helicobacter cinaedi has come to be recognized as an important organism , especially among immunocompromised patients .
h. cinaedi sometimes causes cellulitis but the main symptom is only fever without any focal findings in most cases . furthermore , unlike most other infectious diseases , the clinical course is not acute . for these reasons ,
when an sle patient becomes febrile , we should determine whether there has been an exacerbation of sle itself or whether the condition is a complication of an infectious disease .
this is vital because opposite strategies are used in the treatment of sle and infectious diseases . in the present case ,
h. cinaedi bacteremia occurred in a patient with sle , who initially only showed fever .
these symptoms mimicked a flare of the disease , which led us initiate the bolus administration of methylprednisolone .
her symptoms included a typical rash , severe multiple organ failure , consciousness disturbance and retinal vasculitis .
a laboratory analysis revealed reduced c3 , c4 and ch50 levels ( 20 mg / dl , 7 mg / dl and 10 u / ml , respectively ) and anti - nuclear antibody positivity ( 640 , homogenous , speckled ) , and anti - dna antibody negativity .
she was in a severe condition and was refractory to the initial treatment of prednisolone ( 1 mg / kg / day ) .
plasmapheresis , high - dose methylprednisolone ( 1 g bolus for 3 days ) and an increased dose of prednisolone ( 1.3 mg / kg / day ) were necessary to achieve remission from sle .
after the initial therapy , her condition remained stable with the administration of prednisolone ( 10 mg / day ) for more than 10 years . a regular blood examination showed a gradual increase in the patient 's c - reactive protein ( crp ) level from 3 months before her admission , which had previously been normal . at first , an exacerbation of sle was suspected and the amount of prednisolone was increased to 15 mg / day .
one month before the admission , she suffered from a high grade fever ( 39 ) at night with shivering , chills and mild nausea .
a complete blood cell count revealed anemia ( hb , 7.3 g / dl ) with an increase in the number of fragmented red blood cells , leukocytosis ( wbc , 18,880 /l ) with neutrophilia ( 93.5% ) , and severe thrombocytopenia ( platelet count , 37,000 /l ) .
tests for anti - nuclear antibodies and anti - dna antibodies were negative and the patient 's complement values were normal ( c3 , 87 mg / dl , c4 , 24 mg / dl , and ch50 , 51 u / ml ) .
an interferon- release assay tb was negative and no vegetation was observed by transthoracic echocardiography .
1 ) and the values of alkaline phosphatase and -glutamyltranspeptidase were also elevated ; however , she had no abdominal pain .
the patient 's blood cultures being negative , and the focus of infection had not been detected on the fourth day of admission .
we were therefore forced to start methylprednisolone ( 1,000 mg / day ) due to suspected acalculous cholecystitis which was thought to have occurred due to a flare of sle or a complication of vasculitis .
the next day , the patient 's blood cultures were found to be positive ( after 5 days of incubation ) and microscopy showed gram - negative spiral bacteria ( fig .
the isolate was finally identified as h. cinaedi by a pcr and 16s rrna gene sequencing .
we considered that the patient 's thrombocytopenia had been caused by h. cinaedi bacteremia , and stopped the bolus administration of methylprednisolone ; prednisolone was reduced to the maintenance dose . with the administration of meropenem ,
the patient 's temperature decreased and there was an improvement in her laboratory data with regard to her platelet count , crp level , and the number of fragmented red blood cells .
the antibiotic was changed and de - escalated to ampicillin , which caused a systemic drug eruption .
doripenem was administered for 3 weeks ; thus , the total duration of treatment reached 5 weeks , at which point , the antibiotics were stopped due to pancytopenia .
one month after the treatment , the h. cinaedi bacteremia relapsed with a high - grade fever and cellulitis ( fig .
meropenem was administered intravenously for another 2 weeks , and 6 weeks of oral minocycline were given , no relapse occurred after this treatment .
abdominal ct on admission showing the thickness of the gallbladder wall ( white arrowheads ) .
