Pablo Lorenzo Barreto, Garbiñe Roy Ariño, Fernando Pérez Trapote, Alberto Sáez Marín, Erik S Stiauren Fernández, Beatriz Zarza Sanz, Nuria García Barragán, Carlos de la Puente Bujidos, F Javier Buisán Catevilla
{"title":"Rheumatoid meningitis in a patient with overlap syndrome: The usefulness of anti-citrullinated peptide antibodies determination in CSF.","authors":"Pablo Lorenzo Barreto, Garbiñe Roy Ariño, Fernando Pérez Trapote, Alberto Sáez Marín, Erik S Stiauren Fernández, Beatriz Zarza Sanz, Nuria García Barragán, Carlos de la Puente Bujidos, F Javier Buisán Catevilla","doi":"10.1093/mrcr/rxad003","DOIUrl":"https://doi.org/10.1093/mrcr/rxad003","url":null,"abstract":"<p><p>Rheumatoid meningitis (RM) is a rare complication of rheumatoid arthritis that can manifest as stroke-like episodes. We present the case of a 63-year-old woman with a past history of overlap syndrome and clinical manifestations suggestive of amyopathic dermatomyositis, rheumatoid arthritis, and systemic lupus erythematosus. She presented to the emergency department with sudden onset right-sided clumsiness and numbness, as well as a 2-week history of left hemicranial headache. Laboratory workup revealed positive serum antinuclear antibodies, anti-Ro antibodies, anti-citrullinated peptide antibodies (ACPA), and elevated rheumatoid factor. Lymphocytic pleocytosis, positive ACPA and anti-Ro antibodies with passive diffusion pattern, and negative microbiological studies were demonstrated in the CSF. Brain magnetic resonance imaging showed predominant left fronto-parieto-occipital leptomeningeal and pachimeningeal enhancement. She was diagnosed with RM and received methylprednisolone IV mg/kg once daily. Stroke-like episodes in the setting of a patient with lymphocytic pleocytosis in the cerebrospinal fluid (CSF) and meningeal enhancement should raise suspicion of RM. In this context, serum rheumatoid factor and ACPA levels should always be measured and ACPA should also be measured in CSF. To our knowledge, this is the first reported case of RM in the context of an overlap syndrome. ACPA levels in CSF could be a relevant diagnostic clue in the setting of central nervous system disturbance and overlapping autoimmune conditions that include rheumatoid arthritis. In our case, the presence of a suggestive clinical scenario of RM reinforces the probable pathogenic role of ACPA when it is present in the central nervous system, even without intrathecal synthesis evidence.</p>","PeriodicalId":18677,"journal":{"name":"Modern Rheumatology Case Reports","volume":"7 2","pages":"347-349"},"PeriodicalIF":0.0,"publicationDate":"2023-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9669768","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":"A case of extrapulmonary tuberculosis after use of baricitinib.","authors":"Tetsuro Shimada, Akira Maeyama, Tomonobu Hagio, Kunihide Muraoka, Terufumi Shibata, Yutaro Yamasaki, Taiga Oda, Makoto Hamasaki, Takuaki Yamamoto","doi":"10.1093/mrcr/rxac098","DOIUrl":"https://doi.org/10.1093/mrcr/rxac098","url":null,"abstract":"<p><p>Extrapulmonary tuberculosis (TB) can occur in patients treated with Janus kinase (JAK) inhibitors. We present a case of rheumatoid arthritis complicated by extrapulmonary TB following baricitinib treatment. A 45-year-old Japanese woman was diagnosed with rheumatoid arthritis at another hospital, and she subsequently started treatment with methotrexate (MTX) at 6.0 mg/week and prednisolone at 3.0 mg/day at our institute. The MTX dose was increased to 10 mg/week, and clinical remission was achieved; however, the disease activity flared up 6 months after the initial visit. Isoniazid (INH) prophylaxis was started following positive T-SPOT® screening for TB, and baricitinib (Olumiant®) was introduced 3 weeks later because of an insufficient response to MTX. INH prophylaxis was continued for 6 months. Ten months after starting INH treatment, a painless mass was observed on the left side of the patient's neck. Magnetic resonance imaging showed enlarged lymph nodes with calcification. A subsequent biopsy and pathologic examination led to a diagnosis of tuberculous lymphadenitis, and the patient was started on anti-TB therapy. Ten months later, the patient was still in remission and doing well. Extrapulmonary TB can be difficult to diagnose because of inconsistent physical and laboratory findings. When treating patients with JAK inhibitors, physicians should be cognisant of the potential for extrapulmonary TB to develop.