Buerger’s Disease May be a Chronic Rickettsial Infection with Superimposed Thrombosis: Literature Review and Efficacy of Doxycycline in Three Patients

Buerger’s disease (BD) is a chronic inflammatory vasculitis of unknown etiology. The infectious etiology of BD was proposed by Buerger in 1914. Furthermore, there are scattered reports insisting that BD may be related to rickettsial infection, first asserted by Goodman since 1916, followed by Giroud and other French investigators from the 1940s through the 1960s, Nicolau in the 1960s, Bartolo (1980s), and Fazeli (2010s). However, their causal relationship has hardly been accepted because rickettsial infections are known to be acute febrile, vector-borne illnesses, whereas BD is a chronic afebrile illness. In this article we review the relevant literature on the chronic nature of Rickettsia and Orientia infections and on the rickettsial etiology of BD. Excellent initial responses to doxycycline in three patients with BD are briefly described. Based on these findings, we hypothesize that BD patients acquired a rickettsial infection far before the onset of BD. Over years, the infected area expands to become a segment of the infected vessel. Subsequently, thrombus develops on the luminal surface of the infected endothelial cells, which produces the vascular obstructive manifestations of BD. Collectively, it is postulated that BD is a chronic infection with a member of the family Rickettsiaceae with superimposed thrombosis.

Altered Influenza Vaccination Coverage and Related Factors in Pregnant Women in Korea from 2007 to 2019

Background@#Pregnant women are at a high-risk of influenza infection. We have previously reported a low influenza vaccination coverage rate (4.0%) in Korea during the 2006–2007 influenza season. We conducted follow-up studies in 2011–2012 and 2018–2019 to observe changes in influenza vaccination coverage. @*Methods@#Women who delivered at Inha University Hospital (Incheon, Korea) in 2011–2012 and 2018–2019 were enrolled in the study. We surveyed the rate of influenza vaccination, perception scores, and related factors through telephonic interviews and compared the results from 2011–2012 and 2018–2019 with those from 2006–2007. @*Results@#The number of survey respondents was 227 in 2006–2007, 152 in 2011–2012, and 171 in 2018–2019. The rate of vaccination coverage increased from 4.0% in 2006–2007 to 42.0% in 2011–2012 and 59.3% in 2018–2019. Perception scores also increased progressively from 3.8 in 2006–2007 to 4.2 in 2011–2012 and 5.1 in 2018–2019. Physician recommendations for influenza vaccination also increased from 4.8% in 2006–2007 to 36.8% in 2011–2012 and 49.7% in 2018–2019. The most common reason for not getting vaccinated was the lack of awareness of influenza vaccination during pregnancy (36.9%). The perception scores and physician recommendation rates were significantly lower for unvaccinated women (3.87 and 8.4%, respectively) than for vaccinated women (5.14 and 69.1%, respectively). @*Conclusion@#The influenza vaccination coverage rate in pregnant women has increased significantly since our study in 2006–2007. However, further improvement in the coverage rate is needed. There is a need for active and comprehensive publicity and education regarding this issue among physicians and pregnant women.

Altered Influenza Vaccination Coverage and Related Factors in Pregnant Women in Korea from 2007 to 2019

Background@#Pregnant women are at a high-risk of influenza infection. We have previously reported a low influenza vaccination coverage rate (4.0%) in Korea during the 2006–2007 influenza season. We conducted follow-up studies in 2011–2012 and 2018–2019 to observe changes in influenza vaccination coverage. @*Methods@#Women who delivered at Inha University Hospital (Incheon, Korea) in 2011–2012 and 2018–2019 were enrolled in the study. We surveyed the rate of influenza vaccination, perception scores, and related factors through telephonic interviews and compared the results from 2011–2012 and 2018–2019 with those from 2006–2007. @*Results@#The number of survey respondents was 227 in 2006–2007, 152 in 2011–2012, and 171 in 2018–2019. The rate of vaccination coverage increased from 4.0% in 2006–2007 to 42.0% in 2011–2012 and 59.3% in 2018–2019. Perception scores also increased progressively from 3.8 in 2006–2007 to 4.2 in 2011–2012 and 5.1 in 2018–2019. Physician recommendations for influenza vaccination also increased from 4.8% in 2006–2007 to 36.8% in 2011–2012 and 49.7% in 2018–2019. The most common reason for not getting vaccinated was the lack of awareness of influenza vaccination during pregnancy (36.9%). The perception scores and physician recommendation rates were significantly lower for unvaccinated women (3.87 and 8.4%, respectively) than for vaccinated women (5.14 and 69.1%, respectively). @*Conclusion@#The influenza vaccination coverage rate in pregnant women has increased significantly since our study in 2006–2007. However, further improvement in the coverage rate is needed. There is a need for active and comprehensive publicity and education regarding this issue among physicians and pregnant women.