psl : prednisolone , mpsl : methylprednisolone , mepm : meropenem , abpc : ampicillin , ipm / cs : imipenem / cilastatin , azm : azithromycin , drpm : doripenem , wbc : white blood cell ( /l ) , hb : hemoglobin ( g / dl ) , plt : platelet ( 10/l ) , ast : aspartate aminotransferase ( u / l ) , alt : alanine aminotransferase ( u / l ) , alp : alkaline phosphatase ( u / l ) , crp : c - reactive protein ( mg / dl ) , closed triangle : positive blood culture , open triangle : negative blood culture cellulitis complicated with secondary bacteremia was seen on both extremities and the patient s back .
h. cinaedi is a gram - negative spiral bacterium that belongs to the helicobacteriaceae family .
it is hypothesized that h. cinaedi infection occurs via the oral route . in some reports , the possibility of zoonotic transmission from animal feces to humans has been discussed . however , the patient had no pets and no contact with animals .
the median time for h. cinaedi to become positive in blood cultures is 5 days ( 2 - 12 days ) , and the positive ratios from 5 to 7 days were only 55% and 87% , respectively ( 1 ) . in order to successfully detect h. cinaedi bacteremia , at least 7 days of incubation
should be necessary , although only 1 - 3 days are sufficient for most other organisms .
the other characteristics of h. cinaedi bacteremia are nonspecific symptoms and a tendency of relapse .
it has been hypothesized that h. cinaedi infection is associated with cellulitis , arthritis and gastroenteritis ( 2 - 4 ) ; however , fever is the only symptom in many cases .
actually , in ten cases of h. cinaedi bacteremia that were observed in our hospital in the eight years from 2007 to 2014 , the only symptom was fever in five cases .
the four cases of ten showed skin lesions ; however , these were very mild in all of the cases
the appropriate antimicrobial agents and the duration of the treatment for h. cinaedi infection have not been established .
although we treated the patient for a sufficient length of time ( 5 weeks ) , she nevertheless relapsed .
cinaedi bacteremia cases are usually reported in immunocompromised patients such as those with hiv infection , hematological disorders , malignancies , alcoholism and receiving immunosuppressive therapy ( 5,6 ) .
some other infectious diseases have been reported to mimic sle or a flare of sle , human parvovirus b19 , cytomegalovirus , visceral leishmaniasis , which is only in endemic area , and so on ( 7,8 ) .
some bacterial infections may mimic a flare of sle , and h. cinaedi might be one of them . however , as far as we are aware , reported cases on h. cinaedi bacteremia with sle are very rare ( 9 ) .
we finally diagnosed gallbladder thickening and thrombocytopenia , which were associated with h. cinaedi infection itself , not with a flare of sle .
although the association with hepatobiliary diseases and enterohepatic helicobacter species have been mentioned , most cases were caused by h. bilis or h. hepaticus ( 10 ) , and not h. cinaedi or cinaedi , there have been only a few reports on hepatobiliary diseases in animals ( 11 ) and only one report on hepatobiliary diseases in humans .
our case might represent a possible presentation of the hepatobiliary infections caused by h. cinaedi in humans . in conclusion
, we should be mindful of the presence of h. cinaedi bacteremia , especially when we see febrile immunocompromised patients such as patients with sle .
cinaedi takes longer to diagnose , the most popular symptom is only fever and it is sometimes associated with various symptoms .
once an h. cinaedi infection is diagnosed , it is necessary to treat the patient with antibiotics for more than one month due to the tendency to relapse .
 a 40-year - old woman with systemic lupus erythematosus ( sle ) presented with high - grade fever and severe thrombocytopenia . 
 acalculous cholecystitis and thrombocytopenia were initially suspected to be complicated with sle and vasculitis . 
 contrary to our expectation , however , the patient was finally diagnosed with helicobacter cinaedi bacteremia . 
 sle patients show various symptoms , especially when their condition is complicated with vasculitis , which mimics h. cinaedi bacteremia . 
 it is therefore difficult to provide a definite diagnosis . 
 physicians should be mindful of the presence of h. cinaedi infection . 
Introduction
Case Report
Discussion
The authors state that they have no Conflict of Interest (COI).