</p>","PeriodicalId":18677,"journal":{"name":"Modern Rheumatology Case Reports","volume":"7 2","pages":"364-367"},"PeriodicalIF":0.0,"publicationDate":"2023-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9724073","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":"Combined B-cell immunomodulation with rituximab and belimumab in severe, refractory TAFRO syndrome associated with Sjögren's syndrome: A case report.","authors":"Mitsuru Watanabe, Yoichiro Haji, Mirai Hozumi, Yui Amari, Yukina Mizuno, Takanori Ito, Mizuki Kato, Masato Okada","doi":"10.1093/mrcr/rxad020","DOIUrl":"https://doi.org/10.1093/mrcr/rxad020","url":null,"abstract":"<p><p>TAFRO syndrome is a systemic inflammatory disease of unknown aetiology. It is characterised by thrombocytopenia, anasarca, myelofibrosis, renal dysfunction, and organomegaly. Herein, we report the case of a 60-year-old male with TAFRO syndrome. A few weeks after the patient developed an intermittent fever, he presented to our hospital with diarrhoea, abdominal distension, and whole-body oedema (face, extremities, and abdomen). Autoantibody and lip biopsy findings supported the diagnosis of primary Sjögren's syndrome. High-dose steroids and tocilizumab were used to treat his refractory thrombocytopenia and ascites. However, systemic inflammation and renal dysfunction did not improve, resulting in temporary haemodialysis. Eventually, combined B-cell immunomodulation therapy with rituximab and belimumab ameliorated the patient's symptoms. About 16 weeks after discharge, the overall condition of the patient had improved. The TAFRO syndrome may be a severe manifestation of primary Sjögren's syndrome. Considering the immunological context, combined B-cell immunomodulation therapy provides new insights into improving this life-threatening disease and enables rapid steroid tapering.</p>","PeriodicalId":18677,"journal":{"name":"Modern Rheumatology Case Reports","volume":"7 2","pages":"475-479"},"PeriodicalIF":0.0,"publicationDate":"2023-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9671487","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":"Hepatic veno-occlusive disease accompanied by thrombotic microangiopathy developed during treatment of juvenile dermatomyositis and macrophage activation syndrome: A case report.","authors":"Mariko Mouri, Toru Kanamori, Eriko Tanaka, Kanako Hiratoko, Mariko Okubo, Michio Inoue, Tomohiro Morio, Masaki Shimizu, Ichizo Nishino, Naoko Okiyama, Masaaki Mori","doi":"10.1093/mrcr/rxac086","DOIUrl":"https://doi.org/10.1093/mrcr/rxac086","url":null,"abstract":"<p><p>Hepatic veno-occlusive disease (VOD) is a complication of haematopoietic stem cell transplantation. VOD is associated with the occurrence of thrombotic microangiopathy (TMA). In haematopoietic stem cell transplantation, VOD and TMA are endothelial syndromes resulting from endothelial cell activation and dysfunction. In rheumatic disease, while TMA is not rare, there are few reports of VOD. In idiopathic myositis, only one case with VOD and TMA complications has been reported, and there are no published cases in juvenile dermatomyositis (JDM). We report a case of JDM manifesting VOD and TMA complications during the treatment for myositis and macrophage activation syndrome (MAS). A 5-year-old boy diagnosed as anti-nuclear matrix protein 2 antibody-positive JDM was complicated by MAS. He received pulsed methylprednisolone, prednisolone, and tacrolimus, but JDM and MAS progressed. He was then treated with intravenous cyclophosphamide and cyclosporine A, with improvement in myositis symptoms and MAS. After initiation of cyclophosphamide and cyclosporine A, he developed haemolysis, painful hepatomegaly, liver damage, and ascites. He was diagnosed with VOD and TMA. Cyclophosphamide and cyclosporine A were discontinued, with recovery from VOD and TMA. The patient remained well on treatment with methotrexate, without any relapse of JDM and MAS to date. The presence of vasculopathy and hypercytokinaemia because of JDM and MAS exacerbated endothelial cell damage. In the present case, we suggest that the main cause of VOD was medication with CY and CsA, which had been used to treat acute exacerbation of MAS and JDM.</p>","PeriodicalId":18677,"journal":{"name":"Modern Rheumatology Case Reports","volume":"7 2","pages":"404-409"},"PeriodicalIF":0.0,"publicationDate":"2023-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9673530","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}
Koji Suzuki, Mitsuhiro Akiyama, Sho Ishigaki, Yasushi Kondo, Shuntaro Saito, Jun Kikuchi, Hironari Hanaoka, Yuko Kaneko
