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Published on: November 5, 2019
Chronic meningococcal disease: Systematic literature review
Lukas P Schulz1, Beatrice Gatti1, Gregorio P Milani2
1Family Medicine Institute, Faculty of Biomedical Sciences, Università della Svizzera Italiana, Lugano, Switzerland.
Background:
Chronic meningococcal disease is a rare and under-recognized manifestation of Neisseria meningitidis infection. Unlike acute meningococcal infections, its presentation is insidious, leading to diagnostic delays. We conducted a systematic review to better characterize its clinical, microbiological, and epidemiological profile.
Methods:
A systematic review was performed in accordance with PRISMA 2020 guidelines and registered in PROSPERO (CRD42024608035). We searched Excerpta Medica, National Library of Medicine, Web of Science, Google Scholar, and bibliographies for cases reported since 1960 using the terms "chronic meningococcemia" or "chronic meningococcal disease." Eligible cases involved previously apparently healthy individuals with N. meningitidis isolated from a normally sterile site. Two reviewers independently extracted data on demographics, clinical features, diagnostics, microbiology, treatment, and outcomes.
Results:
A total of 126 cases from 97 reports were included. Most patients (median age: 21 years) presented with recurrent fever (98 %), non-blanching skin lesions (95 %), and musculoskeletal symptoms (90 %) over a period of ≥ 10 days. In over two-thirds of cases, symptoms followed a relapsing-remitting pattern, with alternating episodes of fever, rash, and joint involvement. Diagnosis was delayed longer in adults than in children (P < 0.0001). Blood cultures were positive in 77 % of cases; molecular testing and skin biopsy improved diagnostic yield. N. meningitidis serogroup B (58 %) and C (31 %) were most common. Meningeal signs were largely absent (94 %). Antimicrobial therapy led to complete recovery without sequelae in all patients. Testing for inherited susceptibility was conducted in 52 cases and revealed abnormalities - primarily complement deficiencies - in 13 (23 %) of them.
Conclusions:
Chronic meningococcal disease should be considered in individuals with relapsing fever, vasculitic skin lesions, and joint involvement, especially when cultures are negative or symptoms mimic viral or autoimmune conditions. Early use of molecular diagnostics and awareness of characteristic patterns may improve recognition. The term "relapsing-remitting meningococcal disease" may better reflect its clinical nature.
Insights
Chronic meningococcal disease, a rare Neisseria meningitidis infection, presents insidiously with relapsing fever and skin lesions. Early molecular diagnostics and recognizing its pattern improve recognition and treatment outcomes.
Area of Science:
- Infectious Diseases
- Microbiology
- Clinical Medicine
Background:
- Chronic meningococcal disease is an under-recognized Neisseria meningitidis infection.
- It presents insidiously, differing from acute forms and often causing diagnostic delays.
Purpose of the Study:
- To systematically review and characterize the clinical, microbiological, and epidemiological profile of chronic meningococcal disease.
- To identify key features that aid in earlier diagnosis and management.
Main Methods:
- Systematic review following PRISMA 2020 guidelines.
- Searched multiple databases for cases reported since 1960 using specific keywords.
- Extracted data on demographics, clinical features, diagnostics, microbiology, treatment, and outcomes from eligible cases.
Main Results:
- 126 cases were identified, predominantly in young adults, with recurrent fever (98%), skin lesions (95%), and musculoskeletal symptoms (90%).
- Symptoms often followed a relapsing-remitting pattern; diagnosis was delayed longer in adults.
- Neisseria meningitidis serogroups B and C were most common; meningeal signs were rare. All patients recovered with antimicrobial therapy.
Conclusions:
- Chronic meningococcal disease should be suspected in patients with relapsing fever, vasculitic lesions, and joint pain, especially with negative cultures.
- Early molecular diagnostics and awareness of characteristic patterns are crucial for improved recognition.
- The term 'relapsing-remitting meningococcal disease' may better describe its clinical presentation.
