Case Reports in Rheumatology最新文献

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Severe Antiphospholipid Syndrome and Diffuse Glomerulonephritis After Adalimumab Treatment in a Patient With Ulcerative Colitis. 一名溃疡性结肠炎患者在阿达木单抗治疗后出现严重抗磷脂综合征和弥漫性肾小球肾炎
Case Reports in Rheumatology Pub Date : 2024-11-04 eCollection Date: 2024-01-01 DOI: 10.1155/2024/8024757
Ileana Rivera-Burgos, Luis M Vilá
{"title":"Severe Antiphospholipid Syndrome and Diffuse Glomerulonephritis After Adalimumab Treatment in a Patient With Ulcerative Colitis.","authors":"Ileana Rivera-Burgos, Luis M Vilá","doi":"10.1155/2024/8024757","DOIUrl":"https://doi.org/10.1155/2024/8024757","url":null,"abstract":"<p><p>Tumor necrosis factor alpha inhibitors (TNFi) are biological drugs used worldwide to treat various autoimmune disorders. Paradoxically, TNF-<i>α</i> antagonists can also induce autoimmune diseases being systemic vasculitis, systemic lupus erythematosus, and psoriasis, the most common. We present a 22-year-old woman with ulcerative colitis (UC) who was started on adalimumab 40 mg subcutaneously every 2 weeks. After two doses of adalimumab, she developed gangrene of all toes and acute kidney injury requiring hemodialysis. Skin biopsy showed thrombi in the small vessels of the dermis. Renal biopsy disclosed diffuse proliferative glomerulonephritis (GN) and acute tubulointerstitial nephritis. Serologic work-up showed positive IgG anticardiolipin (ACL) antibodies and low C3 levels. Antinuclear, anti-dsDNA, anti-Smith, anti-SSA, anti-SSB, anti-RNP, antineutrophil cytoplasmic antibodies, ACL (IgA and IgM), and anti-<i>β</i>2-glycoprotein I (IgG, IgM, and IgA) antibodies were not elevated. Lupus anticoagulant test and cryoglobulins were negative. Adalimumab was discontinued, and she was treated with enoxaparin, intravenous (IV) methylprednisolone pulse, IV cyclophosphamide, and plasmapheresis followed by maintenance therapy with warfarin, prednisone, azathioprine, and hydroxychloroquine. She did not have further thrombotic events, and the acute kidney injury completely resolved. ACL IgG antibodies decreased to normal levels, and repeated tests were negative. After 7 years, anticoagulation and immunosuppressive drugs were discontinued. During a follow-up of 24 months, she remained in complete clinical remission. This report highlights the occurrence of autoimmune disorders induced by TNFi. Thus, careful monitoring of adverse immune reactions to TNFi is highly recommended.</p>","PeriodicalId":9622,"journal":{"name":"Case Reports in Rheumatology","volume":"2024 ","pages":"8024757"},"PeriodicalIF":0.0,"publicationDate":"2024-11-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11554406/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142615527","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Granulomatosis With Polyangiitis Mimicking Temporal Arteritis. 模仿颞动脉炎的多发性肉芽肿病
Case Reports in Rheumatology Pub Date : 2024-10-17 eCollection Date: 2024-01-01 DOI: 10.1155/2024/9699571
Ali Dehghan, Mahya Sadat Emami Meybodi, Shokoofeh Fooladmotlagh, Mohsen Zaremehrjardi, Hamidreza Soltani
{"title":"Granulomatosis With Polyangiitis Mimicking Temporal Arteritis.","authors":"Ali Dehghan, Mahya Sadat Emami Meybodi, Shokoofeh Fooladmotlagh, Mohsen Zaremehrjardi, Hamidreza Soltani","doi":"10.1155/2024/9699571","DOIUrl":"https://doi.org/10.1155/2024/9699571","url":null,"abstract":"<p><p>This case represents the first diagnosis of pachymeningitis due to granulomatosis with polyangiitis (GPA) in an elderly Iranian man who initially presented with persistent daily headaches. PCR tests of cerebrospinal fluid for tuberculosis, brucellosis, and fungal infections all yielded negative results. Given the pachymeningitis pattern observed on brain MRI and the absence of infectious and lymphoma diseases, along with positive anti-PR3 and proteinuria (793 mg in a 24-h urine sample), a diagnosis of GPA was established. The patient was treated with five doses of pulse methylprednisolone and one dose of pulse cyclophosphamide (1 g). Additionally, prednisolone 60 mg daily, monthly pulse cyclophosphamide, a daily calcium-D tablet, and alendronate 70 mg weekly were prescribed. Subsequently, the patient's headaches, hearing loss, and vision loss were completely resolved. GPA should be considered in older individuals with persistent daily headaches, especially when pachymeningitis is evident. The use of contrast-enhanced brain MRI is an essential diagnostic tool in such cases.