leishmaniasis is caused by leishmania species and has visceral , cutaneous , and mucocutaneous forms in humans . visceral leishmaniasis ( vl ) , also known as kala - azar , a disseminated and potentially fatal form , is endemic in areas of the tropics , subtropics , and southern europe . classic clinical features of vl include high fever , anorexia , hepatomegaly , marked splenomegaly , and pancytopenia due to severe involvement of reticuloendothelial system ( res ) organs , particularly the liver , spleen , and bone marrow . laboratory tests usually show elevated inflammatory markers , marked eosinopenia , a relative lympho - monocytosis , hypergammaglobulinemia with hypoalbuminemia , and occasionally evidence of liver damage with raised liver enzymes . definite diagnosis requires demonstration of the parasite either histologically in relevant tissues or with leishmania - specific immunologic tests . however , diagnosis could be challenging in sporadic cases and often delayed , as the main clinical presentation is often identical to other infectious and non - infectious diseases . furthermore , some of vl manifestations are associated with immune responses of the host to leishmania that mimic autoimmune diseases like serum sickness . autoimmune phenomena are common in leishmaniasis which could be related to polyclonal b - cell activation , molecular mimicry between microbial and host antigens , and altered - reduced regulatory and suppressor t cell functions . as a consequence of these disturbances , several autoantibodies appear in the sera of patients with vl , albeit , in most cases accompanying clinical manifestations are lacking . on the other hand , there are several reports on concurrent or masquerading presentations with autoimmune diseases , particularly systemic lupus erythematosus ( sle ) , rheumatoid arthritis , and cryoglobulinemia in the literature [ 6 - 8 ] . autoimmune hepatitis ( aih ) is also reported . however , vl resembling aih and primary biliary cirrhosis ( pbc ) overlap hepatitis has not been described before . herein , we report a case of vl with clinical and laboratory features mimicking aih , pbc overlap hepatitis , and sle . a 26-year - old female patient referred to the rheumatology department with complaints of anorexia , malaise , weight loss , joint swelling , and low grade fever . her past history was notable for photosensitivity , oral ulcers , and possible thalassemia trait . on admission , her physical examination was significant for erythema and symmetrical arthritis at ankles , wrists , and hand joints . the spleen was palpated 10 cm below the costal margin , and the liver had a longitudinal size of 15 cm . erythrocyst sedimentation rate ( esr ) and c- reactive protein was 65 and 19.9 mg / l , respectively . complete blood count revealed hemoglobin 10.2 gr / dl , mean corpuscular volume 69 fl , leukocyte 4,000/mm , and platelet 157,000/mm . other pertinent laboratory data was as follows : creatinine 0.7 mg / dl ( normal=0.5 - 1.2 ) , alanine aminotransferase 46 u / l ( alt , normal<40 ) , aspartate aminotransferase 51 u / l ( ast , normal<40 ) , alkaline phosphatase 67 u / l ( alp , n=53 - 141 ) , gama - glutamyl transferase 17 u / l ( ggt , n=0 - 50 ) , albumin 3.2 g / dl , globulin 6.9 g / dl , and lactate dehydrogenase 236 u / l ( n=125 - 243 ) . her laboratory investigation was positive for anti - nuclear antibody immunofluorescence ( ana - ifa ) , anti - smooth muscle antibody ( asma ) , and coombs tests on previous referral center . repeated ana was strongly positive as well as antimitochondrial ( ama - m2 ) antibodies and coombs tests . liver kidney microsomal ( anti - lkm ) , anti - cytosolic liver ( lc-1 ) , antisoluble liver / liver - pancreas ( sla / lp ) , asma , anti - ena , ds - dna tests were negative . igg was 5,810 mg / dl ( n=751 - 1,560 ) with a polyclonal pattern . histological examination of the liver biopsy revealed robust plasma cell and lymphocyte infiltrations in the sinusoidal areas , and periductal and portal small granuloma formation . histochemically , plasma cells , which were stained for methyl grn pyronine ( mgp ) , were found to act as a dominant feature among the inflammatory infiltrates in the liver . this finding also supported and raised the suspicion of autoimmune hepatitis - pbc overlap ( fig . prednisone was started at a dose of 1 mg / kg under a presumptive diagnosis of sle / aih with a rapid clinical improvement on arthritis , fever , malaise and general condition , liver function tests , acute phase response , and hypergammaglobulinemia . a few weeks after the immunosuppression , her leukocyte and platelet counts began to fall gradually , reaching 1,000/mm and 14,000/mm , respectively . the patient was transferred to the infectious diseases department with the diagnosis of neutropenic fever . after 4 days , teicoplanin and fluconazole were added to the treatment because of urinary infection caused by enterococci and candida albicans . although , against this appropriate treatment , her condition got worse , fever continued , and neutropenia and thrombocytopenia persisted . a bone marrow aspiration and biopsy were performed to rule out possible bone marrow pathology . the amastigote tissue forms were seen when her liver biopsy was re - evaluated ( fig . 