Erythematous Patch in Tsutsugamushi Disease – An Atypical Form of Eschar

Tsutsugamushi disease is caused by the bacterium, Orientia tsutsugamushi and transmitted by chigger mites. In addition to the typical dark eschar, various forms of the eschar, including papules and vesicles, develop at chigger bite sites. Macular lesions were reported only in a human inoculation study; the inoculation lesions relapsed as erythematous macules or erythema-based papules concomitant with fever relapse. Herein, we report an erythematous patch as an inoculation lesion in two patients with tsutsugamushi disease, which, additionally, displayed a central small circle of 1 mm in diameter, possibly a chigger bite site, and desquamation around the circle during doxycycline therapy.

Molecular Epidemiology of Ciprofloxacin-Resistant Escherichia coli Isolated from Community-Acquired Urinary Tract Infections in Korea

Background@#Escherichia coli is the predominant causative pathogen for community-acquired urinary tract infections (UTIs), and the increase in fluoroquinolone-resistant E. coli is of great concern in Korea. The objectives of this study were to investigate the genotypic characteristics and molecular epidemiology of ciprofloxacin-resistant (CIP-R) E. coli isolated from community-acquired UTIs in Korea. @*Materials and Methods@#E. coli samples isolated from the blood or urine were collected from patients with community-acquired acute pyelonephritis aged 15 years and more who were admitted to 12 Korean hospitals from 1st April 2010 to 29th February 2012. Phylogenetic typing, multilocus sequence typing, and molecular characterization of β-lactamase and plasmidmediated quinolone resistance determinants were performed for CIP-R E. coli isolates. @*Results@#A total of 569 E. coli isolates were collected, and 122 (21.4%) isolates were CIP-R isolates. The most prevalent sequence type (ST) was ST131 (28.7%, 35/122), followed by ST393 (14.7%, 18/122), ST1193 (13.1%, 16/122), ST38 (9.0%, 11/122), and ST405 (8.2%, 10/122). The antimicrobial resistance rates of ST131 to cefepime (22.9%, 8/35), ST38 to gentamicin (100%, 11/11), and ST405 to cefotaxime (66.7%, 6/9) were significantly higher than the resistance rates of all other STs combined. Notably, 40% (4/10) of ST405 clones produced extendedspectrum β-lactamases and were co-resistant to trimethoprim/sulfamethoxazole. aac(6′)-1b-cr (20%, 7/35) and CTX-M-14 (40%, 4/10) were more frequently observed in ST131 and ST405 compared with other clones, respectively. @*Conclusions@#Among the CIP-R uropathogenic E. coli isolates in this study, ST131, ST38, and ST405 were specifically associated with antimicrobial resistance.

Subcapsular Splenic Hemorrhage in Vivax Malaria

In malaria, splenic rupture is a serious complication potentially leading to death. Subcapsular hemorrhage of spleen is thought to be an impending sign of splenic rupture; however, the characteristics of subcapsular hemorrhage are not well known. We report 3 cases of subcapsular hemorrhage of the spleen in vivax malaria, with varying degrees of severity. Case 1 showed subcapsular hemorrhage without splenic rupture, was treated by antimalarial drug without any procedure. The healing process of the patient's spleen was monitored through 6 computed tomography follow-up examinations, over 118 days. Case 2 presented subcapsular hemorrhage with splenic rupture, treated only with an antimalarial drug. Case 3 showed subcapsular hemorrhage with splenic rupture and hypotension, treated using splenic artery embolization. They all recovered from subcapsular hemorrhage without any other complications. These 3 cases reveal the process of subcapsular hemorrhage leading to rupture and a potentially fatal outcome. The treatment plan of subcapsular hemorrhage should be determined carefully considering the vital signs, changes in hemoglobin, and bleeding tendency.