</p>","PeriodicalId":9622,"journal":{"name":"Case Reports in Rheumatology","volume":"2024 ","pages":"9699571"},"PeriodicalIF":0.0,"publicationDate":"2024-10-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11502123/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142495750","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Bing-Neel Syndrome: An Unknown GCA Mimicker. 宾-内尔综合征:一种未知的 GCA 拟态
Case Reports in Rheumatology Pub Date : 2024-08-12 eCollection Date: 2024-01-01 DOI: 10.1155/2024/2043012
Arifa Javed, Sadia Arooj Javed, Barbara Ostrov, Jiang Qian, Khoa Ngo
{"title":"Bing-Neel Syndrome: An Unknown GCA Mimicker.","authors":"Arifa Javed, Sadia Arooj Javed, Barbara Ostrov, Jiang Qian, Khoa Ngo","doi":"10.1155/2024/2043012","DOIUrl":"10.1155/2024/2043012","url":null,"abstract":"<p><p>Giant cell arteritis (GCA) is a chronic granulomatous vasculitis of medium and large arteries leading to cranial and extracranial manifestations. Temporal artery biopsy is considered the gold standard; however, its sensitivity is low at 47%. We report a unique case of Bing-Neel Syndrome (BNS) presenting as biopsy-proven GCA. BNS is a rare complication (1%) of Waldenstrom Macroglobulinemia (WM), which results from infiltration of lymph plasmacytoid cells and plasma cells into the central nervous system. A 77-year-old female with a past medical history of glaucoma, hypertension, diabetes, and chronic ocular ischemic syndrome in her right eye presented with progressive left eye vision loss for 5 days. Fundoscopic examination was notable for pseudophakic pseudopallor but no optic disc edema. Intraocular pressure was >40 and normalized after acetazolamide. The patient was started on pulse dose steroids by her neuro-ophthalmologist. She was discharged home on 60 mg of prednisone. At follow up with her neuro-ophthalmologist, new dot blot hemorrhages in the left eye were noted and she was readmitted for pulse dose of intravenous methylprednisolone. Temporal artery biopsy was consistent with GCA spectrum. Work up revealed paraproteinemia and subsequent bone marrow biopsy demonstrated WM. The patient was treated for her WM and her ophthalmic complications stabilized.</p>","PeriodicalId":9622,"journal":{"name":"Case Reports in Rheumatology","volume":"2024 ","pages":"2043012"},"PeriodicalIF":0.0,"publicationDate":"2024-08-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11333131/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142006270","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Mystical Myositis: A Case Series from Kalafong Provincial Tertiary Hospital, Pretoria, South Africa. 神秘肌炎:来自南非比勒陀利亚卡拉丰省三级医院的系列病例。
Case Reports in Rheumatology Pub Date : 2024-08-05 eCollection Date: 2024-01-01 DOI: 10.1155/2024/7410630
Michael Myburgh
{"title":"Mystical Myositis: A Case Series from Kalafong Provincial Tertiary Hospital, Pretoria, South Africa.","authors":"Michael Myburgh","doi":"10.1155/2024/7410630","DOIUrl":"10.1155/2024/7410630","url":null,"abstract":"<p><p>Idiopathic inflammatory myositis (IIM) is an expanding field in rheumatology as more myositis-specific antibodies (MSAs) and myositis-associated antibodies (MAAs) become available for testing. Clinical signs and specific clinical phenotypes are found in the MSA group, with as high as 70% of IIM patients having a positive myositis-specific antibody. Although IIM remains a heterogenous disease, assigning a phenotype to these patients will prove to be critical as we learn which cases require more aggressive therapy and what complications to search for as the disease progresses. The IIM patients for the last 5 years were reviewed and profiled using recently available myositis profile testing at our National Health Laboratory Services. Patients from our rheumatology clinic were categorized according to this antibody profile. Three cases diagnosed with dermatomyositis (DM) were selected for discussion in this article which include a patient with each of the following: anti-transcriptional intermediary factor 1-y (TIF1y) DM, anti-melanoma differentiation-associated protein 5 (MDA 5) DM, and anti-signal recognition particle (SRP) DM.