms. NFVT improved symptoms and motor dysfunction in a patient with Raynaud's syndrome, demonstrating how NFVT can increase peripheral blood flow, stimulate the ANS, and improve symptoms in PRP.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":"302-309"},"PeriodicalIF":0.9,"publicationDate":"2024-07-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140900759","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Painful snapping of the middle finger caused by hyperplasia of the ulnar lateral band: A case report.","authors":"Anna Matsuo, Taku Suzuki, Hiroo Kimura, Noboru Matsumura, Takuji Iwamoto, Masaya Nakamura","doi":"10.1093/mrcr/rxad073","DOIUrl":"10.1093/mrcr/rxad073","url":null,"abstract":"<p><p>Snapping of fingers can be caused by pathologies such as stenosing flexor tenosynovitis. However, snapping symptoms in the metacarpophalangeal (MP) joint caused by hypertension and hyperplasia of the lateral band are rare. We present a 26-year-old female with symptoms of painful snapping of the middle finger. When the finger was actively flexed from the hyperextension of the MP joint, the ulnar lateral band was prominent, and a snapping phenomenon occurred. The cause of the snapping finger was considered to be tightness of the ulnar lateral band, and surgery was planned. Intraoperatively, the ulnar lateral band was tense and hyperplastic. The snapping phenomenon disappeared immediately after the resection of the lateral band. It is important to consider this condition as one of the differential diagnoses of snapping finger when the patient complains of an atypical snapping phenomenon.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":"391-393"},"PeriodicalIF":0.9,"publicationDate":"2024-07-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139467384","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Improvement of active salivary gland ultrasonography findings in Sjögren's syndrome in response to short-term glucocorticoid treatment: A case report and review of the literature.","authors":"Takashi Kida, Yutaka Kawahito, Yusuke Suzuki, Shigenori Tamaki, Ikuko Tanaka","doi":"10.1093/mrcr/rxae002","DOIUrl":"10.1093/mrcr/rxae002","url":null,"abstract":"<p><p>Salivary gland ultrasonography is a non-invasive imaging technique that helps in the diagnosis and assessment of disease activity in Sjögren's syndrome. However, it remains unclear whether the salivary gland ultrasonography findings are reversible in response to treatment. We present a case of a woman in her 20s who presented with parotid swelling and pain lasting for 3 months. The patient was diagnosed with anti-SS-A antibody-positive Sjögren's syndrome with active sialadenitis, and short-term glucocorticoid treatment resulted in resolution of clinical symptoms and improvement of salivary gland ultrasonography findings by the Outcome Measures in Rheumatology Clinical Trials scoring system. Notably, the anechoic/hypoechoic foci and vascular signals in the parotid and submandibular glands were reduced after treatment. Furthermore, peak systolic blood flow velocity of the facial artery entering the submandibular gland was decreased. Our case highlights that the findings of 'inflammatory' structural changes and vascularisation on salivary gland ultrasonography, including the Outcome Measures in Rheumatology Clinical Trials scoring system, reflect the disease active of Sjögren's syndrome and are reversible with treatment. Salivary gland ultrasonography has the potential to be a useful tool for monitoring treatment response and stratifying patients by disease activity in Sjögren's syndrome; therefore, further research is needed on the relationship of salivary gland ultrasonography findings with the pathophysiological mechanisms of sialadenitis and long-term clinical outcomes.