3 ) . leishmania igg was found positive at a 1/1,280 titer in her serum specimen by indirect immunofluorescent assay . high dose ( 4 mg / kg / day ) intravenous liposomal amphotericin b was given on days 1 to 5 and 10 , 17 , 24 , 31 , and 38 days . then , her fever resolved , and general conditions greatly improved with the exception of neutropenia . after a dose of granulocyte colony - stimulating factor , her neutrophil count also reached to a normal level . no other complications were observed in the duration of the treatment . on her follow - up examination at 6 months after amphotericin therapy , she was in good clinical condition with free of any complaints and had normal blood counts , biochemical analyzes , and normal gammaglobulin levels . asma become negative , and ana and ama - m3 tests remained weak and strong positives , respectively . leishmaniasis is a parasitic disease , which is transmitted to humans by sandflies in endemic areas . vl is considered endemic in tropical and subtropical regions ( bangladesh , india , nepal , sudan , and brazil ) in the world . however , in other countries , it is detected in a few cases . in turkey , vl is seen mainly in the aegean , mediterranean , and central anatolia regions , and leishmania infantum is the most responsible agent for the human diasese . children were at a higher risk of infection , and only a few adult cases of vl were reported from turkey [ 9,11 - 13 ] . vl is a disseminated intracellular infection with a fatality rate of as high as 90% , unless treated . laboratory tests frequently show pancytopenia , marked eosinopenia , a relative lympho - monocytosis and hypergammaglobulinemia with hypoalbuminemia , and occasionally evidence of liver damages with raised liver enzymes . the diagnosis is based on a combination of demographic , clinical , laboratory findings , and confirmation of the disease via demonstration of amastigote tissue forms of parasites or leishmania - specific immunological tests . vl remains asymptomatic or subclinical in many cases , or can follow an acute , subacute , or chronic course . chronic vl results in delays in diagnosis due to subtle or atypical presentations . . this might be due to a release of high amounts of self antigens as a result of tissue destruction caused by the parasite . vl causes polyclonal b cell activation independent of t cells through mitogen activation or stimulation of cytokines by other immune cells . finally , as demonstrated previously , there are some molecular mimicry between leishmania antigens and host antigens such as ribosomal phosphoproteins expressed on parasites , causing cross - reactivity . reported autoimmune diseases that were mimicked or exacerbated by leishmaniasis are sle , aih , rheumatoid arthritis , polyarteritis nodosa , cryoglobulinemic vasculitis , autoimmune hemolytic anemia , and ewan 's syndrome [ 6 - 9,11,12 ] . all of them , particularly sle and aih have clinical and laboratory features undistinguishable from vl that makes confusion . moreover , many autoantibodies are formed in vl , even disease specific ones , including rf , anticitrulline , ana , ds - dna , asma , anti - ro , anti - la , anti - sm , anti - cardiolipin , and anti - platelet antibodies . positivity of one or more of these tests with vague clinical presentations may be misinterpreted as autoimmune disorders . a correct diagnosis of vl was tricky in our patient because of presentation which was very similar to the autoimmune liver disease and sle . though the liver enzymes are frequently elevated in leishmaniasis , positive autoimmune laboratory tests and liver biopsy reported as consistent with aih - pbc overlap led us to consider autoimmune overlap hepatitis and sle as the cause . hematological manifestations appeared later in the clinical course and that was thought as an azathioprine side - effect . however , discontinuation of this drug and escalating prednisone dose did not improve clinical pictures , and the patient was re - evaluated revealing vl diagnosis . pbs or its serological marker ama - m2 positivity has not been reported in patients with vl , to the best of our knowledge . persistently high titers of ama - m2 even 6 months after the appropriate treatment could be consistent with true diagnosis of pbs in our patient which might be concurrent with vl . leishmaniasis is characterized by multiple immunological abnormalities and protean clinical manifestations that may be misinterpreted as various autoimmune diseases . moreover treatment of autoimmune diseases may worsen the disease and even cause a fatal outcome in vl patients . therefore , in case of atypical presentations or treatment failure in autoimmune diseases , leishmaniasis should be considered and ruled out .
visceral leishmaniasis ( vl ) is a life - threatening infection caused by leishmania species . 
 in addition to typical clinical findings as fever , hepatosplenomegaly , and cachexia , vl is associated with autoimmune phenomena . to date , vl mimicking or exacerbating various autoimmune diseases have been described , including systemic lupus erythematosus ( sle ) , rheumatoid arthritis , and autoimmune hepatitis ( aih ) . 
 herein , we presented a patient with vl who had overlapping clinical features with sle , aih , as well as antimitochondrial antibody ( ama - m2 ) positive primary biliary cirrhosis .