Kawasaki-like syndrome in an HIV-infected adult

G. Barbaro, G. Di Lorenzo and G. Barbarini1

Department of Medical Pathophysiology, University La Sapienza, Rome and 1Department of Infectious and Tropical Diseases, Policlinico S. Matteo, University of Pavia, Pavia, Italy

Correspondence to: G. Barbaro, Viale Anicio Gallo 63, 00174 Rome, Italy. E-mail: g.barbaro{at}tin.it

SIR, Kawasaki disease is an idiopathic vasculitis typically seen in children less than 5 yr of age [1]. Adult HIV-infected patients present with a constellation of signs and symptoms very similar to those of non-HIV children, except that gastrointestinal complaints are more common and cervical lymphadenopathy less pronounced [2]. Investigations of rare clinical syndromes are difficult, as they require either the incidental presentation of an affected individual to clinicians with an intimate knowledge of the disease or well-organized multicentre collaborations that can capture rare events. Because the first of these requirements was fulfilled, we were able to to investigate a Kawasaki-like syndrome in an HIV-infected adult.

A 34-yr-old HIV-infected homosexual male presented to the hospital with an illness of 1 week that began with fevers, sore throat, fatigue, malaise, anorexia, diffuse abdominal pain, nausea and arthralgias. He subsequently developed conjuctival injection, a diffuse pruritic skin rash involving the trunk and extremities, and painful erythema/swelling of his hands and feet. His past medical history was remarkable only for HIV infection, diagnosed in 1996. He had been on stavudine–didanosine (DDI)– indinavir since 1999. His most recent CD4 count was 512 cells/mm3 and the viral load was 850 copies/ml. Results of his physical examination included conjuctival injection without exudate, injection of the oral pharynx without exudate, tender cervical adenopathy (<1 cm), painful erythema/swelling of his hands and feet, and a diffuse macular rash involving the trunk and extremities. He was tachycardic without pathological murmur, his spleen was mildly enlarged, and his joints were unremarkable. He had daily fevers in hospital, with maximum temperature of 38.4°C.

On admission, his antiretroviral therapy was stopped, laboratory studies were performed and a skin biopsy was taken before therapeutic intervention. Because the patient was a Jehovah's Witness and refused all blood products, he was started on a course of methylprednisolone [40 mg intramuscularly twice daily] and aspirin (10 mg/kg once daily) for 1 week rather than standard Kawasaki disease therapy with intravenous immunoglobulin (Ig) and aspirin. He defervesced on the third day of methylprednisolone therapy. The patient continued methyprednisolone at 20 mg daily intramuscularly for an additional week and aspirin at a reduced dose (160 mg daily). After 1 week of therapy, all the patient's protean symptoms had resolved, including the sore throat, pain in his hands and feet and pruritic rash. Periungal peeling was noted on a follow-up out-patient clinic visit 17 days after admission to hospital. At 6 weeks he was symptom-free except for some residual fatigue. Antiretroviral therapy was restarted with a regimen of stavudine–lamivudine– efavirenz, and low-dose aspirin therapy (160 mg daily) was continued.

Table 1 summarizes the immunological data collected before initiation of steroid therapy and 6 weeks after hospital discharge. Our patient had a selective elevation of serum IgA on presentation that had not returned to a normal value 6 weeks later. Cytokine analysis revealed acute elevations of interferon (IFN)-{alpha} and IFN-{gamma} that resolved within 6 weeks. An extensive evaluation for potential infectious aetiologies (including Epstein–Barr virus, cytomegalovirus, parvovirus-19 and human herpesvirus-6) was negative. Skin biopsy showed an infiltrate of lymphocytes, neutrophils and IgA plasma cells surrounding the superficial capillary plexus in the papillary dermis, with destruction of small venules and moderate fibrinoid necrosis. Immunohistochemistry of a skin biopsy sample is shown in Fig. 1.


