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refractory edema in lupus patients

Ratchaya Lertnawapan, Dussadee Sakonlaya
<p>A case report of SLE with PLE in an Asian female; presented with edema, pleural effusion, ascites and profound hypoalbuminemia. She also had severe protein C and protein S depletion from GI loss which caused extensive thrombosis. Her disease was refractory to the treatment with high dose steroid, azathioprine, mycophenolate mofetil and cyclophosphamide. Bowel resection was performed without improvement. Fortunately, the patient responded to another course of pulse methyl prednisolone and a second line medication after surgery...
July 2017: Acta Reumatológica Portuguesa
Gen-Min Lin, Feng-Yee Chang, Wen-Been Wang
Libman-Sacks endocarditis is the most widely encountered aseptic endocarditis among patients with systemic lupus erythematosus. Due to the deformed cardiac valves, secondary infective endocarditis should be considered in lupus patients with acute refractory heart failure and fever of unknown origin. The case is reported of a woman with lupus and Libman-Sacks endocarditis who had concurrent coagulase-negative Staphylococcus infective endocarditis that resulted in cerebral septic emboli and acute pulmonary edema...
March 2015: Journal of Heart Valve Disease
Zahra Habibagahi, Jamshid Ruzbeh, Vahide Yarmohammadi, Malihe Kamali, Mohammad Hassan Rastegar
Angioedema secondary to C1 inhibitor deficiency has been rarely reported to be associated with systemic lupus erythematosus. A genetic defect of C1 inhibitor produces hereditary angioedema, which is usually presented with cutaneous painless edema, but edema of the genital area, gastrointestinal and laryngeal tracts have also been reported. In lupus patients, angioedema may be the result of an acquired type of C1 inhibitor deficiency, most probably due to antibody formation directed against the C1 inhibitor molecule...
July 2015: Iranian Journal of Medical Sciences
Pierina Sansinanea, Sebastián Augusto Carrica, Josefina Marcos, Mercedes Argentina García
A case is presented of a protein-losing enteropathy (PLE) as the initial manifestation of systemic lupus erythematosus (SLE) in a 17 year-old female patient, who presented with ascites, edema and hypoalbuminemia. The diagnosis of SLE was based on the presence of: malar rash, oral ulcers, thrombocytopenia, antinuclear antibodies, IgM anticardiolipin antibody, and lupus anticoagulant. Renal and liver diseases were ruled out. The PLE diagnosis was confirmed with fecal alpha 1-antitrypsin clearance. The PLE was refractory to different lines of immunosuppressive agents like glucocorticoids, cyclophosphamide, azathioprine, and cyclosporine, showing a satisfactory and sustained response with rituximab, allowing steroid sparing and long term remission...
January 2016: Reumatología Clinica
Tomoo Kise, Hiroshi Yoshimura, Shigeru Fukuyama, Masatsugu Uehara
Lupus nephritis (LN) of juvenile onset often has severe disease presentation. Despite aggressive induction therapy, up to 20% of patients with LN are resistant to initial therapy and up to 44% suffer a renal relapse. However, there is no consensus on an appropriate therapeutic regimen for refractory LN. We report a 13-year-old girl with recurrent LN who was not taking her medications. At age of 11 years, she was diagnosed with LN classified as International Society of Nephrology/Renal Pathology Society (ISN/RPS) class IV G (A) + V...
2015: Case Reports in Pediatrics
Pichaporn Artornsombudh, Ofelya Gevorgyan, Abhishek Payal, Sana S Siddique, C Stephen Foster
PURPOSE: To report the outcomes of infliximab treatment of birdshot retinochoroidopathy (BSRC) refractory to conventional immunomodulatory therapy. DESIGN: Retrospective case series. PARTICIPANTS: Twenty-two refractory birdshot retinochoroidopathy patients (44 eyes) who received infliximab between July 2005 and June 2012 were identified by retrospective chart review. METHODS: All patients received 4 to 5 mg/kg infliximab at 4- to 8-week intervals...
March 2013: Ophthalmology
Shuzo Yoshida, Takuya Kotani, Tohru Takeuchi, Kentaro Isoda, Kenichiro Hata, Koko Watanabe, Takeshi Shoda, Toru Inoue, Shigeki Makino, Toshiaki Hanafusa
A 55-year-old woman developed bilateral leg edema in June 2006. Since the edema tended to worsen, she visited our hospital on November 11. Laboratory tests showed a serum albumin level of 2.5 g/dl with 3+ proteinuria, and suggested nephrotic syndrome, which led to her hospitalization on November 14. The findings of discoid erythema, an antinuclear antibody titer of 1: 640, anti-ds DNA antibody titer of 16.8 IU/ml, and ISN/RPS class III (A/C)+V lupus nephritis on kidney biopsy led to the diagnosis of systemic lupus erythematosus...