</p>","PeriodicalId":9622,"journal":{"name":"Case Reports in Rheumatology","volume":"2024 ","pages":"7410630"},"PeriodicalIF":0.0,"publicationDate":"2024-08-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11319052/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141970689","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Arthritis or an Adjacent Fascial Response? A Case Report of Combined Pyomyositis and Aseptic Arthritis. 关节炎还是邻近的筋膜反应?合并化脓性肌炎和无菌性关节炎的病例报告。
Case Reports in Rheumatology Pub Date : 2024-06-25 eCollection Date: 2024-01-01 DOI: 10.1155/2024/2608144
Noa Martonovich, Sharon Reisfeld, Yaniv Yonai, Eyal Behrbalk
{"title":"Arthritis or an Adjacent Fascial Response? A Case Report of Combined Pyomyositis and Aseptic Arthritis.","authors":"Noa Martonovich, Sharon Reisfeld, Yaniv Yonai, Eyal Behrbalk","doi":"10.1155/2024/2608144","DOIUrl":"10.1155/2024/2608144","url":null,"abstract":"<p><p>Pyomyositis, accompanied by aseptic arthritis, has been previously documented in several publications. However, none of the authors in the mentioned case reports offered a pathophysiological explanation for this unusual phenomenon or proposed a treatment protocol. We present a case of a healthy, 70-year-old male who was presented to the emergency department 4 days after tripping over a pile of wooden planks and getting stabbed by a nail to his thigh. The right thigh was swollen. Unproportional pain was produced by a light touch to the thigh. A laboratory test and a CT scan were obtained. The working diagnosis was pyomyositis of the thigh and septic arthritis of the ipsilateral knee. The patient underwent urgent debridement and irrigation of his right thigh. An arthroscopic knee lavage was performed as well. Intraoperative cultures from the thigh revealed the growth of <i>Streptococcus pyogenes</i> and <i>Staphylococcus aureus</i>. Cultures from synovial fluid were sterile; thus, septic arthritis was very unlikely. The source of the knee effusion might have been an aseptic inflammatory response due to the proximity of the thigh infection. Anatomically, the quadriceps muscle inserts on the patella, and its tendon fuses with the knee capsule, creating a direct fascial track from the thigh to the knee. The inflammatory response surrounding the infection may have followed this track, creating a domino effect, affecting adjacent capillaries within the joint capsule, and causing plasma leakage into the synovial space, leading to joint effusion. Our suggested treatment is addressing the primary infection with antibiotics and considering adding anti-inflammatory therapy, given our suspicion that this process has an inflammatory component.</p>","PeriodicalId":9622,"journal":{"name":"Case Reports in Rheumatology","volume":"2024 ","pages":"2608144"},"PeriodicalIF":0.0,"publicationDate":"2024-06-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11219200/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141491012","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
A Severe Case of Overlap of Morphea and Eosinophilic Fasciitis after Burn Injuries 烧伤后莫菲斯病与嗜酸性筋膜炎重叠的严重病例
Case Reports in Rheumatology Pub Date : 2024-05-14 DOI: 10.1155/2024/3123953
H. Sami, Faria Sami, Shahzad Ahmed Sami, A. Nashwan
