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Case Reports in Rheumatology

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https://www.readbyqxmd.com/read/29971178/aortic-aneurysm-as-a-complication-of-granulomatosis-with-polyangiitis-successfully-treated-with-prednisolone-and-cyclophosphamide-a-case-report-and-review-of-the-literature
#1
Naoko Niimi, Tomoko Miyashita, Kana Tanji, Takuya Hirai, Kozo Watanabe, Keigo Ikeda, Shinji Morimoto, Iwao Sekigawa
A 57-year-old Japanese man was admitted to the hospital with back pain and fever, multiple lung nodules, and abdominal aortic aneurysm (AAA). Laboratory tests performed at admission showed an increased proteinase 3 anti-neutrophil cytoplasmic antibody (PR3-ANCA) level. Video-associated thoracoscopic lung biopsy was performed; pathologic examination showed granulation tissue with necrosis and multinucleated giant cells. The diagnosis of granulomatosis with polyangiitis (GPA) was confirmed on the basis of the clinical presentation, laboratory findings, and lung biopsy...
2018: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/29888022/cerebral-venous-thrombosis-mimicking-a-discrete-brain-mass-a-case-report-and-literature-review
#2
Dana DiRenzo, Zsuzsanna H McMahan, Naman S Desai, Rebecca Manno, Michelle Petri
The differential diagnosis for a focal brain lesion in a patient with systemic lupus erythematosus (SLE) is broad and includes infection, malignancy, and vascular and inflammatory etiologies. One rarely considered vascular pathology is cerebral venous thrombosis (CVT), which is often associated with a delay in diagnosis because of variable presentation and rare incidence. We present the case of a young woman with a new discrete brain lesion that appeared in the context of highly active SLE and was ultimately diagnosed with a CVT...
2018: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/29854539/an-underlooked-cause-of-periodic-fever-pfapa-in-an-adult-patient-with-no-response-to-tonsillectomy
#3
Fiaz Alam, Mohammed Hammoudeh
Periodic fever with aphthous stomatitis, pharyngitis, and adenitis (PFAPA) is an autoinflammatory disease manifested as recurrent febrile episodes associated with one of the following cardinal features: aphthous ulceration, pharyngitis, and cervical adenitis. It was initially described in children and thought to be a disease of pediatric age group. Few adult cases were also reported in the literature. We describe the case of a 39-year-old female affected by PFAPA who presented with a history of febrile episodes associated with aphthous ulceration, stomatitis, and tonsillitis for 4 years...
2018: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/29854538/intravascular-large-b-cell-lymphoma-mimicking-temporal-arteritis
#4
Ifeyinwa Emmanuela Obiorah, Metin Ozdemirli
Intravascular lymphoma is a rare type of lymphoma, characterized by growth of lymphoma cells within the microvasculature. The majority of the cases are of B-cell lineage, although rare examples of T or NK lineage have also been reported. The lymphoma is usually widely disseminated in the vascular spaces of any organ at the time of diagnosis including the skin and bone marrow. Lymph nodes are typically spared. The clinical picture depends on the specific organ involvement making the correct diagnosis very difficult...
2018: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/29854537/poncet-s-disease-in-the-preclinical-phase-of-rheumatoid-arthritis
#5
Myat Tun Lin Nyo, Mahmood M T M Ally, Elsa Magreta Van Duuren, Regan Arendse
We report on a patient with seropositive polyarthritis retrospectively diagnosed as Poncet's disease in the preclinical phase of seropositive rheumatoid arthritis. Our patient developed rheumatoid arthritis more than 2 years after being successfully treated for pulmonary tuberculosis and an initial inflammatory polyarthritis consistent with the diagnosis of Poncet's disease. This case illustrates the importance of recognizing Poncet's disease in a patient presenting with polyarthritis in order to avoid inappropriate long-term disease modifying antirheumatic treatment...
