Introduction
Background
Gonococcemia is defined as the presence of Neisseria gonorrhoeae in the bloodstream, which can lead to the development of disseminated gonococcal infection (DGI). Gonorrhea is the second most often reported sexually transmitted disease (STD) in the United States behind chlamydia. About 600,000 people each year in the United States are infected, with only about half being reported.1 Gonococcemia occurs in about 0.5-3% of patients with gonorrhea.
The clinical manifestations of this process are biphasic, with an early bacteremic phase consisting of tenosynovitis, arthralgias,2 and dermatitis, followed by a localized phase consisting of localized septic arthritis. Other potentially severe clinical complications include osteomyelitis, meningitis, endocarditis, adult respiratory distress syndrome (ARDS),3,4 and fatal septic shock.5 Polymyositis is also a rare complication of gonococcemia.
Patients who are pregnant or menstruating may be particularly prone to gonococcemia. Other populations that are at risk of infection include women and those with complement deficiencies, HIV disease, or systemic lupus erythematosus (SLE). DGI is an important, potentially life-threatening, and easily treatable clinical entity that remains the most common cause of acute septic arthritis in young sexually active adults.
Related eMedicine articles include Gonococcal Infections and Gonococcal Arthritis.
Pathophysiology
N gonorrhoeae organisms are spread from a primary site, such as the endocervix, the urethra, the pharynx, or the rectum, and disseminate to the blood to infect other end organs. Usually, multiple sites, such as the skin and the joints, are infected. Neisserial organisms disseminate to the blood due to a variety of factors. Such predisposing factors include host physiologic changes, virulence factors of the organism itself, and failures of the host's immune defenses.6 For example, changes in the vaginal pH that occur during menses and pregnancy and the puerperium period make the vaginal environment more suitable for the growth of the organism and provide increased access to the bloodstream.7,8Organismal virulence factors, such as pili, aid in adherence of the organism to mucosal surfaces and impede phagocytosis by host macrophages. Outer membrane proteins (ie, proteins 1, 2, and 3) are also involved in determining the virulence of the strain of organism and are used to type the strain (ie, protein 2 is involved in adhesion to host cells). Lipo-oligosaccharides of the organism's cell membrane have marked endotoxic action and are also believed to be related to resistance to serum bacteriocidal action. Additionally, some strains of Neisseria species that are particularly pathogenic produce immunoglobulin A (IgA) proteases that aid in the survival of the organism in mucosal tissues.
Defects in the host's immune defenses are also involved in the pathophysiology, with certain patients more likely to develop bacteremia. Specifically, patients with deficiency in terminal complement components are less able to combat infection, as complement plays an important role in the killing of neisserial organisms. As many as 13% of patients with DGI have a complement deficiency. A study of 22 patients with DGI revealed that total serum complement activity was greater than 25% below the normal mean. Other causes of immunocompromise (eg, HIV, SLE) also predispose to dissemination of infection.
Frequency
United States
The incidence of DGI naturally parallels the incidence of gonococcal infection. In the United States, the number of gonococcal infections peaked in the 1970s, the era of the sexual revolution. With the onset of the HIV epidemic and the practicing of safe sex techniques, the incidence has dramatically decreased from 468 cases per 100,000 population in 1975 to 100-150 cases per 100,000 population at the turn of the century.
N gonorrhoeae infection, the second most commonly reported notifiable disease in the United States, has incidence rates that have been either declining or stable since 1996. However, in 2005, the national rate (115.6 cases per 100,000 population) increased for the first time since 1999. Further, from 2000-2005, rates in the western United States increased 42%, from 57.2 cases to 81.5 cases per 100,000 population, whereas rates in the 3 other US regions decreased (South, -22%; Northeast, -16%; Midwest, -5%).9
International
The incidence in developing countries is much greater than that of the United States and Western Europe, where higher levels of education and better access to health care are available. DGI develops in about 0.5-3% of persons with mucosal infection. Azariah and Perkins report increasing prevalence in New Zealand, with the primary risk factors being age younger than 25 years and Māori or Pacific ethnicity.10
Sex
DGI is more likely to occur in women because of a higher incidence of occult infection (difficulty in diagnosis) and also because of menstruation and pregnancy.
Age
Gonococcemia remains an important disease in the adolescent and young adult population, with a peak incidence in males aged 20-24 years and females aged 15-19 years. Symptoms and/or diagnosis in young children should raise the issue of potential child abuse.11 Martin et al report on the identification of N gonorrhoeae from a piece of clothing, which was subsequently used to convict a man in the child sexual abuse case .12
Clinical
History
History reflects the classic clinical manifestations of gonococcemia — cutaneous lesions, arthritis, and, possibly, pericarditis, discussed in Physical. A dramatic increase in the incidence of gonorrhea has been observed, emphasizing the increasing importance of its complications, particularly in pregnancy.7
Physical
The clinical evolution of DGI is biphasic consisting of a bacteremic phase and a localized, suppurative phase.
- Bacteremic phase
- In this phase, which occurs during the first 2-3 days of gonococcemia, the patient experiences polyarthralgias and constitutional symptoms, such as malaise, fever, and weakness.
- The classic skin lesions are acral hemorrhagic pustules.13
Disseminated gonococcemia, acral pustules.
- This phase is associated with a clinical picture of polyarthritis, dermatitis, and tenosynovitis. The joints most commonly involved include those of the extremities, including the wrists, the fingers, the elbows, the knees, and the ankles, with 70% of patients experiencing a migratory polyarthralgia of 1-3 joints and the remaining 30% having involvement of more than 3 joints. This arthritis is believed to be a sterile arthritis with negative culture results. Certain patient populations, such as patients infected with HIV, can experience involvement of unusual joints, such as the sternoclavicular joint and the hips, and the arthritis may have a more aggressive course, with potential destruction of the joint.
- About 75% of patients experience a dermatitis that can vary from macular/papular to vesicular/pustular to necrotic or hemorrhagic erythema. The dermatitis is nonscarring.
- Vasculitis has also been reported.
- Skin lesions are often in multiple stages of development, and 5-50 individual lesions can be present. They are mostly located on the distal extremities. The face, the scalp, the palms, the soles, and the trunk are classically spared, but not always.14 Lesions can be painful but are usually asymptomatic, and they resolve in 4-7 days even without treatment.
- In this bacteremic phase, the possibility of pericarditis, endocarditis, perihepatitis (Fitz-Hugh-Curtis syndrome), osteomyelitis, glomerulonephritis, as well as other end-organ involvement must be considered, but the occurrence of these conditions is rare and continues to decrease with effective treatment and earlier diagnosis of disease.
- Localized, suppurative phase
- This phase usually occurs on days 3-6 of the infection and consists of mainly arthritis. In contrast to the arthritis/arthralgia of the bacteremic phase, this arthritis is a septic arthritis with purulent joint fluid and a positive culture result in 50% of patients. WBC counts in aspirated joint fluid are typically 50-100,000 cells/μL and consist of more than 90% neutrophils.
- Skin findings in this phase are minimal; only 15-20% of patients have active skin lesions, and the remaining 80-85% of patients have resolved dermatitis or resolving dermatitis.
Causes
Risk factors for both mucosal infection and disseminated infection include sexual activity/promiscuity, lower socioeconomic status, ethnic minority, male homosexuality, drug use, lower educational level, and past history of other STDs. See Pathophysiology.
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Further Reading
Keywords
gonococcemia, gonococcal infection, gonorrhea, arthritis-dermatitis syndrome of gonorrhea, disseminated gonococcal infection, DGI, Neisseria gonorrhoeae, N gonorrhoeae