History of Tsutsugamushi Disease in Korea

Tsutsugamushi disease or scrub typhus in Korea was first officially reported in foreign soldiers in 1951 and in indigenous persons in 1986. However, the history is further prolonged. The book Dong Ui Bo Gam, published in 1613, described “Soo Dok” (water poisoning), which is similar to tsutsugamushi disease. Further, the term was mentioned in the book Hyang Yak Gu Geup Bang, first published in 1232–1251. During the Japanese colonial rule (1910-1945), Trombicula akamushi was identified in Suwon, Korea, in 1917. Although cases of tsutsugamushi disease in Korea with a murine typhus-like illness and OXK-positivity were reported in 1935, such atypical presentation was not recognized as tsutsugamushi disease. During the Korean War, in 1951, tsutsugamushi disease developed in two British soldiers stationed in the Imjin River, who presented typical features and positive OXK reactions. Indigenous cases have re-emerged since 1986. Thereafter, there were many studies on various aspects of tsutsugamushi disease: epidemiology, vector, small mammals, clinical features and complications, diagnosis, and treatment. Persistence of Orientia tsutsugamushi and its possible recrudescence with pneumonia were reported in 2012 and 2014, respectively.

The Eschar Size and Early Inoculation Lesion of Tsutsugamushi Disease on Jeju Island, Korea

BACKGROUND@#Tsutsugamushi disease, or scrub typhus, is an acute febrile illness caused by Orientia tsutsugamushi, which is followed by chronic latent infection. People who reside in areas endemic of tsutsugamushi disease may be frequently reinfected with this organism. Volunteers who are experimentally reinfected with O. tsutsugamushi manifest various systemic and local reactions, including the presence of small-sized eschar. The present study recorded the morphology and size of eschars in patients with tsutsugamushi disease on Jeju Island, Korea.@*MATERIALS AND METHODS@#From March 2018 to February 2019, 23 patients manifesting clinical characteristics and epidemiologic features of tsutsugamushi disease on Jeju Island were investigated. For comparison of eschar sizes between the two regions, 12 patients with tsutsugamushi disease in Incheon were similarly examined.@*RESULTS@#Three patients, two on the first day and one on the fourth day of fever, presented with papules of 2 – 5 mm in diameter. Another three patients, one on the second day and two on the fourth day, presented with ruptured vesicles of 5 – 8 mm in diameter. Thirteen patients presented with eschars covered with dark scabs, with a median diameter of 5 (95% confidence interval [CI], 5 – 7.5) × 4 (95% CI, 3 – 5) mm. The medians of the eschar sizes did not differ between the two cities (P = 0.46 by Mann-Whitney U test), but eschars ≥10 mm in diameter were more frequent in Incheon than in Seogwipo-si (4 of 12 vs. 0 of 13 patients, P = 0.04 by Fisher's exact test). One patient presented with multiple eschars, and no eschar was detected in the remaining three patients. Among 11 Jeju Island patients with positive IgG and IgM antibodies, seven patients revealed higher IgG than IgM antibody titers during the acute phase of the illness, i.e., the IgG antibody response, two patients had equal IgG and IgM titers, and two patients presented the IgM antibody response. Life-threatening complications and death were not observed in this study.@*CONCLUSION@#The patients in Seogwipo-si had small-sized eschars and occasionally exhibited non-necrotic lesions. Many patients had serologically reinfected tsutsugamushi disease. Further studies are needed to investigate the association between these findings.

Usefulness of Blood Cultures and Radiologic Imaging Studies in the Management of Patients with Community-Acquired Acute Pyelonephritis