2018: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/29850358/increasing-the-interval-of-canakinumab-administration-effectively-supports-the-remission-of-schnitzler-s-syndrome
#6
Vadim R Gorodetskiy, Svetlana O Salugina, Evgeny S Fedorov
Schnitzler's syndrome (SchS) is a rare, disabling, autoinflammatory disorder characterized by recurrent urticarial rash and monoclonal IgM gammopathy. Interleukin-1 beta (IL-1 β ) plays an important role in the pathophysiology of SchS. Only anecdotal reports demonstrate the efficiency and safety of human monoclonal anti-human IL-1 β antibody (canakinumab) use in SchS therapy. However, there are no generally accepted recommendations concerning the scheme (or frequency) of canakinumab use for this disease. Here, we report the effective long-term treatment of SchS in a 44-year-old male with a standard canakinumab dose (150 mg) but with an increased 4-month injection interval...
2018: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/29850357/erosive-arthritis-fibromatosis-and-keloids-a-rare-dermatoarthropathy
#7
Fawad Aslam, Jonathan A Flug, Yousif Yonan, Shelley S Noland
Polyfibromatosis is a rare disease characterized by fibrosis manifesting in different locations. It is commonly characterized by palmar fibromatosis (Dupuytren's contracture) in variable combinations with plantar fibromatosis (Ledderhose's disease), penile fibromatosis (Peyronie's disease), knuckle pads, and keloids. There are only three reported cases of polyfibromatosis and keloids with erosive arthritis. We report one such case and review the existing literature on this rare syndrome.
2018: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/29808155/a-case-of-familial-mediterranean-fever-with-extensive-lymphadenopathy-and-complex-heterozygous-genotype-presenting-in-the-fourth-decade
#8
Jawad Al-Khafaji, Fran Ganz-Lord, Venkata Rajesh Konjeti, Aaron D Viny
Familial Mediterranean fever (FMF) is an inherited disease caused by loss of function mutations in the MEFV gene encoding pyrin, a negative regulator of interleukin-1. The disease is characterized by recurrent fever and self-limited attacks of joint, chest, and abdominal pain but lymphadenopathy is an infrequent manifestation. While mesenteric lymphadenopathy has been described in several cases in the literature; hilar, paratracheal, axillary, pelvic, and retroperitoneal lymphadenopathy are extremely rare and have been reported separately in very few individuals...
2018: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/29808154/sj%C3%A3-gren-syndrome-complicated-with-cystic-lung-disease-and-pulmonary-amyloidosis
#9
Koichiro Takahashi, Hironori Sadamatsu, Shinsuke Ogusu, Kazutoshi Komiya, Tomomi Nakamura, Shinya Kimura, Naoko Sueoka-Aragane
A 72-year-old Japanese woman was noted to have multiple cystic lung shadows and infiltrates on chest radiography and computed tomography (CT). She complained of dryness of the mouth and eyes, but she did not have respiratory symptoms, such as cough, sputum production, and dyspnea. Her laboratory findings showed high titers of anti-SSA/Ro and anti-SSB/La antibodies. Surgical lung biopsy was performed and demonstrated pathologic findings of amyloid light-chain deposition and bronchiolitis with lymphocytic infiltration...
2018: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/29808153/cardiogenic-shock-an-unusual-initial-presentation-of-churg-strauss-syndrome
#10
M Apirami, J A Pratheepan, T Kumanan, M Guruparan, G Selvaratnam
Churg-Strauss syndrome (CSS) is a rare autoimmune condition, characterized by necrotizing extravascular eosinophil rich granulomatous inflammation of the tissues and disseminated small-medium sized vessel vasculitis in a patient with bronchial asthma and tissue eosinophilia. Though pulmonary involvement is the predominant feature of CSS, extra pulmonary involvement, in particular, cardiac involvement, denotes an adverse outcome. Here we report a 50-year-old female who presented with cardiogenic shock due to an acute coronary event as the initial manifestation of CSS...
