The Korean Association of Internal Medicine
388 32nd World Congress of Internal Medicine (October 24-28, 2014)
PS 1514 Rheumatology
A Case of Neurogenic Bladder in a Patient with Sys- temic Lupus Erythematosus Treated with Intravenous Cyclophosphamide and Glucocorticoid Pulse Therapy
Kyoung Hwa Lee1, Mi Ryoung Seo1, Hee Jung Ryu1, Hyo Jin Choi1, Han Joo Baek1 Gachon University Gil Hospital, Korea1
Systemic lupus erythematosus (SLE) is a chronic infl ammatory disease that can affect any part of the body including the central and peripheral nervous systems. However, neurogenic bladder associated with SLE has rarely been reported. We experienced a case of neurogenic bladder in a female patient with SLE treated with intravenous cyclophosphamide and glucocorticoid pulse therapy. A 46-year-old female visited the hospital because of voiding diffi culty developed 3 days before. She had been diag- nosed with SLE in 2005 and treated with maintenance doses of hydroxychloroquine (200mg/day) and prednisolone (5mg/day). She could not urinate almost at all and residual bladder volume was 400ml checked by Foley catheterization. Blood tests displayed positive antinuclear antibody and anti-double stranded DNA antibody. ESR (57 mm/hour) and CRP (1.07 mg/dL) were elevated but complement level was normal.
CT showed distended bladder with bilateral hydroureteronephrosis. Detrusor muscle contractility was decreased by urofl owmetry study. There was nothing remarkable by brain and spine MRI except small cystic lesion of posterior fossa that did not change in size compared to 10 years before. Her bladder dysfunction was presumed to be complicated by peripheral neuron disease associated with SLE. Intravenous methyl- predisolone (1g/day) for 3 days and cyclophosphosphamide pulse therapy (1g/day) followed by high doses of prednisolone (40mg/day) were administered. After that her urinary retention was gradually improved. She was able to urinate a month later and had no voiding diffi culty at 10-month follow-up.
PS 1515 Rheumatology
A Case of Systemic Lupus Erythematosus Presenting as Digital Gangrene Due to Vasculitis
Hyo Yeop Song1
Wonkwang University of Medicine & Hospital, Korea1
Systemic lupus erythematosus (SLE) is a complicated autoimmune disease that can affect the skin, joints, kidneys, brain and other organs. Digital ulcers and gangrene are common skin manifestations of connective tissue diseases, especially systemic scle- rosis, although they are relatively rare in SLE. In SLE, because digital gangrene results from vasculitis, vasospasm and thromboembolism, physician should consider lupus vasculitis, anti-phospholipid syndrome and other autoimmune diseases. A 51 year- old woman presented to pain and gangrene on the dorsum of foot and the fi rst toe of right side. She was diagnosed SLE one year ago. CT angiography showed irregularity and narrowing in anterior tibia artery, angiography showed diffuse narrowing in vessel below knee but no evidence of atherosclerosis and thromboembolism. Anterior tibia artery showed stenosis, not responded to nitrate and re-stenosis occurred after bal- loon dilatation. There was no evidence of serum anti-phospholipid antibody, ANCA, other thrombotic disease, connective tissue disease and infection or malignancy. We injected intramuscular enoxaparin sodium and intravascular prostaglandin E1 for symptom relief and administrated high dose glucocorticoid and rituximab. After we identifi ed digital gangrene didn’t developed more, performed amputation of necrotic region was done. We report SLE patient who presented with digital gangrene and present a review of the relevant literature.
PS 1516 Rheumatology
Three Cases of Secondary Hemophagocytic Lymphohis- tiocytosis Associated with Systemic Erythematosus Lupus
Eunsoo Lim1, Chang-Hee Suh1, Young geon Kim1, Won sun Choi1, Yu soek Jung1, Jae ho Han1, Chang-Bum Bae1, Ju-Yang Jung1, Hyoun-Ah Kim1
Ajou University Hospital, Korea1
Background: Secondary hemophagocytic lymphohistiocytosis (HLH) can occur during the course of systemic lupus erythematosus (SLE), but also can also be a presenting manifestation.
