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Last updated:
May 15, 2009
Years published: 1992, 1993, 1995, 1996, 1997, 1998, 1999, 2007, 2009
Meningococcemia is a rare infectious disease characterized by upper respiratory tract infection, fever, skin rash and lesions, eye and ear problems, and possibly a sudden state of extreme physical depression (shock) which may be life-threatening without appropriate medical care. There are two forms of meningococcemia. Fluminant meningococcemia develops very rapidly and is more severe than chronic meningococcemia, which has a waxing and waning course.
Meningococcemia is characterized by sudden intense headache, nausea, fever, vomiting, and skin rash. The affected individual may first complain of an upper respiratory infection. Chills may develop, then skin rash on the arms or legs and the trunk. Diarrhea may also be present. Later the rash may become widespread or develop into bleeding spots under the skin (petechiae, ecchymoses, or purpura). There may be associated swelling, muscle pain, skin deterioration or gangrene in the arms and legs. Pneumonia may also develop along with the other symptoms if the affected individual has a suppressed immune system.
In cases where meningitis occurs along with meningococcemia, the affected individual may have the symptoms listed above along with the combination of headache, confusion, stiff neck, and muscle pain from irritation of membranes surrounding the brain and spinal cord (meningismus). (For more information on this disorder, choose “Meningitis” as your search term in the Rare Disease Database).
Fulminant Meningococcemia is also known as Waterhouse-Friderichsen Syndrome and is the most severe form of the disorder. The disease comes on very suddenly and the progression of the symptoms is very rapid. In less than a few hours the affected individual may experience very high fever, chills, weakness, vomiting and severe headache. A red rash appears on the arms and legs and spreads very quickly over the body including the eyes and nose. In addition, the affected individuals blood pressure may drop dangerously, the fever may drop dramatically, and they may go into shock. Without immediate medical treatment this disorder can be life-threatening.
Chronic Meningococcemia is a rarer form of the disease. It is characterized by fever that comes and goes over a period of weeks or months. Muscle and joint pain with headache as well as a skin rash may also come and go. This form of the disorder may also include an enlarged spleen.
Meningococcemia is caused by infection with the meningococci bacteria (Neisseria memingitidis) which are gram-negative diplococci bacteria. There are various groups of this bacteria that cause different forms of the disease and they are grouped by strains A, B, C, D, X, Y, Z, 29E and W135. These groups can be identified by testing the blood, scrapings of the skin rash and samples of the cerebrospinal fluid of the patient. Testing may take up to five days as the cultures are very slow growing.
Infection with the bacteria is usually caused by a carrier. The natural place for the bacteria to be located is in either the nose or throat of the carrier, and they can be spread the infection through airborne or close contact methods. The carrier may spread the infection for weeks or months if they are not diagnosed and treated.
Meningococcemia affects males and females in equal numbers. However, most cases develop in persons twenty years of age or younger and half of these cases are in children under five years of age. In the United States 1.2 cases per 100,000 occur annually. Winter and spring are the most common seasons of the year when cases are reported. Epidemics can occur under crowded conditions and tend to occur at 20 to 30 year intervals. In other parts of the world epidemics are usually caused by the Group A strain of the bacteria. During epidemics, rates of 5 to 24 cases per 100,000 persons have occurred. In Sao Paulo, Brazil, during 1974 the epidemic rate was 370 per 100,000 persons infected with Meningococcemia. In the United States, the most prevalent Group strains of the bacteria are B,C,Y, and W-135.
The diagnosis of Meningococcemia may be confirmed by a thorough clinical evaluation and specialized blood tests.
Treatment
Meningococcemia is usually treated with Penicillin or Ampicillin. In adults the method of treatment is often through intravenous Penicillin G. In children penicillin is still the treatment of choice, however, other organisms must be ruled out before treatment is begun. For persons who are unable to take penicillin, other antibiotics are used such as: cefuroxime, cefotaxime or ceftriaxone.
In persons who survive severe meningococcal septicemia there may be ongoing problems with veins and arteries. There are usually serious orthopedic problems. If gangrene occurs amputation may be necessary. These patients should have continuing medical evaluations as a precaution against other conditions that can arise in later years.
During times of epidemics, prophylaxis with other antibiotics (i.e., Rifampin, minocycline, and sulfadiazine) is used to protect persons exposed to or in close contact with infected patients.
A new orphan product used for the prevention and treatment of purpura fulminans in Meningococcemia is being developed by Immuno Clinical Research Corp. of New York. The name of the new product is Protein C Concentrate (Protein C Concentrate (Human) Vapor Heated, Immuno).
Researchers are studying the safety and effectiveness of an experimental vaccine for Meningococcemia.
The drug recombinant bactericidal/permeability-increasing protein (Neuprex) has received an orphan drug designation for use in the treatment of Meningococcemia. More studies are needed to determine the long-term safety and effectiveness of this treatment of Meningococcemia. For more information, contact:
Xoma Corporation
2910 Seventh Street
Berkeley, CA 94710
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. Government funding, and some supported by private industry, are posted on this government web site.
For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: prpl@cc.nih.gov
For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com
TEXTBOOKS
Cecil Textbook of Medicine, 20th Ed.: J. Claude Bennett and Fred Plum, Editors; W.B. Saunders Co., 1996. Pp. 1619-21.
Harrison’s Principles of Internal Medicine, 14th Ed.: Kurt J. Isselbacher, M.D. et al., Editors; McGraw-Hill, Inc., 1998. P. 912.
Internal Medicine, 4th Ed.: Jay H. Stein, Editor-In-Chief; Mosby-Year Book, Inc., 1994. P. 2114.
Infectious Diseases: Sherwood L. Gorbach, John G. Bartlett, and Neil R. Blacklow, Editors; W.B. Saunders Company, 1992. P. 1455.
Mandell, Douglas and Bennett’s Principles and Practice of Infectious Diseases, 4th Ed.: Gerald L. Mandell, M.D. et al., Editors; Churchill Livingstone Inc., 1995. Pp. 1899-1906.
Clinical Dermatology, 2nd Ed.: Thomas P. Habif, Editor; The C.V. Mosby Company, 1990. Pp. 210-11.
JOURNAL ARTICLES
Chondro-Osseous Growth Abnormalities After Meningococcemia, A Clinical and Histopathological Study. D. P. Grogan et al.; J Bone Joint Surg (July 1989; 71(6)). Pp. 920-28.
Meropenem Pharmacokinetics in a Patient with Multiorgan Failure From Meningococcemia Undergoing Continuous Venovenous Hemodiaflitration. M. M. Meyer et al.; Am J Kidney Dis (Apr 1999; 33(4)). Pp. 790-95.
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Learn more https://rarediseases.org/patient-assistance-programs/medicalert-assistance-program/Ensuring that patients and caregivers are armed with the tools they need to live their best lives while managing their rare condition is a vital part of NORD’s mission.
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