2018: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/29765786/nasal-septal-perforation-in-propylthiouracil-induced-anti-neutrophil-cytoplasmic-antibody-associated-vasculitis
#11
Yusho Ishii, Tsuyoshi Shirai, Yousuke Hoshi, Yoko Fujita, Yuko Shirota, Hiroshi Fujii, Tomonori Ishii, Hideo Harigae
Here, we present the case of a 29-year-old woman with nasal septal perforation and positive myeloperoxidase- (MPO-) anti-neutrophil cytoplasmic antibody (ANCA). She had been diagnosed with Graves' disease and had been treated with propylthiouracil (PTU) for 14 months. A biopsy of the nasal septum revealed an infiltration of inflammatory cells, with no evidence of malignancy or granulomatous change. Because of the use of PTU, destructive nasal lesion, and positive MPO-ANCA, she was diagnosed with drug-induced ANCA-associated vasculitis (AAV) and was treated with prednisolone and methotrexate after the cessation of PTU...
2018: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/29755804/sapho-syndrome-masquerading-as-metastatic-breast-cancer
#12
Devin Malik, Richa Handa, Andrew Petraszko, Sheela Tejwani
SAPHO syndrome is a rare clinical entity composed of synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO). We describe a case of SAPHO syndrome masquerading as metastatic breast cancer in a patient with localized breast cancer who presented with cord compression. There was no pathologic evidence of metastatic cancer; however, a bone scan indicated osseous involvement. After multidisciplinary review of images and with additional findings of pustulosis and acne, a clinical diagnosis of SAPHO was made...
2018: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/29707401/a-rare-but-fascinating-disorder-case-collection-of-patients-with-schnitzler-syndrome
#13
Maaman Bashir, Brittany Bettendorf, Richard Hariman
Background: Schnitzler syndrome is a rare disorder characterized by a chronic urticarial rash and monoclonal gammopathy (IgM in more than 90% of the cases). It is difficult to distinguish from other neutrophilic urticarial dermatoses, and diagnosis is based on the Strasbourg criteria. Interleukin-1 is considered the key mediator, and interleukin-1 inhibitors are considered first line treatment. Here, we present two cases of Schnitzler syndrome, both successfully treated with anakinra...
2018: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/29686921/axial-spondyloarthritis-and-autosomal-dominant-polycystic-kidney-disease-in-two-siblings-a-rare-cooccurrence
#14
Ozan Volkan Yurdakul, Abdulkerim Furkan Tamer, Okan Küçükakkaş, Aylin Rezvani
Autosomal dominant polycystic kidney disease (ADPKD) is the most frequently occurring hereditary kidney disease, and axial spondyloarthritis (SpA) is one of the most frequently occurring rheumatic diseases. Treatment-related decisions for axial SpA may pose a challenge in case of renal involvement. The authors describe two siblings with cooccurrence of these two diseases. The association of these two diseases is not well known. Practitioners should monitor renal function in SpA patients and take treatment-related decisions regarding renal involvement...
2018: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/29670798/anca-vasculitis-and-hemophagocytic-lymphohistiocytosis-following-a-fecal-microbiota-transplant
#15
Adam Amlani, Amy Bromley, Aurore Fifi-Mah
A 69-year-old female with antisynthetase syndrome, a history of multiple recurrent infections, and documented previous negative titres for anti-neutrophil cystoplasmic antibody (ANCA) suddenly developed a de novo MPO-ANCA-associated glomerulonephritis three weeks after a fecal microbiota transplantation (FMT) for recurrent Clostridium difficile infections. Six months following her FMT and less than two weeks following treatment for urosepsis, she developed severe cholestasis, a markedly elevated ferritin and hypertriglyceridemia...