{"title":"A Severe Case of Overlap of Morphea and Eosinophilic Fasciitis after Burn Injuries","authors":"H. Sami, Faria Sami, Shahzad Ahmed Sami, A. Nashwan","doi":"10.1155/2024/3123953","DOIUrl":"https://doi.org/10.1155/2024/3123953","url":null,"abstract":"Background. Generalized morphea is a rare fibrosing skin illness that progresses from erythematous, violet-colored skin patches to sclerotic plaques. Another uncommon immune-mediated connective tissue disease called eosinophilic fasciitis (EF) evolves to cause sclerosis and woody skin induration. The coexistence of the two is extremely rare and has a poorer prognosis. Our case report is one of the first to report burn injuries as a trigger factor for EF and generalized morphea overlap. Case Presentation. A 36-year-old man presented with acute onset of rapidly progressing skin thickening, tender edema, and skin contractures involving all extremities, shortly after enduring burn injuries from a gasoline explosion. Workup was remarkable for peripheral eosinophilia, hypergammaglobulinemia, and elevated C-reactive protein. Skin biopsy demonstrated sclerodermoid changes and sclerotic thickening of subcutaneous fibrous septa associated with stromal mucin, dermal perivascular, diffuse lymphoplasmacytic infiltrate with eosinophils, decreased CD34 expression, and increased factor XIIIa. He was subsequently diagnosed with an overlap of generalized morphea and eosinophilic fasciitis. The patient had only limited improvement with steroids, methotrexate, mycophenolate mofetil, and intralesional triamcinolone acetonide injections. Conclusion. Generalized morphea with concomitant EF indicates some degree of therapeutic resistance and poor prognosis with a low quality of life. Burn injuries can be a trigger factor for this overlap syndrome. Prompt identification of at-risk individuals and initiating aggressive management are necessary.","PeriodicalId":9622,"journal":{"name":"Case Reports in Rheumatology","volume":"39 3","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-05-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140981358","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}
引用次数: 0
A Rare Case of Acute Methotrexate Toxicity Leading to Bone Marrow Suppression. 急性甲氨蝶呤中毒导致骨髓抑制的罕见病例
Case Reports in Rheumatology Pub Date : 2024-03-15 eCollection Date: 2024-01-01 DOI: 10.1155/2024/7693602
Samreen Khuwaja, Matthew Lyons, Beenish Zulfiqar
{"title":"A Rare Case of Acute Methotrexate Toxicity Leading to Bone Marrow Suppression.","authors":"Samreen Khuwaja, Matthew Lyons, Beenish Zulfiqar","doi":"10.1155/2024/7693602","DOIUrl":"10.1155/2024/7693602","url":null,"abstract":"<p><p>Methotrexate is a first-line disease modifying antirheumatic drug used for the treatment of inflammatory arthritis. Bone marrow suppression is a common adverse reaction of methotrexate following its long-term use. However, low dose methotrexate is rarely associated with life-threatening bone marrow suppression. This case represents an atypical presentation of acute bone marrow suppression shortly after initiating treatment with low-dose methotrexate. A 76-year-old male patient presented with oral ulcers, poor oral intake, and acute kidney injury within 3 weeks of initiating 15 mg weekly of methotrexate for seronegative rheumatoid arthritis. Complete blood count was suggestive of pancytopenia with hemoglobin of 10.8 g/dL, total white cell count 3.36 (1000/uL) (absolute neutrophil count 490 micro/L), platelets 19,000, serum albumin 3.1 g/dL, ESR elevated at 83 mm/hr, CRP elevated at 86.6 mg/L, and ferritin mildly elevated at 625 ng/mL. Peripheral blood smear showed signs of bone marrow suppression but no signs of hemolysis or inflammation. Serum methotrexate levels were minimally detectable at 0.05 umol/L. Methotrexate was held, within 48 hours of admission; his WBC dropped to 1.48, Hgb 9.9, and platelets 15,000. ANC reached a nadir of 220. He was treated with broad spectrum antibiotics, high-dose folic acid, fluconazole for oral thrush, and intravenous bicarbonate and leucovorin supplementation, dosed at PO 20 mg daily. On day 7, his blood count showed improvement along with improvement in his symptoms. The patient was discharged home on day 8<sup>th</sup> of hospitalization and upon one month follow-up in rheumatology clinic, his complete blood count had normalized. This case highlights multiple risk factors that triggered pancytopenia in our elderly patient, resulting in acute methotrexate toxicity.