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Case Reports in Rheumatology

Pragya Shrestha, Brian Le, Brent Wagner, William Pompella, Paras Karmacharya
IgG4-related sclerosing cholangitis (IgG4-SC) is one of the most common extra-pancreatic manifestation of IgG4-related disease (IgG4-RD) and is clinically distinct from primary sclerosing cholangitis (PSC). IgG4-RD is an increasingly recognized immune-mediated fibroinflammatory systemic disease, mostly affecting middle-aged and older male populations that can affect multiple organs. The presence of extra-biliary clinical manifestations of IgG4-RD, such as parotid and lacrimal swelling, lymphadenopathy, autoimmune pancreatitis, and retroperitoneal fibrosis, if present could provide important clues to diagnosis...
2018: Case Reports in Rheumatology
Natsuki Shima, Keiichi Sumida, Masahiro Kawada, Akinari Sekine, Masayuki Yamanouchi, Rikako Hiramatsu, Noriko Hayami, Eiko Hasegawa, Tatsuya Suwabe, Junichi Hoshino, Naoki Sawa, Kenmei Takaichi, Kenichi Ohashi, Takeshi Fujii, Yoshifumi Ubara
A 42-year-old woman with systemic lupus erythematosus (SLE) was admitted to our hospital for evaluation of severe thrombocytopenia. She was treated with steroids, intravenous cyclophosphamide, intravenous immunoglobulin, and plasma exchange, but her thrombocytopenia did not improve. Renal biopsy showed class IV-S(C) + V lupus nephritis, according to the classification of the International Society of Nephrology/Renal Pathology Society. The PA-IgG and serum thrombopoietin (TPO) levels were elevated. Her thrombocytopenia responded to off-label administration of eltrombopag, which was discontinued after 42 months...
2018: Case Reports in Rheumatology
Hidekazu Ikeuchi, Kana Koinuma, Masao Nakasatomi, Toru Sakairi, Yoriaki Kaneko, Akito Maeshima, Yuichi Yamazaki, Hiroaki Okamoto, Toshihide Mimura, Satoshi Mochida, Yoshihisa Nojima, Keiju Hiromura
Hepatitis E is an acute self-limiting disease caused by hepatitis E virus (HEV). Recent reports show that HEV can induce chronic hepatitis or be reactivated in immunocompromised hosts. We report a 63-year-old woman with rheumatoid arthritis (RA) who developed hepatitis E during treatment with tocilizumab. Analysis of serially stocked serum samples confirmed that hepatitis was caused by primary infection with HEV and not by viral reactivation. Her liver function improved after discontinuing tocilizumab and remained within the normal range without reactivation of HEV for >5 years after restarting tocilizumab...
2018: Case Reports in Rheumatology
Varun Jain, Maryann Aziz, Mina G Banoub, Jeremy T Neuman, Richard Sidlow
The pulmonary manifestations of systemic lupus erythematosus can range in severity from mild to life threatening and can be particularly marked in women who are recently postpartum. We present below a seventeen-year-old female patient, one month postpartum, who had findings consistent with an acute infectious pneumonia whom upon further query and passage of time was diagnosed with severe pneumonitis due to systemic lupus erythematosus.
2018: Case Reports in Rheumatology
Ramandeep Bains, Tamara Dahhan, Annie Belzowski, Emil R Heinze, Andrew L Wong, Philip J Clements
This case describes a patient presenting with acute onset papilledema, subacute strokes resulting in upper extremity weakness and numbness, arthritis, maculopapular rash, depressed C4 and CH50, and a high titer anti-double-stranded DNA antibody. The patient was given the diagnosis of probable systemic lupus erythematosus, which was supported by the Systemic Lupus International Collaborating Clinics (SLICC) criteria. He was aggressively treated for neuropsychiatric lupus (NPSLE) with pulse dose steroids and a dose of intravenous cyclophosphamide...
2018: Case Reports in Rheumatology
Alexander Zheutlin, Elena Schiopu
Relapsing polychondritis (RP) is an autoimmune disorder that often occurs concomitantly with other autoimmune diseases, though RP has been infrequently associated with ankylosing spondylitis (AS). There is a small, but growing, body of the literature demonstrating case reports describing RP secondary to AS in patients treated with tumor necrosis alpha inhibitors (TNFi's). We present the first case in which RP developed in AS while treated with an interleukin 17A inhibitor (IL-17Ai), secukinumab. With this case report, we hope to raise physician awareness of the possible autoimmune disorders that may arise subsequent to novel immunomodulation therapies, particularly that RP may develop subsequent to inhibition of IL-17A...
2018: Case Reports in Rheumatology
Ayushi Dixit, Adriana Abrudescu
Statin-induced necrotizing autoimmune myopathy (SINAM) is a rare side effect of statin use which manifests as progressive muscle weakness. Because statins are a widely prescribed medication for coronary artery disease, hyperlipidemia, and many other diseases, many patients are at risk of developing SINAM or one of the many other statin-induced myopathies. Due to identification of an antibody specific to this disease, we were able to diagnose SINAM in a patient whose symptoms had progressed to the extent that they were debilitating...
2018: Case Reports in Rheumatology
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
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
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
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
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
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
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
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
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
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
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
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
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
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