The Korean Journal of Internal Medicine Vol. 29, No. 5 (Suppl. 1)
WCIM 2014 SEOUL KOREA 391
Poster Session
PS 1526 Rheumatology
A Case of Dermatomyositis Predominently Involving Psoas and Paraspinal Muscles
Sang Hoon Kwon1, Jung Soo Eun1, Young Mo Kang1, Eon Jeong Nam1 Kyungpook National University Hospital, Korea1
Dermatomyositis (DM) is an idiopathic inflammatory myopathy with characteristic cutaneous manifestations such as heliotrope rash on eyelids and Gottron’s papule.
The myopathy in DM shows proximal and often symmetric muscle involvement in the extremities (hip, thigh and shoulder girdle) but rarely affects paraspinal muscles. Here, we report the case of a 43-year old man diagnosed as DM presenting predominant involvement of psoas and paraspinal muscles.
Case: A 43-year-old man presented with diffi culty of supine to sitting position and lower back pain for 3 months. On physical exams, he presented scaling, erythematous, itchy cutaneous rashes on dorsum of hands, scalp, and face, especially eyelids. He did not sit on his own in supine position and showed only a low grade muscle weakness (grade 4/5) on lower proximal extremities. Laboratory fi ndings confi rmed the muscle enzyme elevation: CPK 595 U/L (N<170), aldolase 17.8 U/L (N<7.6), serum myoglobin 442 ng/mL (N: 14-106), and LDH 1039 U/L (N: 240-480). Other laboratory fi ndings were ESR 39 mm/hr (N<10), and CRP 1.63 mg/dL (N<0.5). ANA and antibodies were negative. EMG showed polyphasic motor unit potential and increased insertional ac- tivity on psoas, paraspinal and gluteus medius muscles. Positron emission tomography demonstrated increased 18FDG uptake in psoas and paraspinal muscles but no 18FDG uptake in proximal muscles of upper and lower extremities. MRI showed hyperintense signals on T2-weighted and gadolinium-enhanced T1-weighted images on psoas and paraspinal muscles. Paraspinal muscle biopsy revealed diffuse infl ammatory endomysial and perivascular infi ltrates, which were compatible with infl ammatory myopathy. He was diagnosed as DM with predominant involvement of paraspinal and psoas muscle involvement. He was treated with high dose of steroid and immunosuppressants and showed decreased muscle enzymes and improved muscular weakness.
PS 1527 Rheumatology
A Case of Silicone Breast Implantation-Induced Sclero- derma
Areum Choe1, Jisoo Lee1, Inje Kim1 Ewha Womans University Mokdong Hospital, Korea1
The etiology of systemic sclerosis (SSc)is unknown, but many environmental agents are reported to be associated with its development. There have been case reports linking silicone implants to autoimmune diseases, especially SSc. Nevertheless, these data do not establish a clear relationship between silicone and SSc. Herein, we report a case of a 80 year old woman who developed SSc after silicone augmentation mammoplasty 40 years ago.
PS 1529 Rheumatology
Isolated Lenticulostriate Artery Aneurysm Rupture in a Patient with Behcet’s Disease
Seongjun Hwang1, YunJong Lee3, SungHae Chang2, SangWan Jung3, YouJung Ha3, EunHa Kang3, YeongWook Song1
Seoul National University Hospital, Korea1, Soonchunhyang University Cheonan Hospital, Korea2, Seoul National University Bundang Hospital, Korea3
Behcet’s disease (BD) is characterized by recurrent oro-genital ulcers, skin lesions, and intraocular infl ammation, but can affect multiple different organ systems. Arterial in- volvement is uncommon in BD and usually presents with luminal stenosis, thrombosis, or aneurysm formation in aorta and peripheral arteries. Intracranial artery involvement has been domestically reported in 4 Korean patients with BD and a single case of BD with lenticulostriate artery aneurysm has been described in the English-language liter- ature. We hereby reported the fi rst case of a Korean BD patient presenting a ruptured lenticulostriate artery aneurysm and reviewed the literature of the reported BD cases with intracranial aneurysms.