December 2008: Nihon Rinshō Men'eki Gakkai Kaishi, Japanese Journal of Clinical Immunology
Yuki Hirai, Masayuki Iyoda, Takanori Shibata, Eijin Ashikaga, Nozomu Hosaka, Hiroki Suzuki, Hisako Nagai, Masanori Mukai, Hirokazu Honda, Aki Kuroki, Kozo Kitazawa, Tadao Akizawa
A 19-year-old female was admitted with general malaise and systemic edema. She had been diagnosed as having autoimmune hemolytic anemia (AIHA) eight years earlier and was successfully managed with oral prednisolone. During the current admission, she was diagnosed as having systemic lupus erythematosus (SLE) based on the presence of renal involvement, hematological abnormalities, and antinuclear and anti-double-stranded DNA antibodies, along with a recurrence of AIHA; her serology revealed a high myeloperoxydase-antineutrophil cytoplasmic antibody (MPO-ANCA) titer...
October 2008: Clinical and Experimental Nephrology
M Voulgarelis, S Giannouli, A Tasidou, D Anagnostou, P D Ziakas, A G Tzioufas
BACKGROUND: The histopathologic features characterizing the involvement of the bone marrow (BM) in systemic lupus erythematosus (SLE) have not been systematically analyzed to date. OBJECTIVES: The aim of this study was to assess morphologic and immunohistochemical characteristics of BM involvement in SLE. PATIENTS AND METHODS: Clinical and serological data of 40 SLE patients with unexplained cytopenias were studied. Ten patients with myelodysplasia of refractory anemia (RA) were used as controls...
August 2006: American Journal of Hematology
Christina Tietmann, Nabil F Bissada
Lupus erythematosus is considered to be a high risk factor for periodontitis. As an autoimmune disease of unknown origin, cutaneous lupus erythematosus (CLE) is subdivided into 3 categories: chronic (CCLE), subacute (SCLE), and acute (ACLE). While the ACLE has a high prevalence of conjunctive periodontal lesions, aggressive periodontitis in patients with CCLE has been rarely reported. This article describes the case of a patient diagnosed with aggressive periodontitis. Three months after the diagnosis of periodontitis, the patient experienced advancing hair loss (alopecia), pale fingers and toes, as well as edema in the legs and around the eyes...
May 2006: Quintessence International
C C Mok, K Y Ying, A Mak, C H To, M L Szeto
OBJECTIVES: To report the efficacy of prednisolone and azathioprine (AZA) in the treatment of systemic lupus erythematosus (SLE)-related protein-losing gastroenteropathy (PLGE). METHODS: Between 1995 and 2002, 16 consecutive patients with SLE-related PLGE were treated with a regimen consisting of high-dose prednisolone (0.8-1 mg/kg/day for 6 weeks, then tapered to < or =10 mg/day) and AZA (2 mg/kg/day). Protein leakage from the gastrointestinal tract was confirmed by 99mTc-labelled human serum albumin scintigraphy and significant urinary loss of protein was excluded...
April 2006: Rheumatology
Abhijit Raut, Dattatraya Muzumdar, Ranjeet Narlawar, Arpit Nagar, Nadeem Ahmed, Priya Hira
A 26-year-old woman currently treated for systemic lupus erythematosus with steroid therapy presented with sudden onset of right hemiplegia. Computed tomography of the brain showed a large frontoparietal ring-enhanced lesion with perifocal edema. Stereotactic aspiration of the lesion revealed Cladosporium bantianum. The size of the abscess did not reduce in spite of optimum antifungal treatment. The abscess was subsequently excised through a frontoparietal craniotomy. At follow up after 24 months, there was no recurrence of the abscess...
August 2003: Neurologia Medico-chirurgica
F Langer, B Eifrig, G Marx, A Stork, S Hegewisch-Becker, D K Hossfeld
Patients with malignancy often present with a variety of coagulation abnormalities which may ultimately lead to recurrent arterial and venous thromboses. Recently the presence of antiphospholipid antibodies in cancer patients has been proposed as one of the potential mechanisms promoting hypercoagulability. Here we report two consecutive patients with localized tumors, one suffering from breast cancer and another presenting with colorectal cancer, who experienced dramatic exacerbation of the antiphospholipid antibody syndrome (APAS) within 4 weeks after surgery...
December 2002: Annals of Hematology
C A Smith, R S Pinals
We have seen two patients with systemic lupus erythematosus (LE) and chronic periorbital edema. Periorbital edema occurs frequently in dermatomyositis, but it has rarely been noted in systemic LE. The edema may be associated with facial rash, but it is unrelated to the occurrence of nephrotic syndrome, cardiac and hepatic dysfunction, or edema elsewhere. This complication of systemic LE may be relatively refractory to corticosteroid therapy.
September 1982: Archives of Internal Medicine
J Moriuchi, Y Ichikawa, M Takaya, H Shimizu, T Kurata, S Arimori
Bolus intravenous cyclophosphamide therapy (IVCY) has recently been the subject of considerable attention because it is occasionally very effective in the treatment of severe lupus nephritis. However only a few reports on this form of therapy have been noted in Japan. Described here is a patient with lupus nephritis accompanied by multi-organ disorders resistant to various therapies including methylprednisolone pulse therapy which responded dramatically to IVCY. The patient, a 37-year-old woman with a history of systemic lupus erythematosus (SLE) starting in 1984, was admitted to our hospital with increasing generalized edema and malaise in January 1987...
August 1989: Ryūmachi. [Rheumatism]
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