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Case Reports in Rheumatology

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https://www.readbyqxmd.com/read/29765786/nasal-septal-perforation-in-propylthiouracil-induced-anti-neutrophil-cytoplasmic-antibody-associated-vasculitis
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
https://www.readbyqxmd.com/read/29552370/effect-of-adalimumab-on-refractory-arthritis-in-juvenile-idiopathic-inflammatory-myopathy-with-anti-mda5-autoantibody
#11
Takako Miyamae, Takuma Hara, Aki Hanaya, Yumi Tani, Takayuki Kishi, Hisashi Yamanaka
A 10-year-old girl manifested persistent fever, skin rash, leg pain, fatigue, and joint pain. Based on muscle weakness, elevated muscle-derived enzymes, magnetic resonance imaging, and skin biopsy results, the diagnosis was juvenile idiopathic inflammatory myopathies (JIIM). Chest CT was normal; the anti-melanoma differentiation-associated protein-5 (anti-MDA5) autoantibody was positive. Initial manifestations subsided after prednisolone (PSL) and methotrexate treatment. After the PSL dosage was decreased, the patient presented with metacarpophalangeal (MCP) joint pain and swelling in both index fingers, synovial fluid, and signals on power Doppler ultrasound...
2018: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/29552369/efficacy-of-cyclosporine-in-the-induction-and-maintenance-of-remission-in-a-systemic-lupus-erythematosus-patient-presenting-with-macrophage-activating-syndrome
#12
Franchesca Cruz-Pérez, Salvador Vilá, Grissel Ríos, Luis M Vilá
Macrophage-activating syndrome (MAS) is a rare condition characterized by dysfunctional macrophage activation leading to overproduction of cytokines and phagocytosis of erythrocytes, leukocytes, and platelets. MAS is associated with infectious diseases, malignancies, and autoimmune rheumatic disorders. Herein, we present a 22-year-old Hispanic woman with SLE who was hospitalized because of a three-week history of fever, fatigue, polyarthralgia, nausea, and abdominal pain. Initial laboratories showed severe pancytopenia with marked elevation of liver enzymes and ferritin levels...
2018: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/29445561/thoracic-paravertebral-mass-as-an-infrequent-manifestation-of-igg4-related-disease
#13
Melissa Matzumura Kuan, Bernard Rubin, Alireza Meysami
Case: A 50-year-old African American male presented with abdominal pain and significant weight loss. On physical examination, he had parotid and submandibular gland enlargement associated with right eye proptosis. Computed tomography showed a thoracic paravertebral soft tissue mass, enlarged lymph nodes, and ascending aortic aneurysm. Laboratory results were remarkable for elevated total IgG and IgG4 subclass. The submandibular gland pathology revealed chronic sclerosing sialadenitis, with a large subset of inflammatory cells positively staining for IgG4...
2017: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/29312791/sarcoidosis-and-systemic-sclerosis-strange-bedfellows
#14
Micah Yu, Vaneet K Sandhu, Sheila D Lezcano, Kanwaljeet Maken, Shannon Kirk, Karina D Torralba
Coexistence of systemic sclerosis and sarcoidosis is rare. Both have predominant lung manifestations, each with distinctive features on computed tomography (CT) of the chest. We present herein a 52-year-old male with limited systemic sclerosis manifested primarily by sclerodactyly and subsequently by shortness of breath. A series of CT scans of the chest were reviewed. Initial CT chest one year prior to sclerodactyly onset revealed bilateral hilar and right paratracheal, prevascular, and subcarinal adenopathy...
2017: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/29259835/efficacy-of-rituximab-in-a-systemic-lupus-erythematosus-patient-presenting-with-diffuse-alveolar-hemorrhage
#15
Gabriela Montes-Rivera, Grissel Ríos, Luis M Vilá
Diffuse alveolar hemorrhage (DAH) is a life-threatening complication of systemic lupus erythematosus (SLE). Although infrequent, its mortality is very high. While there are no established therapeutic guidelines, DAH has been traditionally managed with high-dose intravenous (IV) corticosteroids, cyclophosphamide, and plasma exchange. The efficacy of alternative therapies such as rituximab has been described only in a few cases. Herein, we report a 25-year-old Hispanic man who presented with acute-onset SLE manifested by polyarthralgia, nephritis, seizures, pancytopenia, severe hypocomplementemia, and elevated anti-dsDNA antibodies...