PS 1530 Rheumatology
A Case of Granulomatosis with Polyangiitis (Wegener’s Granulomatosis) Presenting with Hearing Loss
Dong Keun Seok1, Jong Hun Park1, Hyung Seok Yoon1, Eun Joo Lee1, Jin Hyun Woo1 Konkuk University Chungju Hospital, Korea1
Introduction: Granulomatosis with polyangiitis(GPA) is a multi-systemic disease with necrotizing granulomatous vasculitis in the respiratory tract and other organs. The most frequent organ systems involved at the time of diagnosis were upper respiratory tract, lung, skin, musculoskeletal and eye. We present a cases of GPA with otological manifestations as the fi rst symptom. These symptoms are subtle and diagnosis may be easily overlooked.
Case report: A 57-year-old male presented with a 2 months history of hearing loss, nasal obstruction, rhinorrhea and fever. He was treated with upper respiratory track infection at private clinic but his symptoms became worse. He was refered to uni- versity hospital. His underlying disease is only hypertension. Diffuse infl ammation on nasal cavity was also seen through endoscopic examination. Mastoiditis were seen on Magnetic Resonance Imaging of paranasal sinus. And Eustachian tube dysfunction was found by impedence test. An audiogram showed right hearing loss. Laboratory test showed elevated CRP (15.3mg/dL) and positive for MPO-ANCA. No abnormal fi ndings were seen in the enhanced chest CT. Nasal cavity biopsies showed necrotizing gran- ulomatous vasculitis. The patient was received cyclophosphamide pulse therapy and given high dose prednisolon. Patient showed a signifi cant improvement within two months of initiating treatment. C reactive protein decreased and hearing improved.
Conclusion: The clinical presentations of GPA could be diverse. Sometimes clinical manifestations are too subtle to diagnosis properly. In this case, minor various man- ifestations like hearing loss, nasal obstrucion, rhinorrhea might be ignored but after close examinations, we made a diagnosis the patient’s disease as GPA. so, like in our case, careful examinations and other tests including laboratory, image, biopsy etc are needed in order not to miss GPA.
The Korean Association of Internal Medicine
392 32nd World Congress of Internal Medicine (October 24-28, 2014)
PS 1531 Rheumatology
An Overlap Syndrome of Churg-Strauss Syndrome and Rheumatoid Arthritis
Seung Il Bae1, Jong Geol Jang1, Hun Tae Kim1, Hee Yoon Ahn1, Min Jung Kim1, Hyun Jae Kim1, Choong Ki Kim1, Young Hoon Hong1
Yeungnam University Mecical Center, Korea1
Churg-Strauss syndrome (CSS) is a necrotizing vasculitis with extra-, peri-vascular eo- sinophilic infi ltration. Chronic symmetric polyarthritis with the presence of rheumatoid factor (RF) and anti- cyclic citrullinated peptide antibody are the mainstay of rheuma- toid arthritis (RA) diagnosis. Mononeuritis multiplex is a peripheral neuropathy involv- ing more than two separate nerve areas. A 62-year-old male patient was referred for left foot drop and polyarthritis of both hands and feet for four months. Evaluations revealed mononeuritis multiplex on nerve conduction study and electromyography tests; vasculitis with neutrophil, eosinophil, and lymphocyte infi ltration on peroneal nerve biopsy(Fig.1). Pulmonary function test of the patient demonstrated positive re- sponse to methacholin and bronchodilator. Radiologic tests showed peri-articular soft tissue swelling and osteopenia on hand, foot(Fig.2); marked peripheral eosinophilia, high RF,and positive perinuclear anti-neutrophil cytoplasmic antibody on blood tests.
Here we report on a case of overlap syndrome of CSS and RA with reviews of the rel- evant literature, in which a few references to overlap syndrome of CSS and RA were available.