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":"286-290"},"PeriodicalIF":0.9,"publicationDate":"2024-07-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139473128","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Avacopan's potential to decrease MPO-ANCA titres concurrent with ameliorated activity in ANCA-associated vasculitis.","authors":"Tomoki Taniguchi, Ryosuke Hiwa, Mikihito Shoji, Eriho Yamaguchi, Mirei Shirakashi, Hideo Onizawa, Hideaki Tsuji, Koji Kitagori, Ran Nakashima, Shuji Akizuki, Akira Onishi, Hajime Yoshifuji, Masao Tanaka, Akio Morinobu","doi":"10.1093/mrcr/rxae016","DOIUrl":"10.1093/mrcr/rxae016","url":null,"abstract":"<p><p>Avacopan, an orally administered C5a receptor antagonist, is effective in microscopic polyangiitis via the inhibition of neutrophil priming induced by C5a. However, the exact effect of avacopan on the production of myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA) is yet to be clearly established. This report presents a microscopic polyangiitis patient without major organ damage where high levels of MPO-ANCA persisted with high-dose steroid therapy and azathioprine, but the addition of avacopan led to a reduction in MPO-ANCA titres. The present case implies that avacopan-mediated inhibition of C5a may lead to a reduction in MPO-ANCA levels, thereby potentially ameliorating the pathophysiology of ANCA-associated vasculitis. Nevertheless, the impact of avacopan on MPO-ANCA production cannot be asserted solely based on this report; therefore, further examination is necessary through subgroup analysis using data from larger-scale studies.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":"314-317"},"PeriodicalIF":0.9,"publicationDate":"2024-07-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140873989","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Hypothermia, bradycardia, and hypotension during glucocorticoid or cyclosporine A therapy in a boy with Kikuchi-Fujimoto disease.","authors":"Yasuyoshi Hiramatsu, Kazuki Takahashi, Masaki Shimomura, Kota Taniguchi, Yuka Okura, Mitsuru Nawate, Yutaka Takahashi, Ichiro Kobayashi","doi":"10.1093/mrcr/rxae023","DOIUrl":"10.1093/mrcr/rxae023","url":null,"abstract":"<p><p>Kikuchi-Fujimoto disease (KFD) is an inflammatory disease of unknown aetiology characterised by fever and cervical lymphadenopathy. Although KFD is a self-limiting disease, patients with severe or long-lasting course require glucocorticoid therapy. We presently report a 17-year-old boy with KFD who had seven relapses since the onset at 4 years old. He suffered from hypothermia, bradycardia, and hypotension during the treatment with prednisolone or methylprednisolone. All of his vital signs recovered after cessation of the drug in addition to fluid replacement and warming. Thus, glucocorticoid was effective but could not be continued because of the adverse event. Although hypothermia developed during the treatment with 5 mg/kg/day of cyclosporine A (CsA) at his second relapse, he was successfully treated with lower-dose CsA (3 mg/kg/day). Thereafter, he had five relapses of KFD until the age of 12 years and was treated by 1.3-2.5 mg/kg/day of CsA. Hypothermia accompanied by bradycardia and hypotension developed soon after concomitant administration of ibuprofen at his fifth and sixth relapses even during low-dose CsA therapy. Conclusively, glucocorticoid, standard dose of CsA, or concomitant use of non-steroidal anti-inflammatory drugs may cause hypothermia, bradycardia, and hypotension and needs special attention. Low-dose CsA could be a choice for such cases with KFD.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":"352-356"},"PeriodicalIF":0.9,"publicationDate":"2024-07-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141082923","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Reverse V-shaped osteotomy for ankylosing rocker-bottom foot deformity in patients with rheumatoid arthritis: A report of three cases.","authors":"Takaaki Noguchi, Makoto Hirao, Kosuke Ebina, Yuki Etani, Gensuke Okamura, Hideki Tsuboi, Atsushi Goshima, Seiji Okada, Jun Hashimoto","doi":"10.1093/mrcr/rxae005","DOIUrl":"10.1093/mrcr/rxae005","url":null,"abstract":"<p><p>This study evaluated a reverse V-shaped osteotomy for ankylosing rocker-bottom foot deformity in patients with rheumatoid arthritis. Three feet were presented in this study: rheumatoid rocker-bottom deformities with painful and/or infectious bony prominence towards the bottom of the foot, treated with a reverse V-shaped osteotomy in the mid-hindfoot. In all three cases, significant correction was achieved with restoration of the medial longitudinal arch, and improvement in clinical scores was confirmed. Reverse V-shaped osteotomy has the potential to be a useful and definitive procedure for ankylosing rocker-bottom deformity in patients with rheumatoid arthritis.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":"249-254"},"PeriodicalIF":0.9,"publicationDate":"2024-07-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139521423","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}