WCIM 2014 SEOUL KOREA 237
Poster Session
The Korean Journal of Internal Medicine Vol. 29, No. 5 (Suppl. 1)
PS 0727 Rheumatology
Sudden Sensorineural Hearing Loss and Microangio- pathic Antiphospholipid Syndrome (MAPS)
AIDA GALICIA2, JUAN CARLOS ANDA3, MAXIMILIANO GARCIA1
Coordinación De Planeación De Infraestructura Médica-IMSS, Mexico1, Hospital General De Zona 2-A, Mexico2, Hospital General De Zona 2A, Mexico3
A 54-years-old man with history of acute pancreatitis 4 years before and secondary DM, he also had history of primary hypothyroidism and hypertension. He was admitted in hospital in 2010 with sudden and bilateral hearing loss, headache and dizziness.
Neuro-otological examination revealed a sensorineural hearing loss (SNHL) and he was given Dexamethasone trans-tympanic, then he was treated with Ganciclovir (12 mg/kg/24 h) for 10 days + prednisone 50 mg/24 h for 4 weeks. At the end, pure tone audiometry confi rmed a SNHL affecting both ears. aCL antibodies were positives and he was send to internal medicine consultation. The diagnosis of microangiopathic an- tiphospholipid síndrome (MAPS) on the basis of presumed thrombotic cause of SNHL was made and he was formally anticoagulated with warfarin. He has had no additional thrombotic episodes, although SNHL prevails. Full blood count, serum electrolytes, re- nal function, urinalysis and cerebrospinal fl uid analysis were normal.
Coagulation: PT 53.1”, aPTT 92.6”, Lupus anticoagulant: 1.69 (positive<1.2). Anti-ß2 Gp1 IgM:3350 (negative<8 U/ml), Anti-ß2 Gp1 IgG: 6.8 (negative<14.3 U/ml), aCL IgM: >255 (negative<15), aCL IgG: 46.3 (negative<20). ANAs, Anti-dsDNA, Anti-Sm were negative. MRI of brain on T2 weighted showed white matter hyperintensities and subcortical hyperintensities in frontal and parietal lobes, meaning microangiopathic injury on brain parenchymal.
Conclusion: MAPS is a subset of Antiphospholipid Syndrome which occlusive throm- bosis of small blood vessels and microangiophaty dominate the clinical picture. Sev- eral neurological disorders have been described in association with antiphospholipid antibodies (aPL) without thrombosis of large-vessels like Myelitis Transverse, Multiple Sclerosis-like Disease, Dementia, Chorea, Migraine and Epilepsy. SNHL is a rare syn- drome that leads rapidly progressive bilateral hearing loss, and it has been strongly associated with aPL. There are a few cases in medical literature and there is no con- sensus about appropriate treatment but anticoagulation is widely accepted.
PS 0729 Rheumatology
Predictive Signifi cance of CCL21 and CXCL13 Levels in the Minor Salivary Glands of Patients with Sjögren's Syndrome
Kyung-Eun LEE1, Dong-Jin PARK1, Jeong-Won LEE1, Ji Hyoun KANG1, Jung-Ho CHOI1, Yi Rang YIM1, Lihui WEN1, Tae-Jong KIM1, Yong-Wook PARK1, Shin-Seok LEE1 Chonnam National University Hospital, Korea1
Background: In the current study, we investigated whether the laboratory and clinical manifestations of SS patients were associated with CCL21 and CXCL13 expression levels in the minor salivary gland.
Methods: We obtained sociodemographic data on a total of 106 SS patients, docu- mented glandular and extraglandular manifestations of the disease, performed minor salivary gland biopsies, and analyzed laboratory fi ndings. EULAR index values of SS disease activity (ESSDAI values) at the time of biopsy, and SS disease damage index (SSDDI) values, were also noted. An immunohistochemical approach was used to (semiquantitatively) measure the expression levels of CCL21 and CXCL13 in the minor salivary glands.
Results: The minor salivary glands of SS patients stained positively for CCL21 and CXCL13 in 46.2% (49/106) and 70.7% (75/106) of all cases, respectively. Increased expression of CCL21 was associated with an elevated ESR, an increased IgG level, ele- vated anti-SS-A and -SS-B titers, a higher focus score, and a greater ESSDAI value at the time of biopsy. Increased expression of CXCL13 was associated with an decreased WBC, reduced lymphocyte, low platelet, elevated ESR, an increased IgG level, elevated anti-SS-A and -SS-B titers, a rise in the level of rheumatoid factor, a higher focus score, and a greater ESSDAI value at the time of biopsy. In patients with extraglandu- lar manifestations of SS, the prevalence of lymphadenopathy tended to rise with an increase in the level of CCL21.
Conclusions:The expression levels of CCL21 and CXCL13 within the lymphocytic infi l- trates of SS patients were associated with several laboratory features of the disease, lymphadenopathy, and the extent of clinical disease activity. CCL21 and CXCL13 levels should serve as useful markers predicting SS disease activity and prognosis.
PS 0730 Rheumatology
A Case of Sweet’s Syndrome Diagnosed Concomitantly with Sjögren’s Syndrome
Cheol Min JANG1, Seul Ki KIM1, Joong Kyong AHN1, Eun Jeong JOO1 Kangbuk Samsung Hospital, Korea1
Sweet’s syndrome is an uncommon reactive dermatoses characterized by fever, poly- morphonuclear leukocytosis, painful erythematous plaques and dense dermal infi ltrate of neutrophils. Sweet’s syndrome can be associated with several diseases, such as infectious diseases, malignant tumors, and autoimmune diseases. However, there is no case report of Sweet’s syndrome associated with Sjögren’s syndrome in Korea.
A 44-year-old woman presented with acute pustular rashes and fever. The patient had multiple papulopustular skin rashes, and complained fever, chills, and headache.
Our patient had the characteristic clinical and histopathological features of Sweet’s syndrome, in association with Sjögren’s syndrome (SjS), which was diagnosed through a salivary gland scan, positive anti-SSA/SSB antibody, and sicca symptoms simulta- neously. Thus, we report a case of a patient having Sweet’s syndrome diagnosed con- comitantly with SjS.