PS 1532 Rheumatology
Churg–Strauss Syndrome Presenting with Diffuse Alve- olar Hemorrhage
Mi-hee Kang1, Won-Seok Lee2, Wan-Hee Yoo2
Department of Internal Medicine, Chonbuk National University Medical School, Korea1, Division of Rheu- matology, Department of Internal Medicine, Chonbuk National University Medical School and Research Institute of Clinical Medicine of Chonbuk National University Hospital-Chonbuk Nation, Korea2 Introduction: Churg–Strauss syndrome (CSS) is a rare type of systemic vasculitis characterized by the presence of asthma, peripheral eosinophilia, and necrotizing vasculitis with eosinophilic infi ltration in multiple organs. Approximately 3–4% of CSS cases are associated with alveolar hemorrhage. Here we report a case of CSS with diffuse alveolar hemorrhage.
Case report: The patient was a 57-year-old woman with a 30-year history of asthma.
She was hospitalized with the complaints of fever, a multiple erythematous purpuric rash on the right upper arm and left lower leg, and progressively increasing hemop- tysis. Laboratory data indicated anemia, eosinophilia, elevated serum total immuno- globulin E, acute renal failure with nephrotic condition, and low complement level;
however, serum anti-myeloperoxidase anti-neutrophil cytoplasmic antibodies (ANCA) and anti-PR3 ANCA were within normal limits. Chest radiographs showed infi ltrative shadows in both upper lung fi elds and blunting on both Costophrenic angles. Chest computed tomography fi ndings were suggestive of alveolar hemorrhage and pneu- monia with bilateral pleural effusion. Diffuse expiratory wheezing was heard over both upper lung fi elds. The fi nding skin biopsy showed leukocytioclastic vasculitis with eosinophils and partially necrotic tissue. She was eventually diagnosed with CSS and diffuse alveolar hemorrhage. Massive hemoptysis with hypoxemia necessitated inten- sive care, so she was transferred to the intensive care unit and received steroid pulse therapy (1 g/day) for 3 days. Her clinical condition improved after the administration of steroid pulse therapy and cyclophosphamide.
Conclusion: Untreated CSS may have poor prognosis but glucocorticoid and cytotoxic agent treatment may result in clinical remission. Careful diagnosis and understanding of CSS are important to making treatment decisions and providing effective care
PS 1533 Rheumatology
A Case of Polymyalgia Rheumatica Triggered by Par- aspinal Muscle Infl ammation and Sacroiliitis
Seo Kyung Woo1, Jung Hee Koh1, Kyung-Su Park1 The Catholic University of Korea, St. Vincent Hospital, Korea1
Background: Polymyalgia rheumatica (PMR) is an infl ammatory rheumatic disease affecting mainly elderly women and characterized by morning stiffness and pain of shoulder and hip girdle, which shows dramatic response to low dose corticosteroid.
However, it is still not known what causes PMR.
Case: A 68-year-old woman presented with low back pain and left buttock pain which had lasted for 4 months. On laboratory examination, erythrocyte sedimentation rate (ESR) was 120 mm/hour and C-reactive protein (CRP) was 5.43 mg/dL. Mag- netic resonance imaging (MRI) showed bone marrow edema of the left transverse process of the fi fth lumbar spine, left iliac bone, and left sacroiliac joint as well as edema of the left paraspinal muscle (Figure 1). She was treated with vancomycin, ceftriaxone and nonsteroidal anti-infl ammatory drug (NSAID) under the suspicion of infection. Two weeks later, her symptom improved and she was discharged with oral antibiotics and NSAID. Three weeks after the discharge, her back pain aggravated and right shoulder pain developed. Ultrasound of the right shoulder revealed subacromial bursitis and ESR was elevated to 120 mm/hour and CRP to 5.71 mg/dL. However, MRI showed regression of the initial infl ammation (Figure 2). We added prednisolone 15 mg and her pain markedly improved. On the sixth day of prednisolone treatment, CRP decreased to 1.58 mg/day and she was diagnosed as having PMR based on the rapid response to the glucocorticoid.
Conclusion: We report a case of PMR which occurred following the improvement of the initial paraspinal muscle infl ammation and sacroiliitis on MRI. Based on the se- quential occurrence, initial paraspinal muscle infl ammation and sacroiliitis seemed to have caused or triggered PMR although the mechanism is not clear.
