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Case Reports in Rheumatology

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https://www.readbyqxmd.com/read/28316857/progressive-pseudorheumatoid-dysplasia-or-jia
#1
Geetha Wickrematilake
Progressive pseudorheumatoid dysplasia (PPD) or spondyloepiphyseal dysplasia tarda with progressive arthropathy (SEDT-PA) is a rare arthropathy of childhood involving the axial skeleton as well as small peripheral joints. A 10-year-old boy was referred by a general practitioner with pain and deformity in the fingers of hands and limping gait. There was no joint synovitis although the finger joints were bulky on examination with mild flexion deformity. Patient had exaggerated kyphosis and lumbar lordosis with pigeon chest and restricted hip joint movements...
2017: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/28251013/antiproteinase-3-positive-eosinophilic-granulomatosis-with-polyangiitis-presenting-with-heart-failure-and-intraventricular-thrombosis
#2
Dan Zhu, Yiming Luo, Xiangyuan Liu, Lingyun Zu
Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis commonly with cardiac complications. We describe a case of anti-PR3 ANCA-positive EGPA complicated by congestive heart failure and intraventricular thrombosis. Interestingly, the thrombus was resolved rapidly with steroid and cyclophosphamide in the setting of interrupted anticoagulation. To the best of our knowledge, we report the first case of anti-PR3 positive EGPA with extensive cardiac involvement. Our patient had overlapping features with previously studied ANCA-positive and ANCA-negative EGPA cases...
2017: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/28194293/hydralazine-induced-lupus-syndrome-presenting-with-recurrent-pericardial-effusion-and-a-negative-antinuclear-antibody
#3
Praneet Iyer, Ahmed Dirweesh, Ritika Zijoo
Drug induced lupus erythematosus (DIL or DILE) is an autoimmune disorder caused by chronic use of certain drugs. We report a unique case of hydralazine induced lupus syndrome (HILS) with a negative antinuclear antibody in a female patient who was on hydralazine for a period of 1.5-2 years and developed recurrent pericardial effusion as a result of it. Initially her condition was managed with a pericardial window. The recurrence of a massive pericardial effusion necessitated a right hemipericardiectomy. After hydralazine was stopped, she never had any further episodes of pericardial effusion or tamponade...
2017: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/28116207/primary-idiopathic-osteoarthropathy-could-it-be-related-to-alcoholism
#4
Yanal Alnimer, Suresh Subedi, Thair Dawood, Ghassan Bachuwa
Background. Hypertrophic osteoarthropathy (HOA) is a syndrome characterized by abnormal proliferation of the skin and bony structures at the distal extremities resulting in digital clubbing, periosteal bony reaction, and joint effusion. It can be primary (idiopathic) without any clear identifiable etiology as well as secondary to variety of systemic diseases most notably lung pathology. Case Presentation. We describe a rare case of primary idiopathic osteoarthropathy in a male patient who presented with severe pain and tenderness in his legs...
2017: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/28127489/a-unique-case-of-systemic-lupus-erythematosus-pelvic-vasculitis
#5
Pamela Traisak, Shristi Basnyat, Hala Eid, Patrick Cronin, Halyna Kuzyshyn, David Feinstein
The clinical presentation of Systemic Lupus Erythematosus (SLE) is diverse and vasculitis can be a potential manifestation. Cutaneous lesions involving small vessels are the most frequent presentation. However, medium and large vessel vasculitis may present with life-threatening visceral manifestations. We present a unique case of pelvic vasculitis mimicking a pelvic mass as an initial presentation of SLE. There are case reports of systemic vasculitis involving the female genital tract with giant cell arteritis (GCA), polyarteritis nodosa (PAN), and granulomatous with polyangiitis and microscopic polyangiitis (GPA/MPA), among others, but only a few cases attributed to SLE...
2016: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/28116206/postchikungunya-chronic-inflammatory-rheumatism
#6
Keith A Sacco, Razvan M Chirila
A 65-year-old male resident of Guatemala presented with a 5-month history of distal symmetric arthritis and generalized fatigue. This was associated with night sweats, chills, and weight loss. Symptoms were refractory to oral prednisone and hydroxychloroquine.
2016: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/28074166/can-cell-bound-complement-activation-products-predict-inherited-complement-deficiency-in-systemic-lupus-erythematosus
#7
Naveen Raj, Barry Waters
Activation of the classical pathway complement system has long been implicated in stimulating immune complex mediated tissue destruction in systemic lupus erythematosus (SLE). C3 and C4 complement levels are utilized as part of SLE diagnosis and monitoring criteria. Recently, cell bound complement activation products (CBCAPs) have shown increased sensitivity in diagnosing and monitoring lupus activity, compared to traditional markers. CBCAPs are increasingly utilized in rheumatology practice as additional serological markers in evaluating SLE patients...
