{"title":"Eosinophilic Granulomatosis Polyangiitis (EGPA) Masquerading as a Mycotic Aneurysm of the Abdominal Aorta: Case Report and Review of Literature.","authors":"Pooja Kumari, Debendra Pattanaik, Claire Williamson","doi":"10.1155/2021/7093607","DOIUrl":"https://doi.org/10.1155/2021/7093607","url":null,"abstract":"<p><strong>Introduction: </strong>Aortic involvement leading to aortitis in eosinophilic granulomatosis polyangiitis (EGPA) is infrequent, and only 2 cases have been reported so far in the literature. Even more so, aortic aneurysm, secondary to EGPA, has never been reported and remains a diagnostic and therapeutic challenge. <i>Case Presentation</i>. We present a 63-year-old Caucasian male patient with a prior diagnosis of EGPA presenting with abdominal pain, nausea, and loose stools to the emergency department. Physical examination showed periumbilical tenderness. He had no peripheral eosinophilia but had high C-reactive protein and procalcitonin levels. CT abdomen revealed a mycotic aneurysm involving the infrarenal abdominal aorta. The patient declined surgical repair initially and was treated with IV antibiotics only. Unfortunately, 24 hours later, the aneurysm ruptured, leading to emergent axillofemoral bypass surgery. Surgical biopsy showed aortitis, periaortitis, and active necrotizing vasculitis.</p><p><strong>Conclusion: </strong>Abdominal aneurysms should be considered a complication of EGPA, and earlier immunosuppressive therapy should be considered to prevent further complications.</p>","PeriodicalId":9622,"journal":{"name":"Case Reports in Rheumatology","volume":"2021 ","pages":"7093607"},"PeriodicalIF":0.0,"publicationDate":"2021-09-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8452388/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39439693","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}
{"title":"A Fatal Case of Concurrent Disseminated Tuberculosis, Pneumocystis Pneumonia, and Bacterial Septic Shock in a Patient with Rheumatoid Arthritis Receiving Methotrexate, Glucocorticoid, and Tocilizumab: An Autopsy Report.","authors":"Shin-Ichiro Ohmura, Ryuhei Ishihara, Ayaka Mitsui, Yoshiro Otsuki, Toshiaki Miyamoto","doi":"10.1155/2021/7842049","DOIUrl":"https://doi.org/10.1155/2021/7842049","url":null,"abstract":"<p><p>Recently, treatment for rheumatoid arthritis has dramatically improved but increases the risk of bacterial and opportunistic infections. Herein, we report a fatal case of concurrent disseminated tuberculosis, pneumocystis pneumonia, and septic shock due to pyelonephritis caused by extended-spectrum <i>β</i>-lactamase-producing <i>Escherichia coli</i> in a patient with rheumatoid arthritis who received methotrexate, glucocorticoid, and tocilizumab. Despite undergoing intensive treatment, the patient developed respiratory failure and died after 7 days of admission. An autopsy indicated that pulmonary tuberculosis were the ultimate causes of death, while pyelonephritis was controlled.</p>","PeriodicalId":9622,"journal":{"name":"Case Reports in Rheumatology","volume":"2021 ","pages":"7842049"},"PeriodicalIF":0.0,"publicationDate":"2021-09-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8440108/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39424108","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}
S Biuden, K Maatallah, H Riahi, H Ferjani, M D Kaffel, W Hamdi
{"title":"SAPHO Syndrome Mimicking Infectious Spondylodiscitis and Bone Metastasis.","authors":"S Biuden, K Maatallah, H Riahi, H Ferjani, M D Kaffel, W Hamdi","doi":"10.1155/2021/5577257","DOIUrl":"https://doi.org/10.1155/2021/5577257","url":null,"abstract":"<p><p>The acronym SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) includes diseases with similar osteoarticular manifestations and skin conditions. Making this diagnosis is not always obvious, especially when the clinical presentation does not fit the typical pattern of the disease or it occurs in a particular field. We described three cases where the diagnosis was difficult. A 46 year-old woman presented with cervical pain. The cervical X-ray showed the aspect of an ivory C5 vertebra. The patient had, however, preserved general condition, no signs of underlying neoplasia, nor other joint complaints. Blood analysis was normal. Tomography did not find any suspect lesion but showed sclerosis and hyperostosis of the manubrium. Scintigraphy showed the characteristic \"bullhead\" appearance. A 61-year-old