2017: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/29225988/effective-administration-of-rituximab-in-anti-mda5-antibody-positive-dermatomyositis-with-rapidly-progressive-interstitial-lung-disease-and-refractory-cutaneous-involvement-a-case-report-and-literature-review
#16
Yuka Ogawa, Dai Kishida, Yasuhiro Shimojima, Koichi Hayashi, Yoshiki Sekijima
We describe the case of a 48-year-old man with dermatomyositis (DM) who demonstrated rapidly progressive interstitial lung disease (RP-ILD) and refractory cutaneous involvement together with high levels of anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5-Ab). Even after combination immunosuppressive therapy including a corticosteroid, cyclosporine A, and intravenous cyclophosphamide, his respiratory insufficiency and cutaneous involvement progressively worsened. However, the administration of rituximab (RTX) resulted in clinical remission as well as a visible reduction in anti-MDA5-Ab levels, suggesting that RTX could be a useful remedy in cases refractory to conventional immunosuppressive agents, especially those of RP-ILD related to anti-MDA5-Ab-positive DM...
2017: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/29209549/macrophage-activation-syndrome-a-report-of-two-cases-and-a-literature-review
#17
Asaad Alkoht, Ibrahem Hanafi, Basheer Khalil
Macrophage activation syndrome (MAS) is a severe, potentially fatal condition that may complicate autoimmune diseases, and it belongs to hemophagocytic lymphohistiocytosis (HLH) disorders. MAS occurs in adults and children. However, it is rare in juvenile systemic lupus erythematosus (jSLE), and it is extremely rare to be the initial presentation of jSLE. Here, we report two patients with juvenile SLE who initially presented with MAS. One of the two patients is 4 years old. This is the youngest reported patient to our knowledge...
2017: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/29104809/-mycobacterium-intracellulare-infection-mimicking-progression-of-scleroderma
#18
Simon Krabbe, Merete Engelhart, Sören Thybo, Søren Jacobsen
This case report describes a patient with scleroderma who developed Mycobacterium intracellulare infection, which for more than a year mimicked worsening of her connective tissue disorder. The patient was diagnosed with scleroderma based on puffy fingers that developed into sclerodactyly, abnormal nail fold capillaries, interstitial lung disease, Raynaud's phenomenon, esophageal dysmotility, and positivity for rheumatoid factor and anti-SSA antibodies. She developed massive inflammatory changes of the cutis, the subcutis, and the muscle fasciae of the right leg, that after several failed attempts of immunosuppressive treatments were found to be caused by Mycobacterium intracellulare ...
2017: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/29104808/the-foot-that-broke-both-hips-a-case-report-and-literature-review-of-tumor-induced-osteomalacia
#19
Sara Beygi, Alfred Denio, Tarun S Sharma
Tumor-induced osteomalacia (TIO) is a rare paraneoplastic syndrome characterized by hypophosphatemia and clinical symptoms of osteomalacia. Only discussed as case reports, there is still limited knowledge of this condition as a potentially curable cause of osteomalacia among clinicians and pathologists. In this article, we present a case of tumor-induced osteomalacia in a 59-year-old gentleman followed by an up-to-date review of the existing literature on TIO.
2017: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/29062579/successful-treatment-of-hemophagocytic-lymphohistiocytosis-associated-with-lupus-nephritis-by-using-mycophenolate-mofetil
#20
Takashi Nawata, Makoto Kubo, Kosaku Shiragami, Yukinori Nakamura, Masafumi Yano
An estimated 0.9% to 2.4% of patients with systemic lupus erythematosus (SLE) also have hemophagocytic lymphohistiocytosis (HLH). HLH associated with autoimmune diseases is often refractory to corticosteroid treatment; thus, additional immunosuppressive drugs, such as cyclosporine, cyclophosphamide, or tacrolimus, are required. Here, we describe the case of a 44-year-old Japanese woman who developed HLH associated with lupus nephritis. Initially, her HLH was refractory to treatment with a corticosteroid, tacrolimus, and mizoribine...
2017: Case Reports in Rheumatology
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