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June 2009KUWAIT MEDICAL JOURNAL 149Case ReportScrub Typhus Associated with Systemic LupusErythematosus: A Case ReportMohsen Nasr 1 , Mohamed Mostafa Abdelwahab Mostafa 1 , Soondal Koomar Surrun 21Department of Internal Medicine, Al-Jahra Hospital, Kuwait2Department of Internal Medicine, Singapore General Hospital, SingaporeABSTRACTKuwait Medical Journal 2009; 41 (2): 149-151The etiology of systemic lupus erythematosus (SLE) islargely unknown and there is a probable contribution ofgenetic, hormonal, immunological and environmentalfactors for its manifestation. The widespreadimmunological destruction of many organs in SLE andthe associated decreased immunity increase the riskof infections. Steroids and other immunosuppressantare important in the treatment of SLE, but they furtherincrease the risk of infections, and sometimes with rareorganisms. We present a case of an adolescent girl withprolonged fever, joint pains and without skin rashes. Theinitial diagnosis was SLE. She was treated with steroidswith improvement in her general condition and relief ofjoint pains. However the fever persisted and subsequentinvestigations revealed an associated scrub typhus. Thefever subsided after treatment with oral tetracycline.There were no complications of scrub typhus. Sincescrub typhus infection is not common in the ArabianPeninsula the disease was not initially thought of. In theinvestigation of prolonged fever in SLE, viral, bacterial,protozoal as well as rickettsial diseases should be bornein mind.KEY WORDS: pyrexia of unknown origin, scrub typhus, systemic lupus erythematosusINTRODUCTIONSystemic lupus erythematosus (SLE) may presentclinically in many subtle ways. Initial presentationas prolonged fever is common. The course of SLE issometimes punctuated by infections, sometimes withrare organisms [1] . Infections remain a major causeof morbidity and mortality in SLE. When a patientwith SLE becomes infected with scrub typhus, theassociated infection may not be entertained initially,especially in non-endemic regions leading to delayin investigations and diagnosis. The typical escharmay be absent, and other skin lesions of scrubtyphus may be mistaken for other febrile illnessesincluding SLE [2] . Scrub typhus infection is endemicin Asia, the Pacific islands including northernAustralia and New Zealand. It is a zoonosis thatis transmitted by mites and before the advent ofantibiotics the disease was occasionally fatal. Aswith SLE, fever is a common presenting symptomin scrub typhus. Additionally, lymphadenopathy,an eschar or skin rashes may also be present.During the Second World War scrub typhus was adreaded disease causing substantial morbidity andoccasional deaths. Nowadays, it occasionally occursoutside the endemic areas and rarely in the ArabianPeninsula.CASE HISTORYA 17-year-old girl was admitted with easy fatigue,recurrent bouts of fever, painful wrists and elbowsand loss of hair of two months’ duration. The painoccurred simultaneously in the affected joints, wascontinuous and partially relieved by paracetamol.She did not notice any loss of weight or dryness ofthe mouth, and there was no morning stiffness. Shewas previously in good health and had no majormedical or surgical problems.On examination she was pale, without jaundiceor cyanosis. There were no skin rashes or nailchanges. There was no discoid alopecia. Hertemperature was 38.8 o C, pulse 80 beats per minuteand blood pressure 110/80 mm Hg. There wasno generalized lymphadenopathy. Both elbowsand wrists were swollen and red, with impairedextension of the elbows. Movement of the wriststhough painful was full. There was no wasting ofthe interossei, thenar or hypothenar eminences.Examination of the eyes was normal. HeartAddress correspondence to:Dr. Mohsen Nasr, PhD, Department of Internal Medicine, Al-Jahra Hosptial, PO Box 3038, Kuwait 01032. E-mail: mohsennasr@hotmail.com