{"title":"A severe cerebral infarction associated with giant cell arteritis, which developed during tocilizumab therapy and was successfully treated with intravenous cyclophosphamide.","authors":"Koji Suzuki, Mitsuhiro Akiyama, Sho Ishigaki, Yasushi Kondo, Shuntaro Saito, Jun Kikuchi, Hironari Hanaoka, Yuko Kaneko","doi":"10.1093/mrcr/rxad009","DOIUrl":"https://doi.org/10.1093/mrcr/rxad009","url":null,"abstract":"<p><p>Giant cell arteritis (GCA) is a large vessel vasculitis that primarily involves aorta and its major branches. Cerebral infarction is a serious complication that can occur secondary to GCA in up to 3% of patients with a mortality rate of over 50%. Due to the rarity of this severe complication, no therapeutic strategies are currently available. Furthermore, despite the recent progress in molecular-targeted therapy for GCA, it remains unknown whether tocilizumab is effective for severe ischemic complications such as cerebral infarction. The accumulation of individual cases in which this fatal complication could be treated is apparently required to build a better management of the disease. We present our case of GCA that developed severe cerebral infarction during high-dose glucocorticoid and tocilizumab therapy, and its symptoms and image findings were improved by switching to intravenous cyclophosphamide. Our case suggests that an intensive immunosuppressive therapy, including cyclophosphamide, may be necessary to stabilise this fatal complication of GCA.</p>","PeriodicalId":18677,"journal":{"name":"Modern Rheumatology Case Reports","volume":"7 2","pages":"453-457"},"PeriodicalIF":0.0,"publicationDate":"2023-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9724089","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}
Naoki Tanomogi, Takahiro Ota, Daisuke Sato, Shingo Itagaki, Kota Shimada
{"title":"Fatal cerebral venous sinus thrombosis with preceding thrombocytopenia in a patient with new-onset eosinophilic granulomatosis with polyangiitis.","authors":"Naoki Tanomogi, Takahiro Ota, Daisuke Sato, Shingo Itagaki, Kota Shimada","doi":"10.1093/mrcr/rxad004","DOIUrl":"10.1093/mrcr/rxad004","url":null,"abstract":"<p><p>A previously healthy, 44-year-old, female patient was hospitalised for acute abdominal pain and bilateral pneumonia. Eosinophilic granulomatosis with polyangiitis (EGPA) was diagnosed on the basis of eosinophilia, eosinophilic tissue inflammation, polyneuropathy, and bilateral pneumonia. She had a fatal cerebral venous sinus thrombosis following thrombocytopenia, which was apparently caused by platelet consumption. It may have been possible to prevent the deterioration of the venous thrombosis by starting immunosuppressive or anticoagulant therapy earlier. If a patient with EGPA presents with unexplained thrombocytopenia, the physician should assess for physical findings or laboratory abnormalities suggestive of thrombosis. Additionally, if the patient complains of headache or nausea with normal head computed tomography findings, magnetic resonance imaging or magnetic resonance venography should be performed to assess for cerebral venous sinus thrombosis.</p>","PeriodicalId":18677,"journal":{"name":"Modern Rheumatology Case Reports","volume":"7 2","pages":"410-415"},"PeriodicalIF":0.0,"publicationDate":"2023-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9724091","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":"Development of ANCA-associated vasculitis followed by SARS-CoV-2 vaccination in a patient with HLA-DRB1*09:01 allele.","authors":"Takuro Kawamura, Daigo Nakazawa, Saori Nishio, Taiki Isozaki, Maki Komatsumoto, Tatsuya Atsumi","doi":"10.1093/mrcr/rxac093","DOIUrl":"https://doi.org/10.1093/mrcr/rxac093","url":null,"abstract":"<p><p>Coronavirus disease 2019 (COVID-19), caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), presents with severe pneumonia and fatal systemic complications. Currently, SARS-CoV-2 vaccines are effective in reducing the risk of new onset and getting worse of the disease. However, autoimmune diseases such as antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) have been reported to develop after COVID-19 vaccine administration. A 71-year-old woman presented with fever, malaise, urinary abnormalities, and renal dysfunction after receiving the COVID-19 vaccine (Pfizer-BioNTech). We clinically diagnosed AAV with her manifestations and serological test (myeloperoxidase-ANCA-positive). Her clinical findings were improved after immunosuppressive therapy. We examined her genetic susceptibility to AAV, and we found that her allele was HLA-DRB1*09:01, which is a risk allele of myeloperoxidase-AAV. Mechanistically, SARS-CoV-2 vaccines would activate immunity, including neutrophils, and trigger AAV onset in this patient with a genetic risk to develop AAV. The pathophysiology of this case would share with that of autoimmune/inflammatory syndrome induced by adjuvants in the absence of external adjuvants.</p>","PeriodicalId":18677,"journal":{"name":"Modern Rheumatology Case Reports","volume":"7 2","pages":"426-430"},"PeriodicalIF":0.0,"publicationDate":"2023-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9661952","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}
Antonio Tonutti, Edoardo Simonetta, Anna Stainer, Giulia Suigo, Maria De Santis, Carlo Selmi, Chiara Masetti, Ana Lleo, Luigi Maria Terracciano, Stefano Aliberti, Francesco Amati
