Postgrad Med J (1991) 67, 297 - 298 i) The Fellowship of Postgraduate Medicine, 1991 Postgrad Med J: first published as 10.1136/pgmj.67.785.297 on 1 March 1991. Downloaded from
Disseminated gonococcal infection associated with deficiency ofthe second component ofcomplement P.H.M. McWhinney, P. Langhorne, W.C. Love and K. Whaley' Department ofInfection and Tropical Medicine, Ruchill Hospital, Glasgow G20 9NB and 'University of Glasgow Department ofPathology, Western Infirmary, Glasgow GIl 6NT, UK
Summary: A case of C2 deficiency presenting with disseminated gonococcal infection is described. The predisposition ofC2-deficient individuals to infection in addition to the commoner problem ofimmune complex diseases is noted. Attention is drawn to the absence of documented cases of gonococcal infection associated with C2 deficiency. No other homozygous C2 deficient family members were identified. Lifelong penicillin prophylaxis was recommended for the patient.
Introduction Deficiency of the second component of comple- Apart from a single temperature spike of38TC he ment (C2) is an autosomal recessive disorder often remained apyrexial. He had marked painful limita- associated with systemic lupus erythematosus tion of movement in his wrists, knees and ankles (SLE) like syndromes,' but also frequently but no synovial effusion. Sparse cutaneous lesions - associated with recurrent sepsis. The most com- small erythematous macules, tiny pustules and monly implicated organisms are meningococci, crusted lesions - were noted. After admission a few by copyright. pneumococci and Haemophilus influenzae.2 The more pustules developed. Otherwise examination association of terminal complement deficiency was unremarkable. Routine biochemical and (particularly C8) with disseminated gonococcal haematological investigations were normal except infection (DGI) is well documented,3 but C2 for an erythrocyte sedimentation rate of 49 and a deficiency has not been noted to be associated with C-reactive protein of 21 mg/l (normal range < 6). gonococcaemia. We report a case of subcute His ASO titre was less than 200 Todd units. A gonococcal septicaemia associated with C2 throat swab grew group C beta haemolytic strepto- deficiency. cocci. No organisms were grown from the skin
lesions. Blood cultures grew Neisseria gonorrhoeae http://pmj.bmj.com/ serogroup WI (genetic serovar: Aedgkih, Phar- Case report macia serovar: Arost), which was sensitive to less than 0.015 mg/1 penicillin. Tests for antinuclear A 22 year old single male presented with a 6 day factor, rheumatoid factor, anti-Ro, anti-La, anti- history of sore throat and a migrating arthropathy Sm and anti-RNP were all negative. Viral sero- of knees and wrists associated with a skin rash. logical screens were negative as was serology for While on holiday in Spain 2 months prior to meningococci, yersinia, syphilis and Borrellia burg- admission he had a similar episode of self limiting dorferi. Details of complement levels are shown in on September 29, 2021 by guest. Protected arthropathy, associated with diarrhoea but no skin Table I. rash. He had mild asthma, and gave a history ofan He was treated with benzyl penicillin and made a episode ofbronchitis and two episodes of pleurisy. rapid and complete recovery. There was no His only medications were a salbutamol inhaler evidence of other sexually transmitted disease. His and, just prior to admission, indomethacin. He had immediate family all have normal levels of C2. recent unprotected sexual contact with a known Lifelong prophylaxis with phenoxymethyl penicil- female partner, but denied any other contacts. lin has been recommended.
Discussion
Correspondence: P.H.M. McWhinney, M.R.C.P., C2 deficiency appears to present most frequently Department of Medical Microbiology, Royal Free with immune complex diseases.4 However, in a Hospital, Hampstead, London NW3 2QG, UK series of 38 cases of which 36 had presented with a Accepted: 16 October 1990 connective tissue disorder, many had a history of 298 CLINICAL REPORTS Postgrad Med J: first published as 10.1136/pgmj.67.785.297 on 1 March 1991. Downloaded from
Table I Complement levels case represented the coincidence of two independ- ent conditions. Furthermore it has been shown that Component Level Normal range in vitro killing ofN. gonorrhoeae is slowed when C2 deficient plasma is used.5 CH50 0 U/ml 150-250 U/ml alternative pathway The organism isolated in this case is a serum- Haemolytic activity 175 U/ml 143-384 U/ml resistant strain typical of those associated with Clq 121% 63-158% DGI.6 Even these do not usually cause systemic CIr 113% 64-158% disease, and individuals who develop DGI may CIs 181% 70--145% have defective host defence mechanisms. Addi- C2 Undetectable 56-175% tionally gonococci not usually associated with C3 1.3 mg/ml 0.72-1.8 mg/ml dissemination may cause invasive disease in such C4 439 jg/ml 199-574 tg/ml patients.7 Cl inhibitor 310 fg/ml 160-370 fg/ml C2 deficiency is the most common complement Factor B 148 jug/ml 149-421 jug/ml deficiency with a prevalence of 1:10,000. It has already been suggested that gonococcal septi- caemia is an indication to screen for complement significant bacterial infections.' In another series of deficiencies,8 and this case adds further weight to 77 individuals with C2 deficiency,2 32 had had that suggestion. Measurement of the CH50 is a significant bacterial infections, including I1 indi- convenient and readily available screening test viduals with pneumococcal sepsis on one or more which will prompt further investigation where occasion and 3 each ofmeningococci, H. influenzae appropriate. Diagnosis ofthe defect has important and tuberculosis, but no cases ofDGI. The absence implications for the long term prophylaxis of of cases of DGI in both of these series and other individuals who are prone to infections (a sub- published cases is striking, implying either that C2 group of C2 deficient individuals).9 As the most deficiency does not predispose to gonococcal sepsis commonly diagnosed pathogen is S. pneumoniae, or that there has been a failure to diagnose C2 oral penicillin is an appropriate antibiotic. deficiency in infected patients. Alternatively, cases Pneumococcal vaccine may not be effective. Theby copyright. may not have been reported due to the mistaken predisposition to infections may only become ap- assumption that the well documented association parent when individuals (as in this case) are aged of C2 deficiency with neisserial sepsis includes over 20 years, and family members should be DGI. As DGI is a less spectacular infection there screened, so primary prophylaxis may be offered may be a tendency for cases not to be investigated. where appropriate. It has also been suggested that as aerial transmis- sion is important for meningococci they have greater opportunities for spread to susceptible Acknowledgement
individuals than gonococci.2 In view of the suscep- http://pmj.bmj.com/ tibility of C2-deficient individuals to infections by We are grateful to Dr R. Fallon, Department of Micro- capsulated bacteria it would be surprising if this biology, Ruchill Hospital for his advice.
References
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