</p>","PeriodicalId":9622,"journal":{"name":"Case Reports in Rheumatology","volume":"2024 ","pages":"7693602"},"PeriodicalIF":0.0,"publicationDate":"2024-03-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10959578/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140206301","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Rare Case Studies of Bilateral and Symmetric Sacroiliac Disease. 双侧和对称性骶髂关节疾病的罕见病例研究。
Case Reports in Rheumatology Pub Date : 2024-03-07 eCollection Date: 2024-01-01 DOI: 10.1155/2024/8893089
Stephen Soloway, Alyxandra M Soloway, Tyler G Chin, Timothy Lieske
{"title":"Rare Case Studies of Bilateral and Symmetric Sacroiliac Disease.","authors":"Stephen Soloway, Alyxandra M Soloway, Tyler G Chin, Timothy Lieske","doi":"10.1155/2024/8893089","DOIUrl":"10.1155/2024/8893089","url":null,"abstract":"<p><strong>Introduction: </strong>Inflammatory sacroiliitis is common in rheumatology practice. Spondyloarthritis is often underdiagnosed due to the lack of proper evaluation of the sacroiliac joints (SIJs), clinically and radiographically. If SIJ is inflamed or arthritic, the arthritic said patient typically has spondyloarthritis, in the absence of infections or crystal arthritis. Sacroiliitis, in particular, when diagnosed between 12 and 45 years of age, is indicative of spondyloarthritis. People are often misdiagnosed and mislabeled as fibromyalgia because their serologies are negative. Our goal is to point out the importance of proper evaluation, diagnosis, and importance of inflammatory SIJ disease and conditions that involve SIJ inflammation.</p><p><strong>Cases: </strong>We present three rare conditions presenting with bilateral and symmetric SIJ disease, none of which is ankylosing spondylitis, Crohn's colitis, ulcerative colitis, psoriatic arthritis, and reactive arthritis (Reiter syndrome); there are reports of concurrent SIJ disease in rheumatoid arthritis and SLE.</p><p><strong>Conclusion: </strong>The authors believe that SIJ disease is overlooked, is underdiagnosed, and can lead to incorrect treatment. We suggest a greater focus on SIJ imaging in the diagnosis and treatment of unexplained illnesses associated with low back pain, morning stiffness, or unexplained buttock pain. Providers should review their own SIJ films. The meaning of SIJ widening, cortical irregularity, spurs, and the significance of the anterior inferior SI joints, bone marrow edema, and fusion (namely, the natural history of sacroiliac pathophysiology).</p>","PeriodicalId":9622,"journal":{"name":"Case Reports in Rheumatology","volume":"2024 ","pages":"8893089"},"PeriodicalIF":0.0,"publicationDate":"2024-03-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10940025/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140130800","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
An Unusual Case of Peripheral Nerve Vasculitis. 一个罕见的周围神经血管炎病例
Case Reports in Rheumatology Pub Date : 2024-01-29 eCollection Date: 2024-01-01 DOI: 10.1155/2024/3469182
S Wang, Arsany A, D Feinstein, P Traisak, H Eid, M Karpoff
{"title":"An Unusual Case of Peripheral Nerve Vasculitis.","authors":"S Wang, Arsany A, D Feinstein, P Traisak, H Eid, M Karpoff","doi":"10.1155/2024/3469182","DOIUrl":"10.1155/2024/3469182","url":null,"abstract":"<p><p>Peripheral neuropathy is a common manifestation of systemic vasculitis. The etiology of vasculitic peripheral neuropathy is generally classified into two groups: systemic and nonsystemic. In systemic vasculitic neuropathy (SVN), neuropathy is a consequence of a systemic disease, most commonly involving medium and small vessels throughout the body. There are three main clinical presentations: multifocal neuropathy, distal symmetric polyneuropathy, and overlapping multifocal neuropathy. Specifically, distal symmetric