Moore, M D

Person, Donald A.

eVols at University of Hawaii at Manoa

Harry L. Arnold Jr. MDCase of the MonthChronic Meningococcemia MimickingAcute Rheumatic FeverDonald L Person, MD, Colonel, MC, USA*and Mary D. Moore, MD **chronic meningococcemia is an uncoimnon feature of meningococcal disease, particularly in childhood. In fact, less than two dozencases have been previously reported in the English literature. Thepresentation of chronic meningococcemia is common to a numberof infectious and rheumatic diseases. In Benoit’s review of 148cases: all had fever and chills, 138 (93%) had rash, and 104 (70%)had arthritis/arthralgia.’ A positive blood culture for Neisseriameningitidis establishes the diagnosis. The differential diagnosis isconsiderable and notably includes: Henoch - Scholein purpura(HSP), acute rheumatic fever (ARF), subacute bacterial endocarditis (S BE), Lyme disease, systemic lupus erthematosus (SLE), andsystemic juvenile rheumatoid arthritis (JRA). The case of a seven-year-old boy with chronic meningococcemia whose condition completely fulfilled the updated Jones criteria2for the diagnosis of ARF(to include the isolation of a Il-hemolytic streptococcus from histhroat), is here reported.Case ReportA previously healthy seven-year-old white boy presented withpain in his tight ankle and a limp. His arthritis subsided over twodays only to recur along with fever to 102.5T. He was hospitalized* Formerly, Assistant Professor ot Pediatrics, BaylorCollege ot Medicine, Attending Pediatrician and Rheumatologist, Texas Children’s Hospital, Houston,TX. Currently,Clinical Protessor ot Pediatrics, John A. Burns School otMedicine of the University of Hawaii at Manoa, Protessor ofClinical Pediatrics, F. Edward Herbert School of Medicine ofthe Uniformed Services University of the Health Sciences(Bethesda) and Chief, Department of Clinical Investigation,Tripler Army Medical Center, Honolulu, HI 96859-5000.Formerly Pediatric Rheumatology Fellow, Baylor College of Medicine, Houston, TX. Currently, Associate Professor of Clinical Pediatrics, University of Iowa Hospitals,Iowa City, IA 52242.Requests for reprints may be submitted to COL DonaldA. Person, MC, 1 Jarrett White Road, Department of ClinicalInvestigation, Tripler Army Medical Center, Honolulu, HI96859-5000.The opinions expressed are those of the author andshould not be construed as official policy of the Departmentof the Army or the Department of Defense.in a community hospital for eight days during which time hedeveloped evanescent pink, subcutaneous nodules on his extremities. His fever would resolve then recur every two to three days. Sixblood cultures were negative. A transient left knee effusion wasnoted on the seventh hospital day. A radionuclide bone scan wasnormal. He received no medication other than acetaminophen forfever and was referred on the eighth day of his illness. On admissionto the Texas childrens’ Hospital (Houston, TX), he had a temperature of lOT F and appeared moderately ill. Examination of the skinrevealed ten round, tender, red nodules on his right leg, buttock,back, occiput, and neck. The nodules varied in size from 5-10 mm.in diameter and were exquisitely tender to touch. He did not havepetechiae or purpura. A pericardial friction rub was heard on the firstday and resolved spontaneously. He had arthritis with a smalleffusion of his right ankle, complete blood count and urinalysiswere normal with the exception of a mild thrombocytosis (plateletcount was 569,000). His erythrocyte sedimentation rate (ESR) was50 mmlhour. Rheumatoid factor (RF) was negative as was theantinuclear antibody (ANA, negative <1:10). His C3 (third component of complement) was 150 mg/dl (normal 95-195) and the 4(fourth component of complement) was 23 mg/dl (normal 15-150).Total hemolytic complement activity (CH50)was 495 u/ml (normal304-480). His Inimunoglobulin G (IgG) was 1100 mg/dl (normal631-1298), Immunoglobulin A (IgA) 160 mg/dl (normal 70-3 12),Immunoglobulin M (1gM) 123 mg/dl (normal 56-258). Streptozymewas 400 STZ units, the antistreptolysin 0 (ASO) was 1:333 andantthyaluronidase was 1:512. Serologies for hepatitis A and Bviruses (HB antigen, HB antigen, HB antigen, anti-HB, anti-MB,anti-HB, and anti-HA antibody) were negative. A test for circulating immune complexes was normal (clq binding 9%, normal <13%). The PPD skin test was