woman had thoracic and lumbar pain. MRI showed spondylodiscitis in D3-D4, D4-D5, D5-D6, D6-D7, and L1-L2 with paraspinal soft tissue involvement, simulating infectious spondylodiscitis. Infectious investigations and discovertebral biopsy performed twice were negative. SAPHO syndrome was then suspected. Bone scintigraphy showed uptake in the chondrosternal articulations and D4 to D7 vertebrae. The diagnosis of SAPHO was established. The third case was a 46-year-old man with a lung adenocarcinoma. Staging for metastatic disease, a TAP tomography was performed and showed osteosclerosis of D8 to D12 and intra-articular bridges in the sacroiliac joints. MRI and scintigraphy eliminated malignancy and confirmed the diagnosis of SAPHO. In our cases, imaging findings could facilitate differentiating SAPHO syndrome from other diseases.</p>","PeriodicalId":9622,"journal":{"name":"Case Reports in Rheumatology","volume":"2021 ","pages":"5577257"},"PeriodicalIF":0.0,"publicationDate":"2021-09-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8437625/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39420427","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}
{"title":"A Case of Granulomatosis with Polyangiitis with Various Breast Lesions as the Initial Symptoms: A Case-Based Review.","authors":"Masatoshi Kawataka, Toshiki Kido, Reina Tsuda, Takafumi Onose, Ryoko Asano, Miho Yamazaki, Naonori Sugishita, Hiroyuki Hounoki, Toshiko Kakiuchi, Koichiro Shinoda, Kazuyuki Tobe","doi":"10.1155/2021/4416072","DOIUrl":"https://doi.org/10.1155/2021/4416072","url":null,"abstract":"<p><p>A 44-year-old woman presenting with pus-like discharge from the nipples visited our hospital for scleritis. Subcutaneous induration and ulceration were found on her breast. She was diagnosed with granulomatosis with polyangiitis (GPA) considering scleritis, sinusitis, cutaneous granuloma formation, and antiproteinase 3-antineutrophil cytoplasmic antibodies and was successfully treated with glucocorticoids. Fifteen months later, she developed pulmonary consolidation and a right breast nodule. Biopsies of the breast nodule showed granulomatous vasculitis, and she was treated with rituximab. While breast involvement in GPA is rare, unilateral breast mass is a typical clinical feature; thus, GPA should be considered in such cases.</p>","PeriodicalId":9622,"journal":{"name":"Case Reports in Rheumatology","volume":"2021 ","pages":"4416072"},"PeriodicalIF":0.0,"publicationDate":"2021-08-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8448995/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39434603","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}
{"title":"A Case of Pulmonary Infarction Resembling Pneumonia during Immunosuppressive Treatment for Rheumatoid Arthritis.","authors":"Toshiki Kido, Koichiro Shinoda, Kazuyuki Tobe","doi":"10.1155/2021/5983580","DOIUrl":"https://doi.org/10.1155/2021/5983580","url":null,"abstract":"<p><p>A 67-year-old woman with rheumatoid arthritis (RA) presented with fever and dyspnea. Chest radiography and computed tomography (CT) revealed pulmonary infiltrates with ground-glass opacities. We considered bacterial or pneumocystis pneumonia because she was immunocompromised due to RA treatment. However, she had tachycardia and elevated D-dimer levels. We performed contrast-enhanced CT and subsequently diagnosed her with pulmonary embolism (PE). Though PE is not usually accompanied by parenchymal pulmonary shadows, pulmonary infarction may cause pulmonary infiltrates that can be mistaken for pneumonia. As RA is a thrombophilic disease, clinicians should be aware of PE and pneumonia as differential diagnoses in such patients.</p>","PeriodicalId":9622,"journal":{"name":"Case Reports in Rheumatology","volume":"2021 ","pages":"5983580"},"PeriodicalIF":0.0,"publicationDate":"2021-08-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8390160/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39365206","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}
Genessis Maldonado, Roberto Guerrero, Maria Intriago, Carlos Rios