150 Scrub Typhus Associated with Systemic Lupus Erythematosus: A Case Reportsounds were normal and there were no murmurs.The chest examination was normal. There was nohepatosplenomegaly and neurological examinationwas normal.Hematological tests showed hemoglobin of 8.4gm/dl, white blood cell count of 2.2 x 10 9 /ml anda platelet count of 230 x 10 9 /ml. ESR was 118 mm/hr, CRP negative, coagulation profile normal andCoomb’s test negative. Serum iron was 5 µmol/l,bilirubin was normal with an AST of 151 iu/l, ALT of87 iu/l and ALP of 423 iu/l. Urine microscopy wasnormal, the pH 5 and the 24-hour urine protein was0.19 g/day. Complement tests showed a C3 of 78(normal range 75-135) mg/dl and C4 of 16 (normalrange 12-72) mg/dl. Hepatitis profile, monospottest, brucella antigen test and Widal test werenegative. Chest radiograph, the abdomen and pelvicultrasound were normal. The rheumatoid factor(RF) IgM titer was 26 µmol/ml (n < 20), RF IgG 14µmol/ml (n < 20), RF IgA 10 µmol/ml (n < 15), ANA1/320 (speckled pattern), and anti-dsDNA antibody76 IU/L. A clinical diagnosis of SLE was made andprednisolone was started at a dose of 40 mg per day.The patient felt well, the pain in the joints improvedbut the fever persisted. White blood cell count roseto 14.4 x 10 9 /ml, liver enzymes remained high andthe CRP became positive on repeated examination.An associated infective process was then consideredand a diagnosis of pyrexia of unknown origin wasentertained.Repeated chest radiographs and blood cultureswere normal. Further blood investigations werenegative for HIV, toxoplasma, parvovirus andfungus. Gallium scan was negative. However,Weil-Felix reaction was positive for OX-K proteusantigen with a titer of 1/320, rising to 1/640 afterthree days.A definitive diagnosis of scrub typhuscomplicating SLE was made. The patient wasprescribed 500 mg of oral tetracycline every sixhours by the medical on-call team. The fever abatedwithin two days and the patient felt much better.On clinical grounds the treating team preferred notto change tetracycline to doxycycline. The generalcondition of the patient continued to improve andthe white blood cell and liver function tests becamenormal. She was discharged on oral prednisolone40 mg daily and tetracycline 500 mg qds for atotal of two weeks. On review the patient wasasymptomatic and well.DISCUSSIONSLE presenting as prolonged fever is a wellknownclinical entity. When classical clinicalsymptoms and signs are present and the appropriatelaboratory investigations are positive the diagnosisis usually straightforward. However borderlineJune 2009cases may take some time as the laboratoryinvestigations, ANA and anti-dsDNA antibody maynot be positive at the beginning of the disease [3,4] .When the diagnosis of SLE is definitive and thedisease is treated with adequate dose of steroidsand other immunosuppressant the fever settlesquickly and skin rashes gradually fade or disappearaltogether.If fever does not settle quickly or if prolongedfever occurs, the diagnosis of an associated infectionshould be entertained. In our patient, when thediagnosis of SLE was made and oral steroidsprescribed, joint pains were relieved but feverpersisted. As the ESR remained elevated, the CRPbecame positive and the liver enzymes remainedhigh, we investigated for a possible infectivecause. Serological tests revealed an associatedinfection with scrub typhus. Isolation of Orientiatsutsugamushi (O. tsutsugamushi) was not attempted.With the appropriate dose of tetracycline the patientpromptly responded and the fever abated.Many major infections complicate the course ofSLE, and scrub typhus is rarely incriminated. Scrubtyphus is a mite-borne infectious disease caused byO. tsutsugamushi. The organism is a gram-negativecoccobacillus that is antigenically distinct fromthe typhus group rickettsiae, which is distributedthroughout the Asia Pacific rim. Scrub typhus isendemic in South East Asia, Australia but found onrare occasions in the Arabian Peninsula.Patients with scrub typhus develop high fever,generalized headache, diffuse myalgia with thepresence of a skin rash or a typical necrotic lesioncalled an eschar. Sometimes there is also generalizedlymphadenopathy and splenomegaly [5] . Laboratoryfindings include leucopenia or leucocytosis,elevation of hepatic enzymes and bilirubin [5] .The pathological hallmark of scrub typhus is alymphohistiocytic vasculitis seen on biopsy of theeschar or skin rashes [6] . It is difficult to culture O.tsutsugamushi and this is done only in specializedlaboratories. Scrub typhus lasts for 14 to 21 dayswithout treatment. Severe infections may becomplicated by interstitial pneumonia, pulmonaryedema, congestive heart failure, circulatory collapse,and a wide array of signs and symptoms of centralnervous system dysfunction, including delirium,confusion, and seizures. These complications maylead to death, usually late in the second week ofthe illness. Fortunately none of these complicationswere noted in our patient who responded quicklyto tetracycline.As scrub typhus is not endemic in the ArabianPeninsula and the patient did not travel abroad,the diagnosis of scrub typhus was not consideredinitially. Furthermore, she did not rememberbeing bitten by a mite and there were no skin
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