{"title":"Hepatic and pulmonary involvement in a patient with PR3-ANCA vasculitis following SARS-CoV-2 vaccination: A case report.","authors":"Antonio Tonutti, Edoardo Simonetta, Anna Stainer, Giulia Suigo, Maria De Santis, Carlo Selmi, Chiara Masetti, Ana Lleo, Luigi Maria Terracciano, Stefano Aliberti, Francesco Amati","doi":"10.1093/mrcr/rxad005","DOIUrl":"https://doi.org/10.1093/mrcr/rxad005","url":null,"abstract":"<p><p>We here report the first case of anti-proteinase 3-positive anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis following the severe acute respiratory syndrome coronavirus 2 Pfizer-BioNTech vaccine presenting with prominent liver involvement and alveolar haemorrhage. Two weeks after vaccination, a 49-year-old man developed inflammatory arthralgias and hypertransaminasaemia. Two months later, fever and haemoptysis appeared; the patient tested positive for anti-proteinase 3 autoantibodies. High-dose steroids and rituximab were started, and complete remission was achieved. Systemic autoimmune diseases, including ANCA-associated vasculitis, should always be considered in the differential diagnosis of hypertransaminasaemia, especially when the clinical context is suspicious.</p>","PeriodicalId":18677,"journal":{"name":"Modern Rheumatology Case Reports","volume":"7 2","pages":"440-443"},"PeriodicalIF":0.0,"publicationDate":"2023-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9669766","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}
Hiroshi Kataoka, Tomoko Tomita, Mika Nakanowatari, Makoto Kondo, Masaya Mukai
{"title":"Gradual increase of avacopan dose with concomitant ursodeoxycholic acid use may help avoid the risk of C5a receptor inhibitor-induced liver injury in antineutrophil cytoplasmic antibody-associated vasculitis.","authors":"Hiroshi Kataoka, Tomoko Tomita, Mika Nakanowatari, Makoto Kondo, Masaya Mukai","doi":"10.1093/mrcr/rxad019","DOIUrl":"https://doi.org/10.1093/mrcr/rxad019","url":null,"abstract":"<p><p>Microscopic polyangiitis is a necrotising vasculitis characterised by anti-neutrophil cytoplasmic antibodies against myeloperoxidase. The complement component 5a receptor inhibitor avacopan effectively sustains remission in microscopic polyangiitis with a reduction in prednisolone dosage. Liver damage is a safety concern for this drug. However, when it occurs and how to treat it remain unknown. A 75-year-old man developed microscopic polyangiitis and presented with hearing impairment and proteinuria. Methylprednisolone pulse therapy followed by 30 mg/day prednisolone and two doses of weekly rituximab were administered. Avacopan was initiated to taper prednisolone for sustained remission. After 9 weeks, liver dysfunction and sparse skin eruptions developed. The cessation of avacopan and the initiation of ursodeoxycholic acid improved liver function without discontinuation of prednisolone and other concomitant drugs. After 3 weeks, avacopan was rechallenged with a small dose that was gradually increased; ursodeoxycholic acid was continued. Full-dose avacopan did not induce recurrence of liver injury. Therefore, gradually increasing the dose of avacopan with concomitant ursodeoxycholic acid use may help avoid possible avacopan-induced liver injury.</p>","PeriodicalId":18677,"journal":{"name":"Modern Rheumatology Case Reports","volume":"7 2","pages":"444-447"},"PeriodicalIF":0.0,"publicationDate":"2023-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9674629","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}
Takeshi Mochizuki, Koichiro Yano, Katsunori Ikari, Ken Okazaki
{"title":"Combination treatment with Janus kinase inhibitor and nintedanib for rheumatoid arthritis with progressive interstitial lung disease: A case report.","authors":"Takeshi Mochizuki, Koichiro Yano, Katsunori Ikari, Ken Okazaki","doi":"10.1093/mrcr/rxad021","DOIUrl":"https://doi.org/10.1093/mrcr/rxad021","url":null,"abstract":"<p><p>Interstitial lung disease (ILD) is a common extra-articular manifestation of rheumatoid arthritis (RA). Nintedanib has been approved for the treatment of idiopathic pulmonary fibrosis. Herein, we described the case of an 87-year-old woman treated with Janus kinase inhibitor (JAKi). Chest computed tomography revealed increased honeycombing; she was administered nintedanib while continuing RA treatment. The combination treatment (JAKi and nintedanib) controlled the RA disease activity without ILD deterioration. This case shows the potential of combination treatment with JAKi and nintedanib for the prevention of worsening of disease activity in RA and ILD.</p>","PeriodicalId":18677,"journal":{"name":"Modern Rheumatology Case Reports","volume":"7 2","pages":"350-353"},"PeriodicalIF":0.0,"publicationDate":"2023-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10292149","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}