BACKGROUND: The objective of this study was to examine the usefulness of blood cultures and radiologic imaging studies for developing therapeutic strategies in community-acquired acute pyelonephritis (CA-APN) patients. MATERIALS AND METHODS: We prospectively collected the clinical data of CA-APN patients who visited 11 hospitals from March 2010 to February 2011. RESULTS: Positive urine and blood cultures were obtained in 69.3% (568/820) and 42.7% (277/648), respectively, of a total of 827 CA-APN patients. Blood culture identified the urinary pathogen in 60 of 645 (9.3%) patients for whom both urine and blood cultures were performed; the organisms isolated from urine were inconsistent with those from blood in 11 and only blood cultures were positive in 49 patients. Final clinical failure was more common in the bacteremic patients than the non-bacteremic ones (8.0% vs. 2.7%, P = 0.003), as was hospital mortality (3.6% vs. 0.3%, P = 0.003). Likewise, durations of hospitalization and fever were significantly longer. Bacteremia was independent risk factor for mortality (OR 9.290, 1.145-75.392, P = 0.037). With regard to radiologic studies, the detection rate of APN was 84.4% (445/527) by abdominal computed tomography and 40% (72/180) by abdominal ultrasonography. Eighty-one of 683 patients (11.9%) were found to have renal abscess, perinephric abscess, urolithiasis, hydronephorosis/hydroureter or emphysematous cystitis, which could potentially impact on clinical management. Patients with Pitt score ≥ 1, flank pain or azotemia were significantly more likely to have such structural abnormalities. CONCLUSION: Blood cultures are clinically useful for diagnosis of CA-APN, and bacteremia is predictive factor for hospital mortality. Early radiologic imaging studies should be considered for CA-APN patients with Pitt scores ≥1, flank pain or azotemia.

In Vitro Activity of Tigecycline Against Orientia tsutsugamushi

Scrub typhus is a zoonosis caused by Orientia tsutsugamushi (O. tsutsugamushi) occurring mainly in autumn in Korea. The need of new antibiotics has arisen with a report on strains resistant to antibiotics and chronic infection. This study aims to identify susceptibility of tigecycline in-vitro as a new therapeutic option for O. tsutsugamushi. Antibacterial activity of tigecycline against the O. tsutsugamushi was compared with doxycycline using flow cytometry assay. The inhibitory concentration 50 (IC50) was 3.59×10(-3) µg/mL in doxycycline-treated group. Whereas in 0.71×10(-3) µg/mL tigecycline-treated group. These findings indicate that tigecycline may be a therapeutic option for the treatment of scrub typhus.

Therapeutic Drug Concentrations of Teicoplanin in Clinical Settings

BACKGROUND: Teicoplanin is a glycopeptide antibiotic that is widely used in clinical practice for the treatment of infections caused by drug-resistant Gram-positive bacteria. The aim of this study was to analyze plasma teicoplanin concentrations to determine the percentage of patients in whom therapeutic concentrations of teicoplanin were achieved in clinical practice. MATERIALS AND METHODS: The plasma teicoplanin concentrations of hospitalized patients receiving treatment at a teaching hospital were retrospectively analyzed. The target level was defined as a plasma teicoplanin concentration of 10 mg/L or greater, since this was generally regarded as the lower limit of the optimal concentration range required for the effective treatment of a majority of infections. RESULTS: Patients with sub-optimal (< 10 mg/L) plasma teicoplanin concentrations constituted nearly half of the total study population. The majority of these patients received the recommended loading dose, which were three 400 mg doses administered every 12 hours. Sub-group analysis showed a trend that the group receiving loading dose was more likely to reach the optimal teicoplanin concentration. CONCLUSIONS: The data revealed that a significant proportion of patients in clinical practice achieved only sub-optimal teicoplanin concentrations, which emphasizes the importance of the mandatory use of loading dose and routine therapeutic drug monitoring. Treatment reassessment and simulation of individual dose regimens may also be necessary to achieve optimal drug concentrations.

Susceptibility of Escherichia coli from Community-Acquired Urinary Tract Infection to Fosfomycin, Nitrofurantoin, and Temocillin in Korea

With increase of multi-drug resistant Escherichia coli in community-acquired urinary tract infections (CA-UTI), other treatment option with a therapeutic efficacy and a low antibiotic selective pressure is necessary. In this study, we evaluated in vitro susceptibility of E. coli isolates from CA-UTI to fosfomycin (FM), nitrofurantoin (NI), temocillin (TMO) as well as trimethoprim-sulfamethoxazole (SMX), ciprofloxacin (CIP) and cefepime (FEP). The minimal inhibitory concentrations were determined by E-test or agar dilution method according to the Clinical and Laboratory Standards Institute guidelines, using 346 E. coli collected in 12 Korean hospitals from March 2010 to February 2011. FM, NI and TMO showed an excellent susceptibility profile; FM 100% (346/346), TMO 96.8% (335/346), and NI 99.4% (344/346). Conversely, resistance rates of CIP and SMX were 22% (76/346) and 29.2% (101/349), respectively. FEP still retained an activity of 98.5%. In Korea, NI and TMO in addition to FM are a good therapeutic option for uncomplicated CA-UTI, especially for lower UTI.