2016: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/28050304/chronic-and-asymptomatic-diffuse-alveolar-haemorrhage-with-microscopic-polyangiitis-a-case-report-and-review-of-the-literature
#8
Hiroki Tashiro, Koichiro Takahashi, Hironori Sadamatsu, Masaru Uchida, Shinya Kimura, Naoko Sueoka-Aragane
Diffuse alveolar haemorrhage (DAH) is one of the major causes of death in microscopic polyangiitis (MPA) patients, because of acute respiratory failure with various respiratory symptoms. We, herein, present a case of chronic and asymptomatic DAH in a patient with MPA who was diagnosed by fibreoptic bronchoscopy. The patient showed localized reticular shadows, without any respiratory symptoms, and absence of inflammatory reactions, such as fever and CRP elevation, which is atypical for DAH. Three months after appearance of the lung abnormalities, DAH with MPA was diagnosed by fibreoptic bronchoscopy...
2016: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/28018698/combination-immunosuppressive-therapy-including-rituximab-for-epstein-barr-virus-associated-hemophagocytic-lymphohistiocytosis-in-adult-onset-still-s-disease
#9
Eva Johanna Schäfer, Wolfram Jung, Peter Korsten
Hemophagocytic lymphopcytosis (HLH) is a life-threatening condition. It can occur either as primary form with genetic defects or secondary to other conditions, such as hematological or autoimmune diseases. Certain triggering factors can predispose individuals to the development of HLH. We report the case of a 25-year-old male patient who was diagnosed with HLH in the context of adult-onset Still's disease (AOSD) during a primary infection with Epstein-Barr virus (EBV). During therapy with anakinra and dexamethasone, he was still symptomatic with high-spiking fevers, arthralgia, and sore throat...
2016: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/27994906/swift-and-complete-healing-of-digital-ulcers-after-macitentan-treatment
#10
Emilio Giner Serret
Digital ulcers are a burdensome and painful condition with sparse options of treatment. We report the case of a 78-year-old female patient with limited cutaneous systemic sclerosis that sequentially developed digital ulcers. After the appearance of digital ulcers in the soles of her feet she was successfully treated with bosentan. The report of two new digital ulcers in her hands 9 months later alongside with elevated transaminase levels led to a switch to macitentan treatment. A swift and complete healing of both digital ulcers was observed after 3 months, with the restoration of normal biochemical values...
2016: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/27885350/a-case-of-dermatomyositis-and-anti-ej-autoantibody-with-chronic-intestinal-pseudoobstruction-successfully-treated-with-octreotide
#11
Chiho Yamada, Shinji Sato, Noriko Sasaki, Takayoshi Kurabayashi, Sho Sasaki, Yasushi Koyama, Naofumi Chinen, Takayuki Wakabayashi, Yasuo Suzuki
Chronic intestinal pseudoobstruction (CIPO) is a serious complication in patients with connective tissue disease (CTD) and is sometimes life-threatening or fatal despite intensive medical treatment. Here, we report a patient with dermatomyositis (DM) and anti-EJ autoantibody who developed CIPO that was improved by octreotide. Because her abdominal pain and bloatedness were so severe and persistent, we introduced octreotide to relieve symptoms. In this case, continuous intravenous administration as well as long-acting subcutaneous injection of octreotide was effective for treating CIPO...
2016: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/27843668/rituximab-not-effective-for-hearing-loss-in-cogan-s-syndrome
#12
Daniel R Bunker, Leslie Dubin Kerr
Importance. Rituximab was not effective in ameliorating the hearing loss in a patient with atypical Cogan's syndrome. Observations. We report the case of a patient who developed acute bilateral uveitis and sensorineural hearing loss. A diagnosis of atypical Cogan's syndrome was made. The patient's hearing loss did not improve despite high dose steroids and azathioprine. Rituximab was administered given a recent report of its efficacy in a patient with refractory disease; however, our patient's hearing loss did not improve...
2016: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/27822400/hemorrhagic-pericardial-effusion-with-tamponade-a-rare-adverse-effect-of-infliximab-case-report-and-literature-review
#13
Henry D Lather, J Michelle Kahlenberg
Introduction. Antitumor necrosis factor (TNF) alpha agents are commonly used biologic therapies for a wide variety of rheumatic and inflammatory diseases. Here, we present a case of hemorrhagic pericarditis as a consequence of infliximab and review the literature on pericardial complications stemming from this drug class. Methods. For the literature review, search terms using versions of antitumor necrosis factor alpha AND pericardial effusion OR pericarditis OR pleuropericarditis OR cardiac tamponade were used...
2016: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/27818826/macrophage-activation-syndrome-associated-with-adult-onset-still-s-disease-successfully-treated-with-anakinra
#14
Aswini Kumar, Hiroshi Kato
Macrophage activation syndrome (MAS) is a potentially fatal complication of Adult-Onset Still's disease (Still's disease). Whereas an increasing body of evidence supports interleukin-1 (IL-1) blockade as a promising treatment for Still's disease, whether it is therapeutic for MAS associated with Still's disease remains unclear. We report a 34-year-old Caucasian man with one-decade history of TNF-blockade-responsive seronegative arthritis who presented with abrupt onset of fever, serositis, bicytopenia, splenomegaly, hepatitis, and disseminated intravascular coagulation...