{"title":"Autoinflammatory/Autoimmunity Syndrome Induced By Adjuvants (ASIA) Due to Silicone Incompatibility Syndrome.","authors":"Genessis Maldonado, Roberto Guerrero, Maria Intriago, Carlos Rios","doi":"10.1155/2021/5595739","DOIUrl":"https://doi.org/10.1155/2021/5595739","url":null,"abstract":"The adjuvant-induced autoimmune syndrome (ASIA) is associated with a dysregulation of the innate and adaptive immune system after exposure to chemical compounds, including liquid paraffin, silicone gel, acrylamides, and hyaluronic acid. Due the increase of the use of these compounds in cosmetic procedures, the prevalence of this syndrome is increasing. We present the first report in Ecuador associated to ASIA after an elective silicone breast prosthesis procedure, manifested as polyarthralgia, positive antinuclear antibody, anticentromere antibody, and a moderate positive Sclero-70.","PeriodicalId":9622,"journal":{"name":"Case Reports in Rheumatology","volume":"2021 ","pages":"5595739"},"PeriodicalIF":0.0,"publicationDate":"2021-08-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8382526/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39344261","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}
Moshiur Rahman Khasru, Md Abu Bakar Siddiq, Kazi Mohammad Sayeeduzzaman, Tangila Marzen, Abul Khair Mohammad Salek
{"title":"Coexistence of Rheumatoid Arthritis, Systemic Lupus Erythematosus, Sjogren Syndrome, Antiphospholipid Syndrome, and Ankylosing Spondylitis.","authors":"Moshiur Rahman Khasru, Md Abu Bakar Siddiq, Kazi Mohammad Sayeeduzzaman, Tangila Marzen, Abul Khair Mohammad Salek","doi":"10.1155/2021/8491717","DOIUrl":"https://doi.org/10.1155/2021/8491717","url":null,"abstract":"<p><p>A 37-year-old Bangladeshi woman presented with low back and several joints pain and swelling for months together; there was significant morning stiffness for more than two hours. Repeated abortions, dry eye, hair fall, photosensitivity, and oral ulcer were the additional complaints. Clinical examination unveiled asymmetrical peripheral and both sacroiliac joint tenderness, positive modified Schober's test, and limited chest expansion. Schirmer's test was positive. The history of rheumatoid arthritis (RA) and ankylosing spondylitis (AS) among 1st-degree relatives was also significant. Biochemical analysis revealed pancytopenia, raised erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP), and mild microscopic proteinuria. The patient was seropositive for rheumatoid factor (RF), antibodies against cyclic citrullinated peptides (anti-CCP), antinuclear antibody (ANA), anti-Sm antibody, anti-Sjögren's-syndrome-related antigen A and B (anti-SSA/SSB), antiphospholipid (aPL-IgG/IgM), and HLA B27; however, serum complement (C3 and C4) levels were normal. Basal cortisol level measured elevated. Besides, X-ray and MRI of lumbosacral spines demonstrated sacroiliitis. There was radiological cardiomegaly, echocardiography unveiled atrial regurgitation, and ascending aorta aneurysm. Based on the abovementioned information, RA, AS, and systemic lupus erythematosus (SLE) have been diagnosed. Moreover, the patient developed Sjogren's syndrome (SS), antiphospholipid lipid syndrome (APS), Cushing syndrome, ascending aorta aneurysm, and atrial regurgitation. Her disease activity score for RA (DAS28), DAS for AS (ASDAS), SLE disease activity index (SLEDAI), and Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR) scores were 3.46, 2.36, 23, and 5, respectively. The patient received hydroxychloroquine (200 mg daily), pulsed cyclophosphamide, prednisolone (20 mg in the morning), and naproxen 500 mg (twice daily). To our best knowledge, this is the first report documenting RA, AS, and SLE with secondary SS and APS.</p>","PeriodicalId":9622,"journal":{"name":"Case Reports in Rheumatology","volume":"2021 ","pages":"8491717"},"PeriodicalIF":0.0,"publicationDate":"2021-08-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8376431/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39337178","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}
{"title":"An Interesting Case of Bilateral Facial Palsy due to Granulomatosis with Polyangiitis.","authors":"Rajish Sanjit Kumar Shil, Jamal Ali Teir","doi":"10.1155/2021/9963564","DOIUrl":"10.1155/2021/9963564","url":null,"abstract":"<p><p>Granulomatosis with polyangiitis (formerly called Wegener's granulomatosis) is a systemic autoimmune disease, which can lead to necrotizing vasculitis affecting small vessels and cause inflammation of blood vessels in the nose, sinuses, throat, lungs, and kidneys. In rare instances, it has shown involvement of the brain and cranial nerves as well. We are reporting a case of granulomatosis with polyangiitis, complicated by bilateral facial palsy due to lower motor neuron involvement of the facial nerve, which has responded well to immunosuppressive treatment, particularly rituximab. It is prudent to be vigilant in investigating patients with atypical presentation for systemic autoimmune diseases, as this approach would affect the patient morbidity and mortality with early initiation of treatment for the disease.</p>","PeriodicalId":9622,"journal":{"name":"Case Reports in Rheumatology","volume":"2021 ","pages":"9963564"},"PeriodicalIF":0.0,"publicationDate":"2021-08-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8360741/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39313467","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}