Susceptibility of Escherichia coli from Community-Acquired Urinary Tract Infection to Fosfomycin, Nitrofurantoin, and Temocillin in Korea

With increase of multi-drug resistant Escherichia coli in community-acquired urinary tract infections (CA-UTI), other treatment option with a therapeutic efficacy and a low antibiotic selective pressure is necessary. In this study, we evaluated in vitro susceptibility of E. coli isolates from CA-UTI to fosfomycin (FM), nitrofurantoin (NI), temocillin (TMO) as well as trimethoprim-sulfamethoxazole (SMX), ciprofloxacin (CIP) and cefepime (FEP). The minimal inhibitory concentrations were determined by E-test or agar dilution method according to the Clinical and Laboratory Standards Institute guidelines, using 346 E. coli collected in 12 Korean hospitals from March 2010 to February 2011. FM, NI and TMO showed an excellent susceptibility profile; FM 100% (346/346), TMO 96.8% (335/346), and NI 99.4% (344/346). Conversely, resistance rates of CIP and SMX were 22% (76/346) and 29.2% (101/349), respectively. FEP still retained an activity of 98.5%. In Korea, NI and TMO in addition to FM are a good therapeutic option for uncomplicated CA-UTI, especially for lower UTI.

In vitro Synergism between Chloroquine and Antibiotics against Orientia tsutsugamushi

BACKGROUND: To investigate whether chloroquine enhances the effect of antibiotics against Orientia tsutsugamushi, the causative organism of scrub typhus, we compared the effect of antibiotics in combination with chloroquine with the effect of antibiotics alone in vitro. MATERIALS AND METHODS: The Boryong or AFSC-4 strain was inoculated into ECV304 cells, and incubated in medium containing doxycycline (4 microg/mL), rifampin (4 microg/mL), azithromycin (0.5 microg/mL), chloroquine (1 microg/mL), and each of these antibiotics in combination with chloroquine for 7 d. Immunofluorescence (IF) staining for O. tsutsugamushi was performed 4 hr and 7 d after inoculation of the bacteria, and IF-positive foci were enumerated. RESULTS: Chloroquine inhibited the growth of O. tsutsugamushi by 15.5%. In combination with chloroquine, the antimicrobial effects increased by 4.4% for doxycycline (a 92.9% reduction of bacterial numbers for doxycycline versus a 97.3% reduction for doxycycline plus chloroquine), 4.6% for rifampin (90.0% versus 94.6%), and 8.3% for azithromycin (86.9% versus 95.2%). The antimicrobial effect of the antibiotics alone was significantly different compared to the combined effect of antibiotics and chloroquine (Wilcoxon signed-rank test, P = 0.001). CONCLUSIONS: The combined use of chloroquine with an antibiotic for the treatment of O. tsutsugamushi infections may be useful for increasing the efficacy of the antibiotics.

In vitro Antagonism between Cefotaxime and Anti-Rickettsial Antibiotics against Orientia tsutsugamushi

We have investigated the in vitro antimicrobial effects of antibiotic combinations against Orientia tsutsugamushi, the causative agent of scrub typhus. ECV304 cells were infected with the Boryong strain of O. tsutsugamushi and incubated in a medium containing doxycycline (4 microg/mL), azithromycin (0.5 microg/mL), rifampin (4 microg/mL), ciprofloxacin (25 microg/mL), gentamicin (5 microg/mL), cefotaxime (2 microg/mL), or combinations of these agents for 7 days, after which immunofluorescent staining for O. tsutsugamushi was performed. The percentages of infective foci in cultures containing antibiotics compared to those in cultures without antibiotics were 6.2% for doxycycline, 9.6% for azithromycin, 8.8% for rifampin, 96.6% for cefotaxime, 29.7% for doxycycline plus cefotaxime, 23.6% for azithromycin plus cefotaxime, and 41.4% for rifampin plus cefotaxime. These findings show an in vitro antagonism between anti-rickettsial agents and cefotaxime against O. tsutsugamushi. These results suggest that the efficacy of antibiotic combinations involving cefotaxime for the treatment of patients with scrub typhus, particularly those with severe pneumonia, needs to be investigated.