2016: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/27803833/coexistent-pseudogout-and-mycobacterium-avium-intracellulare-septic-arthritis-in-a-patient-with-hiv-and-esrd
#15
Wais Afzal, Omer M Wali, Kelly L Cervellione, Bhupinder B Singh, Farshad Bagheri
Pseudogout is a crystal-induced arthropathy characterized by the deposition of calcium pyrophosphate dihydrate (CPPD) crystals in synovial fluid, menisci, or articular cartilage. Although not very common, this entity can be seen in patients with chronic kidney disease (CKD). Septic arthritis due to Mycobacterium avium-intracellulare (MAI) is a rare entity that can affect immunocompromised patients such as those with acquired immunodeficiency syndrome (AIDS) or those who are on immunosuppressive drugs. Here, we describe a 51-year-old female who presented with fever, right knee pain, swelling, warmth, and decreased range of motion for several days...
2016: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/27752386/a-case-of-abdominal-aortic-retroperitoneal-and-mesenteric-amyloid-light-chain-amyloidoma
#16
Kazuhiro Yokota, Dai Kishida, Hidekazu Kayano, Masahide Yazaki, Yuki Shimada, Yuji Akiyama, Toshihide Mimura
We report the case of a Japanese woman with amyloid light chain (AL) amyloidoma in the abdominal aortic retroperitoneum and mesentery. Irregular soft tissue mass lesions with calcification in the abdominal aortic retroperitoneum and mesentery were initially detected by computed tomography at another hospital. The lesions gradually compressed the duodenum, causing symptoms of bowel obstruction. The patient was clinically diagnosed with retroperitoneal fibrosis, and prednisolone was administered at a dose of 40 mg/day...
2016: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/27752385/adult-onset-henoch-schonlein-purpura-and-intussusception-a-rare-presentation
#17
Mridula Krishnan, Joseph Nahas
We present an unusual case of a young 26-year-old male who was diagnosed with Henoch-Schonlein Purpura (HSP). Initial presentation was primarily mild gastrointestinal symptoms, which progressed to a life threatening intussusception and subsequently resolved with prompt glucocorticoid use rather than typical surgical intervention. Of importance, the patient's initial gastrointestinal symptoms without associated skin manifestations made the diagnosis difficult. In conclusion, it is important to recognize uncommon presentations of HSP as it may lead to life threatening complications and surgical intervention may be avoided with prompt treatment...
2016: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/27703830/a-curious-case-of-proximal-muscle-weakness-with-eosinophilic-polymyositis
#18
Ciel Harris, Robert Ali, Julio Perez-Downes, Firas Baidoun, Marianne DeLima, Jaimin Shah, Win Aung, Raafat F Makary
Eosinophilic polymyositis (EPM) is part of a rare disorder, eosinophilic myopathies (EM), which is a form of polymyositis characterized by the presence of eosinophils in muscle biopsy sections and occasionally blood eosinophilia. Herein, we are presenting an interesting case of eosinophilic polymyositis presenting with muscle pain with no other organ systems involved.
2016: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/27699076/heparin-related-thrombocytopenia-triggered-by-severe-status-of-systemic-lupus-erythematosus-and-bacterial-infection
#19
Satoshi Suzuki, Shihoko Nakajima, Taiki Ando, Keisuke Oda, Manabu Sugita, Kunimi Maeda, Yutaka Nakiri, Yoshinari Takasaki
A patient with severe lupus nephritis developed thrombocytopenia during treatment with high-dose steroids. In addition to viral- or disease-induced cytopenia, the pathology was believed to arise from diverse contributing factors, such as thrombotic microangiopathy and heparin-related thrombocytopenia (HIT). By combining plasma exchange therapy and intravenous cyclophosphamide, we successfully controlled the SLE activity and improved the thrombocytopenia. An antecedent bacterial infection or SLE activity is believed to have contributed to the concurrent HIT...
2016: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/27672472/a-case-of-acute-budd-chiari-syndrome-complicating-primary-antiphospholipid-syndrome-presenting-as-acute-abdomen-and-responding-to-tight-anticoagulant-therapy
#20
Naofumi Chinen, Yasushi Koyama, Shinji Sato, Yasuo Suzuki
A 34-year-old woman with primary antiphospholipid syndrome was admitted to the Gastroenterology Department of our hospital with fever, acute abdomen, watery diarrhea, and extremely high levels of inflammatory parameters. She had a history of left lower limb deep vein thrombosis and pulmonary embolism and was taking warfarin potassium. Acute gastroenteritis was suspected and an antibiotic was administered, but symptoms progressed. Abdominal ultrasonography showed occlusion of the left hepatic vein and the middle hepatic vein and her D-dimer level was high...
2016: Case Reports in Rheumatology
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