polyneuropathy affects multiple somatic nerves diffusely in a symmetric and length-dependent pattern (also known as the classic stocking-glove pattern). This case represents an atypical presentation of SVN, presenting with widespread symmetric polyneuropathy.A 73-year-old woman presented with distal acute on chronic bilateral upper and lower extremity weakness, sensory changes, and widespread pain. Symptoms started about three months prior and gradually worsened with progressive difficulty with ambulation and required assistive devices. Elevated ESR is at 70 mm/hour, CRP at 25.66 mg/dL, elevated c-ANCA titers at 1 : 320 and PR3 at 5.0 AI, and elevated creatine kinase (CK) at 500-600 U/L. A muscle biopsy of the left vastus showed neurogenic atrophy without myositis. Initial improvement was with oral prednisone, but was stopped on discharge. Many purpuric and petechial lesions were developed on distal legs/feet and right fourth digit distal gangrene. EMG showed distal, symmetric, and axonal polyneuropathy affecting the upper and lower extremities and acute denervation in more distal muscles. The patient received pulse dose steroids and two doses of rituximab induction therapy and was discharged with an oral steroid taper. The patient's symptoms started as distal symmetric neuropathy at the onset and progressively worsened over the course of 3 months. Neuropathy, both on the exam and on EMG, seemed to have developed more rapidly than expected, regardless of its distribution. The EMG showed severe peripheral nerve damage and denervation, which is unusual for ANCA-associated systemic vasculitis.</p>","PeriodicalId":9622,"journal":{"name":"Case Reports in Rheumatology","volume":"2024 ","pages":"3469182"},"PeriodicalIF":0.0,"publicationDate":"2024-01-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10843867/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139691315","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
A New Case of Granulomatosis with Polyangiitis Presented with Tolosa-Hunt Syndrome Manifestations. 多发性肉芽肿病伴有托洛萨-亨特综合征表现的新病例。
Case Reports in Rheumatology Pub Date : 2024-01-22 eCollection Date: 2024-01-01 DOI: 10.1155/2024/5552402
Maryam Mohebbi, Shahriar Nafissi, Majid Alikhani
{"title":"A New Case of Granulomatosis with Polyangiitis Presented with Tolosa-Hunt Syndrome Manifestations.","authors":"Maryam Mohebbi, Shahriar Nafissi, Majid Alikhani","doi":"10.1155/2024/5552402","DOIUrl":"10.1155/2024/5552402","url":null,"abstract":"<p><strong>Background: </strong>Tolosa-Hunt syndrome (THS) is a rare disorder involving the orbital and retro-orbital space. The typical symptoms include sensory loss in the trigeminal nerve's distribution, orbital pain, swelling, headaches, and cranial nerve palsies. <i>Case Presentation</i>. We report a 40-year-old female who initially presented with biparietal headache, unresponsive to medication, which then led to ophthalmoplegia and orbital pain. Serological findings demonstrated positive CANCA-PR3. She was initially treated with 1 g pulse methylprednisolone for three days. Based on the rheumatological evaluation and her positive lung nodule, hematuria, dysmorphic red blood cells, and positive antiproteinase 3 classic antineutrophil cytoplasm antibodies (CANCA-PR3) and also based on the diagnostic criteria for granulomatosis with polyangiitis criteria for Wegner disease, her treatment was continued with prednisolone 1 mg/kg and also rituximab at the first and 14<sup>th</sup> day of treatment.</p><p><strong>Conclusion: </strong>In our case of THS, we achieved satisfactory improvement in symptoms through the administration of high-dose steroids.</p>","PeriodicalId":9622,"journal":{"name":"Case Reports in Rheumatology","volume":"2024 ","pages":"5552402"},"PeriodicalIF":0.0,"publicationDate":"2024-01-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10824575/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139575262","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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