In Vitro Bacteriostatic Effects of Rifampin on Orientia tsutsugamushi

We performed an in vitro cell culture experiment to ascertain whether rifampin exhibits bactericidal effects against Orientia tsutsugamushi, the causative agent of scrub typhus. ECV304 cells were infected with the Boryong or AFSC-4 strain of O. tsutsugamushi and then, the cultures were maintained in media with increasing concentrations of rifampin, azithromycin, doxycycline, or chloramphenicol for 4 days. On day 5, the media were replaced with fresh antibiotic-free medium and the cultures were maintained until day 28. On days 5, 13, and 28, immunofluorescence (IF) staining of O. tsutsugamushi was performed. IF staining on days 13 and 28 revealed increasing numbers of IF-positive foci in all cultures, even in cultures initially exposed to the highest concentration of rifampin (80 microg/mL), azithromycin (80 microg/mL), doxycycline (20 microg/mL), or chloramphenicol (100 microg/mL). The present study reveals that rifampin has no bactericidal effect against O. tsutsugamushi as observed for azithromycin, doxycycline, and chloramphenicol. A subpopulation of the bacteria that are not killed by high concentrations of the antibiotics may explain the persistence of O. tsutsugamushi in humans even after complete recovery from scrub typhus with antibiotic therapy.

Detection of an Infected N-butyl-2-cyanoacrylate Plug by F-18 FDG PET/CT Scan in a Patient Who Received Endoscopic Intervention for Gastric Variceal Bleeding

Injection of N-butyl-2-cyanoacrylate has been used successfully for treatment of gastric variceal bleeding. Bacteremia after injection of N-butyl-2-cyanoacrylate is well known, however, the method for diagnosis of infected endovascular injected material has remained uncertain. This is the first case reporting use of F-18 FDG PET/CT in detection of the source of infection after control of endoscopic bleeding with N-butyl-2-cyanoacrylate.

A Case of Vivax Malaria Complicated by Adult Respiratory Distress Syndrome and Successful Management with Extracorporeal Membrane Oxygenation

Complicated malaria is mainly caused by Plasmodium falciparum, but, increasingly, Plasmodium vivax is also being reported as a cause. Since the reemergence of indigenous vivax malaria in 1993, cases of severe malaria have been steadily reported in Korea. Herein, we report a case of vivax malaria complicated by adult respiratory distress syndrome (ARDS) that was successfully managed with extracorporeal membrane oxygenation (ECMO). A 59-year-old man presented at our hospital with fever and abdominal pain, which had persisted for 10 days. On admission, the patient had impaired consciousness, shock, hypoxia and haziness in both lungs, jaundice, thrombocytopenia and disseminated intravascular coagulation, metabolic acidosis, and acute kidney injury. A peripheral blood smear and a rapid diagnostic test verified P. vivax mono-infection. Ten hours after admission, hypoxia became more severe, despite providing maximal ventilatory support. The administration of antimalarial agents, ECMO, and continuous venovenous hemofiltration resulted in an improvement of his vital signs and laboratory findings. He was discharged from the hospital 7 weeks later, without any sequelae.

Erratum: Correction of Title

This erratum is being published to correct of title.

Extensive Thrombosis in a Patient with Familial Mediterranean Fever, Despite Hyperimmunoglobulin D State in Serum: First Adult Case in Korea

Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by recurrent episodes of fever accompanied by peritonitis, pleuritis, arthritis, or erysipelas-like erythema. It is known to occur mainly among Mediterranean and Middle Eastern populations such as non-Ashkenazi Jews, Arabs, Turks, and Armenians. FMF is not familiar to clinicians beyond this area and diagnosing FMF can be challenging. We report a 22-yr old boy who presented with fever, arthalgia and abdominal pain. He had a history of recurrent episodes of fever associated with arthalgia which would subside spontaneously or by antipyretics. Autosomal recessive periodic fever syndromes were suspected. Immunoglobulin D (IgD) level in the serum was elevated and DNA analysis showed complex mutations (p.Glu148Gln, p.Pro369Ser, p.Arg408Gln) in the MEFV gene. 3D angio computed tomography showed total thrombosis of splenic vein with partial thrombosis of proximal superior mesenteric vein, main portal vein and intrahepatic both portal vein. This is a case of FMF associated with multiple venous thrombosis and elevated IgD level. When thrombosis is associated with elevated IgD, FMF should be suspected. This is the first adult case reported in Korea.