View this table:
[in this window]
[in a new window]
 
TABLE 1. Immunological data of the patients

 


View larger version (145K):
[in this window]
[in a new window]
 
FIG. 1. Direct immunohistochemistry of the skin using an IgA-specific monoclonal antibody (x40), showing marked infiltration of IgA+ B lymphocytes (stained) within the lesions. There was minimal deposition of complement (C1q, C3) in the dermis vessel walls. This figure can be viewed in colour as supplementary material at Rheumatology Online.

 
A feature unique to paediatric Kawasaki disease is the presence of IgA plasma cells within the vasculitic lesions [3]. This feature may also be observed in HIV-infected patients with Kawasaki-like syndrome, suggesting a common pathophysiology [2] rather than a common aetiology, because several agents might lead to the same histochemical and cytokine pattern. Moreover, the low levels of some complement fractions (C1q and C3) observed both in the acute and in the recovery phase is not a feature of Kawasaki disease, in which a classical activation pathway has been described [5]. HIV practitioners need to be aware of hypersensitivity reactions to antiretroviral agents because of the potential for fatal outcomes [4]. This was a remote possibility in our case, as the patient had been on therapy with DDI and indinavir for 3 yr without any specific reaction. On the contrary, abacavir hypersensitivity should always be considered as a possible aetiology for a vasculitic syndrome in an HIV-infected patient [4].

From a therapeutic perspective, a distinction between the syndromes is not critical as the treatments for pediatric Kawasaki disease and adult HIV–Kawasaki-like syndrome are identical. HIV-infected patients with Kawasaki-like syndromes respond to intravenous Ig and aspirin, the standard therapy for paediatric Kawasaki disease [2]. As in the paediatric syndrome, steroids appear to be a reasonable alternative for HIV-infected patients unable to be treated with standard immunoglobulins. Though it is somewhat counterintuitive, short-term steroids are not likely to be detrimental to the control of HIV viraemia. Immunohistochemical evaluation for IgA plasma cells in skin biopsies may be a useful adjunct in making a diagnosis of a Kawasaki-like syndrome in an HIV-infected adult. Although our data suggest a common pathophysiology, final resolution of the relationship between these syndromes awaits identification of the aetiological agent of paediatric Kawasaki disease.

The authors have declared no conflicts of interest.

References

  1. Kawasaki T, Kosaki F, Okawa S, Shigematsu I, Yanagawa H. A new infantile acute febrile mucocutaneous lymph node syndrome (MLNS) prevailing in Japan. Pediatrics 1974;54:271–6[Abstract]
  2. Johnson RM, Little JR, Storch GA. Kawasaki-like syndromes associated with human immunodeficiency virus infection. Clin Infect Dis 2001;32:1628–34[CrossRef][ISI][Medline]
  3. Rowley AH, Eckerley CA, Jack HM, Shulman ST, Baker SC. IgA plasma cells in vascular tissue of patients with Kawasaki syndrome. J Immunol 1997;159:5946–55[Abstract]
  4. Hewitt RG. Abacavir hypersensitivity reaction. Clin Infect Dis 2002;34:1137–42[CrossRef][ISI][Medline]
  5. Kohsaka T, Abe J, Asahina T, Kobayashi N. Classical pathway complement activation in Kawasaki syndrome. J Allergy Clin Immunol 1994;93:520–5[ISI][Medline]
Accepted 5 March 2003





This Article
Full Text (PDF)
Alert me when this article is cited
Alert me if a correction is posted
Services
Email this article to a friend
Similar articles in this journal
Similar articles in ISI Web of Science
Similar articles in PubMed
Alert me to new issues of the journal
Add to My Personal Archive
Download to citation manager
Disclaimer
Request Permissions
Google Scholar
Articles by Barbaro, G.
Articles by Barbarini, G.
PubMed
PubMed Citation
Articles by Barbaro, G.
Articles by Barbarini, G.
Related Collections
Other Rheumatology