Case1: The 13-year old girl who was diagnosed with SLE a year ago presented with fever. Since she had redness and tenderness on her left fi fth fi ngertip, we assumed that cellulitis was the cause of fever and treated her with antibiotics. Her fever persisted, so we increased her steroid to treat SLE fl are which might have been overlooked. However, pancytopenia, hyperferritinemia and hypertriglyceridemia developed, we biopsied her bone marrow. She was diagnosed with secondary HLH and received steroid pulse therapy.
Case2: The 27-year old woman presented with fever and bleeding tendency. Her laboratory results revealed pancytopenia, hyperferritinemia and hypertriglyceridemia.
We biopsied her bone marrow and she was diagnosed with HLH. She had pericardial effusion, proteinuria and low serum complement level and was positive for anti-nucle- ar antibody and anti-double strand DNA antibody. As a result, she was diagnosed with SLE at the same time. We treated her with steroid, etoposide and cyclosporine.
Case3: The 30-year old woman who was diagnosed with SLE fifteen years ago presented with fever. We suspected SLE flare and increased her steroid. Despite treatment, she constantly had fever and her laboratory results showed pancytopenia, hyperferritinemia and hypertriglyceridemia, we gave her steroid pulse therapy and biopsied her bone marrow. She was diagnosed with secondary HLH. All three patients became afebrile and recovered from their pancytopenia after treatment.
Conclusion: Since pancytopenia develops in less than 10 percent of SLE cases, the investigation for HLH is necessary when otherwise unexplained pancytopenia persists despite adequate treatment. Hereby we report three successfully treated cases, and discuss the prevalence, characteristics, treatments and prognosis for secondary HLH associated with SLE.
PS 1517 Rheumatology
Mucosal-Associated Invariant T Cells Are Numerically and Functionally Defi cient in SLE and Their Defi ciency Refl ect Disease Activity
Ki-Jeong Park1, Young-Nan Cho1, Hye-Mi Jin1, Kyung-Eun Lee1, Jeong-Won Lee1, Jeong-Hwa Kang1, Hyun-Ju Jung1, Yi-Rang Yim1, Jung-Ho Choi1, Dong-Jin Park1, Sung-Ji Lee1, Tae-Jong Kim1, Shin-Seok Lee1, Yong-Wook Park1
Department of Rheumatology, Chonnam National University Hospital, Korea1
Background: Mucosal-associated invariant T (MAIT) cells contribute to protection against certain microorganism infections and play an important role in mucosal immuni- ty. However, the role of MAIT cells remains enigmatic in autoimmune diseases. Here, we examined the level and function of MAIT cells in patients with rheumatic diseases.
Methods: Patients with systemic lupus erythematosus (SLE; n = 54), rheumatoid arthritis (RA; n = 66), Behçet’s disease (n = 9), ankylosing spondylitis (n = 21), and healthy controls (n = 136) were enrolled in the study. MAIT cell, cytokine and pro- grammed death-1 (PD-1) levels were measured by fl ow cytometry.
Results: Circulating MAIT cell levels were signifi cantly reduced in SLE and RA pa- tients. In particular, this MAIT cell deficiency was more prominent in CD8+ and double-negative T cell subsets, and signifi cantly correlated with disease activity, such as SLE disease activity index (SLEDAI) and 28-joint disease activity score (DAS28).
Interestingly, MAIT cell frequency was significantly correlated with natural killer T (NKT) cell frequency in SLE patients. IFN-γ production in MAIT cells was impaired in SLE patients, which was due to an intrinsic defect in the Ca2+/calcineurin/NFAT1 signaling pathway. In SLE patients, MAIT cells were poorly activated by a-galactosyl- ceramide-stimulated NKT cells, thereby showing the dysfunction between MAIT cells and NKT cells. Notably, an elevated expression of PD-1 in MAIT cells and NKT cells was associated with SLE. In RA patients, MAIT cell levels were signifi cantly higher in synovial fl uid than in peripheral blood.
Conclusions: Our study primarily demonstrates that MAIT cells are numerically and functionally defi cient in SLE. In addition, we report a novel fi nding that this MAIT cell defi ciency is associated with NKT cell defi ciency and elevated PD-1 expression. These abnormalities possibly contribute to dysregulated mucosal immunity in SLE.