negative with a positive tetanus toxoidcontrol. His throat culture yielded a 13-hemolytic streptococcus, lateridentified as group F. Thejoint-bone scan, chestradiograph, electrocardiogram, and echocardiogram were all normal. He was afebrileuntil the third hospital day when he developed a temperature oflOOT, at which time two separate blood cultures were obtained.Penicillin VK (250 mg p.o. qid) and aspirin (80 mglkg/d in fourdivided doses) were started on the fourth day when the throat culturewas reported positive for a 13 hemolytic streptococcus and thediagnosis of ARF appeared to be established. On hospital day six theblood cultures obtained on day three were reported as positive for N.meningitidis, type A, establishing the diagnosis ofchronic meningococcemia, a full two weeks after the onset ofhis symptoms. His feverand nodules completely resolved within 24 hours ofstarting therapy.His right ankle effusion resolved within 72 hours and he had nofurther joint complaints. Aspirin was discontinued. His familyreceived rifampin prophylaxis, and the patient was treated with a tenday course of oral penicillin. At follow-up, two months afterdischarge, the child was found to be free of all symptoms.DiscussionAcute rheumatic fever was the main diagnostic consideration inthis child, The presence of migratory polyarthritis, a pericardialfriction rub, subcutaneous nodules (including occipital nodules),fever, and an elevated ESR all strongly supported a diagnosis ofARF.2The isolation of a 13-hemolytic streptococcus (later shown tobe group F) from the patient’s throat and positive serologic tests forHAWAII MEDICAL JOURNAL. V0L57, AUGUST1998583ant.istreptococcal antibodies confirmed the probable diagnosis ofARF and prompted the institution of penicillin and aspirin therapy.The patient was asymptomatic and had already received 48 hours oforal penicillin when the blood culture results became available.Thus, the decision for continued oral antibiotic therapy was made.Remarkably, T. Duckett Jones, in his classic paper on the diagnosisof rheumatic fever mentions “menigococcic septicemia” as well as“gonococcic arthritis” in the differential diagnosis of ARF.3Chronic meningococcemia is uncommon in children as comparedto adults. Conversely, meningococcal disease, overall, is much morecommon in children and the incidence is the highest in infants lessthan one year of age. Since 1902, only 20 patients less than 18 yearsof age with chronic Ineningococcemia have been described in theEnglish speaking literature.4’5Leibel et at reviewed 13 of these casesin l974. The symptoms in children were similar to those in adults,but less severe. However, the duration of illness, prior to diagnosiswas shorter in children, and the first positive blood culture occurredearlier in the course of the illness than in adults. Ploysangam andSheth reviewed the literature and added the twentieth case ofchronicchildhood meningococcemia in 1996.Diagnosis is based on the finding ofapositive blood culture for N.meningiditis. Treatment with penicillin for a 10-14 day course isrecommended by most authors. The parenteral route is preferred.Since 1970, there have been several reports of individuals withchronic meningococcemia who were found to have deficiencies ofvarious complement components, including C6, C7, and C8.69 Onecase ofchronic meningococcemia and mild 1gM deficiency has beenreported.’° Deficienciesof C3, C5, C6, C7, andC8 have been associated with recurrentepisodes of disseminatedgonococcal infectionand acute meningococcal meningitis.7’8A 14month old boy withIgG, subclass deficiency and recurrentacute meningococcemia has been described,11 as has a 15-year-old French boywith IgA deficiency.’2The overall incidenceof complement deficiencies in the generalpopulation is low withC2 deficiency being themost common, the frequency of C2QO beingless than 1%. Completedeficiencies of the earlyclassic C componentsare associated withrheumatic diseaseswhich resemble SLE,vasculitis, and JRA.Homozygous deficiencies of the Membrane Attack Complex (MAC)(i.e. C5b-C9) predispose to recurrent neisserial sepsis. Ellison et atidentified six patients with complement component deficiencieswhen they sequentially screened 20 patients presenting with a firstepisode of meningococcal disease. Thus, 30% of their patients withacute meningococcal disease had an