2018: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/29670797/recurrent-migratory-transient-bone-marrow-edema-of-the-knees-associated-with-low-vitamin-d-and-systemic-low-bone-mineral-density-a-case-report-and-literature-review
#16
Omar Alsaed, Mohammad Hammoudeh
Transient bone marrow edema (TBME) is a self-limiting disease characterized by joint pain with localized bone marrow edema by MRI and has been reported in many case series and case reports. It is well known that joints of the lower extremity including hips, knees, ankles, and feet are the classical sites for TBME. Many theories have been proposed for the pathogenesis of TBME. Systemic osteopenia and vitamin D deficiency is one of the theories that have been suggested in the last few years. In this case report, we present a middle-aged male patient, who presented with 4 attacks of TBME in both knees between September 2016 and August 2017...
2018: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/29610700/pulmonary-sarcoidosis-following-etanercept-treatment-for-ankylosing-spondylitis-a-case-report-and-review-of-the-literature
#17
A Majjad, A Bezza, A Biyi, M R El Ochi, A El Maghraoui
Antitumor necrosis factor therapies have revolutionized the treatment of some inflammatory diseases. However, the use of these agents is associated with the development of many paradoxical autoimmune diseases. Less well-recognized is the association with sarcoidosis. We report a 55-year-old female with long-standing ankylosing spondylitis who developed persistent dry cough and dyspnea while receiving etanercept therapy. High-resolution computed tomography scanning showed mediastinal lymphadenopathy and multiple nodules in both lung fields developed two months after the administration of etanercept...
2018: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/29610699/hemorrhagic-tamponade-as-initial-manifestation-of-systemic-lupus-with-subsequent-refractory-and-progressive-lupus-myocarditis-resulting-in-cardiomyopathy-and-mitral-regurgitation
#18
Nicole Marijanovich, Alexandra Halalau
Systemic lupus erythematosus (SLE) is a heterogeneous autoimmune disease with a wide range of clinical and serological manifestations. Cardiac disease among patients with SLE is common and can involve the pericardium, myocardium, valves, conduction system, and coronary arteries. We are reporting a case of SLE in a young woman that is unique is unique in that initial symptoms consisted of pericarditis and hemorrhagic tamponade which remained progressive and resistant to aggressive immunosuppressive treatment and led to severe cardiomyopathy (ejection fraction of 25%) and severe (+4) mitral regurgitation...
2018: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/29610698/three-case-reports-of-rhupus-syndrome-an-overlap-syndrome-of-rheumatoid-arthritis-and-systemic-lupus-erythematosus
#19
Gul Devrimsel, Munevver Serdaroglu Beyazal
We present the clinical and serological characteristics of three patients with rhupus. The 3 patients with rhupus presented ACR criteria for SLE as well as for RA, ANA positive with a titer of 1/100 in all patients, and positive anti-DNA in 2 of the 3 patients, with the predominance of symmetrical polyarthritis. We found anti-CCP positivity and rheumatoid factor positivity and high titers in all patients, positive anti- anti-SSA in one patient, and positive anti- anti-Sm in one patient. Renal and liver function tests were normal in all patients...
2018: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/29610697/a-case-of-spondyloarthritis-in-patient-affected-by-unicentric-castleman-s-disease-effectively-managed-with-surgery-resection-and-tocilizumab-treatment
#20
M Filippini, S Cartella, O Bonzanini, E Morello, A Tincani
A 38-year-old woman was referred to our hospital for rheumatologic manifestations (migrant arthritis and tenosynovitis), without psoriasis or family history of psoriasis, gastroenteric manifestations, or recent genitourinary infections. The instrumental and laboratory tests have suggested a diagnosis of undifferentiated seronegative HLA-B27-positive spondyloarthritis with predominantly peripheral involvement. The symptoms were very severe and resistant to anti-inflammatory drugs and steroids. She had a history of hyaline-vascular unicentric Castleman's disease (HBV, HIV, and HHV-8 negative) treated with surgery resection...
2018: Case Reports in Rheumatology
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