mild symptoms , such as arthritis and skin rash , predominate in frequency , but life - threatening manifestations requiring aggressive immunosuppressive therapy can also develop . thrombocytopenia may precede the other manifestations of sle , but severe thrombocytopenia accompanying spontaneous bleeding is rare . as other diseases can cause thrombocytopenia , thrombocytopenia alone in the absence of other symptoms of sle may cause the physician to delay in starting aggressive therapy . here , we report the case of a 22-year - old woman who suffered from spontaneous hemoperitoneum as the initial manifestation of sle . a 22-year - old woman suffering from vaginal bleeding was referred to our hospital from a local clinic . after admission in the gynecology department the vaginal bleeding had continued for more than 10 days after her last menstruation . the patient 's body temperature , blood pressure , pulse rate , and respiratory rate were 36.9 , 140/80 mm / hg , 88/minute , and 22/minute , respectively . petechiae were found on the entire body , but there was no specific tender point on her abdomen . the complete blood count showed a white blood cell count of 8,960/l ( 85.5% neutrophils , 11.9% lymphocytes , 1.5% monocytes ) , hemoglobin 6.7 g / dl , hematocrit 22.4% , and platelet count 7,000/l . the values of coagulation tests were within normal limits . since the pelvic ultrasound suggested a large amount of fluid , computed tomography ( ct ) of the abdomen and pelvis was performed , and demonstrated a large amount of blood with a 4.5  4 cm ill - defined mass - like lesion in the right adnexa suggesting a ruptured hemorrhagic corpus luteal cyst ( fig . 1 ) . given the severe thrombocytopenia , emergency surgery was delayed and a blood transfusion was started . evaluating the possible causes of thrombocytopenia , the gynecologists consulted us for rheumatologic causes . we considered sle and idiopathic thrombocytopenic purpura ( itp ) as possible causes of thrombocytopenia . additional laboratory findings were as follows : increased antinuclear antibody titer ( over 1:320 , speckled pattern ) ; false - positive venereal disease research laboratory ( vdrl ; fta - abs and tpha tests were negative ) ; decreased complement fractions ( complement 3 : 69 mg / dl [ normal , 90 to 180 ] , complement 4 : 10 mg / dl [ normal , 10 to 40 ] ) ; positive platelet associated immunoglobulin g ( igg ) antibody . the anti - dsdna antibody , anti - smith antibody , antibodies targeting extractable nuclear antigens , anticardiolipin antibody ( igg and igm ) , and lupus anticoagulant were negative . serologic tests for epstein - barr virus , cytomegalovirus , and human immunodeficiency virus ( hiv ) were all negative . findings compatible with sle were clear , but were insufficient to conclude that sle was the cause of the thrombocytopenia ; itp seemed to be a more likely cause . on the 3rd day of admission we performed brain magnetic resonance imaging ( mri ) , which showed multifocal cortical and subcortical hyperintensities in both cerebral and cerebellar hemispheres ( fig . 2 ) . angiography revealed multifocal stenosis and post - stenotic dilatation of the cerebral arteries compatible with vasculitis ( fig . 3 ) . those findings led us to conclude that the patient had sle and she was transferred to our rheumatology department . since the patient 's thrombocytopenia was severe , we administered intravenous ( iv ) immunoglobulin first , and then started intravenous steroid pulse therapy ( 1 g / day ) for 3 consecutive days . after the therapy , we recommended monthly cyclophosphamide ( cyc ) pulse therapy ( 750 mg / m ) because of her life - threatening manifestat - ions : severe thrombocytopenia and cerebral vasculitis . the complement 3 and 4 levels were increased to 97 and 15 mg / dl , respectively . pelvic ultrasound performed after the therapy showed only a scant amount of fluid . as the improvements in her symptoms and laboratory findings were marked , we discharged the patient and recommended she take prednisolone at a dose of 1 mg / kg . thrombocytopenia lower than 100,000/l during the course of sle is found in about 20% of cases , but severe thrombocytopenia causing spontaneous bleeding is rare . in this case , there was severe thrombocytopenia , but other symptoms suggesting sle were not prominent initially . although laboratory findings , including a false - positive vdrl and high antinuclear antibody titer , supported the diagnosis of sle , they were insufficient to declare that the patient had sle . alternatively , the positive platelet - associated igg antibody and bone marrow findings suggested the diagnosis of itp . however , the development of a seizure and the mri and angiographic findings confirmed the diagnosis of sle . approximately 3 to 16% of patients with itp not meeting the american college of rheumatology criteria for sle ultimately evolve into sle , emphasizing the need for further monitoring of patients who seem to have itp . the neuropsychiatric syndromes of sle ( npsle ) include 19 manifestations , including psychosis and seizures , as classification criteria . although severe neurological manifestations of sle mandate immediate therapy , only one randomized controlled trial of the effectiveness of intravenous cyc for npsle has been conducted . at present , even without evidence , cyc seems to be the most effective drug for severe , inflammatory neuropsychiatric lupus . based on studies of the effectiveness of cyc for severe thrombocytopenia and npsle , we treated the patient with iv cyc with high - dose steroid and she improved . many cases with a ruptured corpus luteum and hemoperitoneum are reported in the general population , but only one case of spontaneous ovarian rupture with hemoperitoneum has been reported in an sle patient . we experienced a patient with sle , presenting with spontaneous hemoperitoneum due to severe thrombocytopenia as the first manifestation . in conclusion an effort to discover symptoms , signs , and laboratory findings associated with the disease should be made so appropriate therapy is not delayed .
thrombocytopenia is included in the classification criteria for systemic lupus erythematosus ( sle ) . 
 however , severe thrombocytopenia causing spontaneous bleeding is rare . here , we describe a 22-year - old woman who presented with spontaneous hemoperitoneum as the first manifestation of sle . 
 laboratory findings compatible with sle included positive antinuclear antibody and a false - positive venereal disease research laboratory . 
 symptoms suggesting the disease were not prominent early after admission , but headache and seizures that developed on the 3rd day of admission led to the diagnosis of sle . 
 the brain magnetic resonance imaging and angiography findings were compatible with the neuropsychiatric manifestations of sle . 
 high - dose steroid and monthly intravenous cyclophosphamide pulse therapy were effective at improving the headache and seizure , as well as the hemoperitoneum .