underlying deficiency of acomplement component.8The pathogenesis of chronic meningococcemia is not well understood. No predominant strain ofmeningococcus has been associatedwith this entity. Chronic meningococcemia appears to be a problemof host defense. One current view is that the disorder represents aserum sickness-like illness with the symptoms occurring secondaryto the circulation and deposition of antigen-antibody complexes.Certainly the recent histopathologic demonstration ofleukocytoclastic vasculitis in the skin lesions ofa 17-month-old boywith chronic meningococcemia lend credence to support that view.5It is not clear why our patient contracted a relatively benign formof meningococcemia. His nonnal immunoglobulin and complementlevels rule out an obvious deficiency of immunoglobulin or complement. This child was clinically and by all laboratory parameters,immunocompetent. There is no known association of chronic meningococcemia and an antecedent streptococcal infection. However,anecdotally, one of us (DAP) has observed recurrences, recrudescences, and/or exacerbations of HSP, JRA, and Kawasaki diseasewith concomitant group A, 13-hemolytic streptococcal pharyngitis.This child’s streptococcal infection certainly compounded the interpretation of his illness and was presumably responsible for theinduction of the anti-streptococcal antibodies. The classic syndromeassociated with ARF and that seen in our patient with chronicmeningococcemia may very well represent the final common pathway of the host-parasite relationship. Immunological reactions arevery likely operative and antigen-antibody complexes no doubt playa pivotal role. Only the antigens are different - streptococcal in ARFand meningococcal in chronic meningococcemia. This case, at thevery least, illustrates the importance of obtaining several bloodcultures in a child with fever, rash, and polyarthritis in order toestablish the correct diagnosis.References1. Benoit FL Chronic meningococcemia case report and review of the literature. Am JMed 1963,35:103-112.2. Special Writing Group of the Committee on Rheumatic Fever, Endocardtiis, and Kawasaki Disease ofthe Council on Cardiovascular Disease inthe Young of the American HeartAssociation. Guidelines forthe diagnosis of rheumatic fever. Jones Criteria, 1992 Update. JAMA. 1992, 268:20692O73.3. Jones TD. The diagnosis of rheumatic fever. JAMA. 1944, 126:481.484.4. Leel RL Fangman JJ, Ostrovsky MC: Chronic meningococcemia in childhood, Am J Die Child.1974,127:94-98.5. Ploysangam T, Sheth AP. Chronic Meningococcemia in childhood: case report and review of theliterature. Pediatric Derm. 1996,13:483-487.6. CloughJD, Clough ML WeinsteinA, etal Familial latecomplementcomponent (C6, C7) deficiencywithchronic meningococcemia. Arch Intern Med. 1980,140:929-933.7. Peterson BH, Lee TJ, Snyderman R, et al: Neisseria meningitldis and Nelsseria gor,orrhaeaebacteremia associated with C6, C7, or C8 deficiency. Ann Intern Med. 1979,90:917-920.8. Ellison RT, Kohler PF, Curd JG, et al. Prevalence of congenital or acquired complement deficiency inpatients with sporadic meningococcal disease. N EngJ Med 1983,308:913-916.9. Adams EM, Hustead 5, Rubin P, et al. Absence of the seventh component of complement in a patientwith chronic meningococcemia presenting as vasculitis. Ann Intern Med 1983,99:35-38.10. Fass RJ, Saslaw S. Chronic meningococcemia. Arch Intern Med. 1972,130:943-946.11. Bass JL, Nus R, Mehia K, et al. Recurrent meningococcemia associated with lgG2 - subclassdeficiency. NEngJMed. 1983,309:403.12. Farron F, CheseauxJJ, Pelet B. Meningococcemie chronique etdeficiten IgA chez un adofescent. ArchPediafc 1996,3:149.151.HAwAIIPATHOLOGISTSLABORATORYThe Full Service LabOffering ComprehensiveServices in..• Clinical Pathology• Surgical Pathology• Frozen Section Diagnosis• Pap Smears• Special Cytology• Flow Cytometry• Fine Needle Aspiration• Bone Marrow Interpretation• Specimen Photography• Image Analysis1301 Punchbowl StreetHonolulu, Hawaii 96813547-4271 Fax 547-4045HAWAII MEDICAL JOURNAL, VOL 57, AUGUST 1998584

Chronic meningococcemia mimicking acute rheumatic fever.

http://evols.library.manoa.hawaii.edu/bitstream/10524/53956/1/1998-08p583-584.pdf

Chronic meningococcemia mimicking acute rheumatic fever.

Abstract

Similar works

Full text

Available Versions

eVols at University of Hawaii at Manoa