Daniela Oliveira, Vanessa Chaves, José Carlos Martins, Carlos Vaz, Miguel Bernardes, Jorge Almeida
{"title":"Pancreatic Cancer and Primary Sjögren's Syndrome: A Case Report.","authors":"Daniela Oliveira, Vanessa Chaves, José Carlos Martins, Carlos Vaz, Miguel Bernardes, Jorge Almeida","doi":"10.1155/2021/9915881","DOIUrl":"https://doi.org/10.1155/2021/9915881","url":null,"abstract":"<p><p>Primary Sjögren syndrome (SS) is a chronic inflammatory systemic autoimmune disease with a high risk of malignancy development, namely, lymphoproliferative neoplasms. Few studies also reported a high risk of solid cancers; however, the coexistence of primary SS and pancreatic cancer has been rarely described. In this paper, we aim to describe a case of a 59-year-old woman who was an active smoker with sicca symptoms and symmetrical polyarthritis and was diagnosed with primary SS two years before the development of metastatic pancreatic adenocarcinoma. Despite institution of chemotherapy, the patient succumbed to the malignancy. Besides that, we explore the link between primary SS and solid cancers including the main predictors of malignancy and the role of primary SS as a paraneoplastic syndrome. Patients with primary SS should be closely monitored for malignancy, not only for hematological cancer, but also for solid tumors. Further research is necessary to understand which are the predictors of cancer proliferation in primary SS patients.</p>","PeriodicalId":9622,"journal":{"name":"Case Reports in Rheumatology","volume":"2021 ","pages":"9915881"},"PeriodicalIF":0.0,"publicationDate":"2021-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8356001/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39313466","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}
{"title":"Utilization of Rituximab for Refractory Rowell Syndrome.","authors":"Sukhraj Singh, Sandra Sheffield, Nisha Chowdhury, Swetha Nuthulaganti, Zareen Vaghaiwalla, Karishma Ramsubeik","doi":"10.1155/2021/2727382","DOIUrl":"https://doi.org/10.1155/2021/2727382","url":null,"abstract":"<p><p>Rowell syndrome describes the occurrence of erythema multiforme-like lesions in patients with cutaneous lesions of lupus erythematosus. The clinical picture of atypical erythema multiforme-like lesions, presence of chilblains, speckled ANA pattern, anti-Ro/SSA, or anti-La/SSB antibodies, and absence of infectious or pharmacologic triggers in a patient with systemic lupus erythematosus are some of the classic clinical and serologic features. Histopathologic and serologic findings can help differentiate this process from erythema multiforme. We present a case of young woman with systemic lupus erythematosus, end-stage renal disease due to lupus nephritis, and a remote history of Steven-Johnson syndrome due to sulfa allergy who presented to the hospital with a recurrent, progressive, targetoid erythematous rash involving more than 60% of her body surface area. Our patient had several hospitalizations in the recent past for this erythematous rash and had failed oral therapy with prednisone 1 mg/kg and hydroxychloroquine. In view of the minimal improvement and increasing severity and patient exhibiting early features of mast cell activation syndrome, the patient was treated with pulse intravenous glucocorticoids followed by rituximab with an excellent response. We highlight a unique case report of progressive Rowell syndrome refractory to standard of care with an excellent response to rituximab.</p>","PeriodicalId":9622,"journal":{"name":"Case Reports in Rheumatology","volume":"2021 ","pages":"2727382"},"PeriodicalIF":0.0,"publicationDate":"2021-07-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8342167/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39291484","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}