Methods: Immune evaluations were negative except for occasional Howell-Jolly bodies on their blood smears. Immunoglobulins were normal as were their post vaccine responses to pneumococcal polysaccharide vaccine-23 (Pneumovax®). Complement levels: C3, C4 and CH50 were normal. Lab but not clinical markers of SLE were present. In both patients, the spleen was anatomically present. In both, there was near absent splenic function on a heat damaged tagged RBC TC 99 m spleen scan. A literature search was carried out using Medline/PubMed and Google.
Results: Streptococcus pneumonic infections make up about 6-18% of all bacterial infections in SLE; most are pneumonia. Although several cases of pneumococcal sepsis/shock have been reported in such patients, we could not find similar cases of recurrent pneumococcal meningitis in patients with inactive, untreated SLE.
Conclusion: Recurrent pneumococcal meningitis is uncommon in adults and is usually associated with humoral immune deficiency, CSF leaks or cochlear implants. Complement deficiency (primary) is rarely found. Sickle cell disease and other hemoglobinopathies have also been associated with pneumococcal sepsis and meningitis.
SLE and other autoimmune connective tissue disorders are associated with functional asplenia, even when clinically inactive. These patients are at increased risk for invasive pneumococcal disease.
Functional asplenia in adult patients is often overlooked in patients with severe or recurrent infections caused by polysaccharide encapsulated bacteria.
We report on two patients with recurrent pneumococcal meningitis and SLE. Functional asplenia and complement deficiency are the primary factors when such patients develop invasive or recurrent infections. Demonstration of a poorly functional spleen by a TC 99 heat denatured RBC spleen scan when the spleen is anatomically present confirms the diagnosis.
D. Fink-Bennett, None