AUTHOR AND EDITOR INFORMATION
Section 1 of 10
Author: Geofrey Nochimson, MD, Consulting Staff, Department of Emergency Medicine, Sentara Careplex Hospital
Geofrey Nochimson is a member of the following medical societies: American College of Emergency Physicians
Editors: Robert L Norris, MD, Associate Professor, Department of Surgery; Chief, Division of Emergency Medicine, Stanford University Medical Center; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; Jeter (Jay) Pritchard Taylor III, MD, Compliance Officer, Attending Physician Emergency Medicine Residency, Department of Emergency Medicine, Palmetto Richland Memorial Hospital, University of South Carolina; John D Halamka, MD, MS, Associate Professor of Medicine, Harvard Medical School, Beth Israel Deaconess Medical Center; Chief Information Officer, CareGroup Healthcare System and Harvard Medical School; Attending Physician, Division of Emergency Medicine, Beth Israel Deaconess Medical Center; Robert G Darling, MD, FACEP, Clinical Assistant Professor of Military and Emergency Medicine, Uniformed Services University of the Health Sciences, F Edward Hebert School of Medicine; Associate Director, Center for Disaster and Humanitarian Assistance Medicine
Author and Editor Disclosure
Synonyms and related keywords:
Q fever, query fever, Coxiella burnetii, C burnetii, rickettsial infection, rickettsial disease, biological warfare, biological warfare agent, category B agent
Background
First described in Australia in 1935, Q fever is a rickettsial disease with acute and chronic stages. Q fever differs from other rickettsial diseases in that it is caused by inhalation of infected particles, not by a tick bite. Because Q fever is transmitted via an inhalational route, it can be used as a biological warfare agent. The Centers for Disease Control and Prevention (CDC) classifies Q fever as a Category B agent.
Pathophysiology
The respiratory system is the main organ system affected, although GI and cardiac systems also are affected. Incubation period varies from 9-40 days, with an average range of 18-21 days.
Frequency
United States
Frequency is difficult to ascertain even though Q fever is a reportable disease in all US states except Delaware, Iowa, Oklahoma, Vermont, and West Virginia. In 2005, 136 cases were reported to the CDC; in 2006, 169 cases were reported. Dairy and slaughterhouse workers are most at risk. In 2006, the incidence was reported to be 0.06 per 100,000 population.
International
Incidence of Q fever is worldwide and varies in frequency and presentation from country to country. A recent outbreak occurred in the Netherlands in 2008.1 From January to July, 660 cases have been reported. This is the second outbreak to occur in the Netherlands since 2007. At this time, the cause is still unknown.
Mortality/Morbidity
The mortality rate with acute infection is reportedly as high as 2.4% but generally is less than 1%.
Sex
Males are affected more than females. Of the 169 cases reported to the CDC in 2006, 127 were in males and 42 were in females.
Age
Adults are affected more than children. The highest age range is between 40 and 64 years.
History
- Patients initially present with influenzalike symptoms.
- Respiratory symptoms appear 4-5 days after initial onset of illness (most prominently a dry nonproductive cough and pleuritic chest pain).
- Symptoms of Q fever include the following:
- Fever
- Severe headache
- Myalgias
- Anorexia
- Cough
- Pleuritic chest pain
- Sweats
- Chills
- Nausea, vomiting, and diarrhea (rare)
Physical
Often in acute Q fever, specific findings may not exist. In chronic Q fever, findings consistent with endocarditis and hepatitis more frequently are found.
- Findings in endocarditis include the following:
- Vegetations on any valve (although aortic and prosthetic valves are favored)
- Clubbing of digits
- Hepatomegaly and splenomegaly in approximately one half of patients
- Arterial emboli in approximately one third of patients
- Purpuric rash in approximately 20% of patients
- Findings in hepatitis include the following:
- Fever
- Malaise
- Hepatomegaly with right upper quadrant pain
- Jaundice (occasional)
- Aseptic meningitis/encephalitis occurs in approximately 1% of acute and chronic Q fever cases.
Causes
Coxiella burnetii, a pleomorphic coccobacillus that is much smaller than other rickettsias, is the etiologic agent of Q fever. Humans are infected by inhalation of C burnetii. Why chronic Q fever develops in certain patients is unknown.
- Q fever is extremely virulent; 1 bacterium can cause infection.
- Q fever is extremely resistant to inactivation; it can survive for months in dust and feces particles.
- Tick species, naturally infected, infect domestic and small mammals (eg, cats).
- C burnetii localizes in the mammary glands, uterus, and feces of these animals. Exposure to feces can lead to disease.
- Laboratory outbreaks have occurred. Only 1 case of documented human-to-human transmission exists.
- C burnetii exists in 2 antigenic states, phase I (virulent) and phase II (avirulent).
Endocarditis
Hepatitis
Legionnaires Disease
Mononucleosis
Pneumonia, Mycoplasma
Pneumonia, Viral
Tick-Borne Diseases, Colorado
Tick-Borne Diseases, Ehrlichiosis
Tick-Borne Diseases, Lyme
Tick-Borne Diseases, Q Fever
Tick-Borne Diseases, Relapsing Fever
Tick-Borne Diseases, Rocky Mountain Spotted Fever
Tick-Borne Diseases, Tularemia
Lab Studies
- Acute Q fever
- Complete blood count is normal in 70% of patients with acute Q fever, but an elevated white blood count (WBC) is present in 30% of patients.
- Liver function tests often show a slight elevation (2-3 times) of transaminases, but bilirubin usually is normal.
- Serologic tests are not helpful acutely but later may confirm diagnosis. The indirect immunofluorescence assay (IFA) is the most dependable and widely used serologic method. ELISA tests are also becoming more widely available. A 4-fold rise in complement-fixing antibody titer against phase II antigen occurs. This requires a baseline sample and another sample in 3-4 weeks.
- Chronic Q fever
- Serologic testing
- Complement-fixing antibody titer against phase I antigen of greater than or equal to 1:200, or phase I immunoglobulin A (IgA) or immunoglobulin G (IgG) greater than or equal to phase II titer
- Culture-negative endocarditis
- Granulomas on liver biopsy
- Cerebral spinal fluid findings are consistent with aseptic meningitis (eg, elevated WBC with mainly monocytes, elevated protein, normal glucose, negative Gram stain)
Imaging Studies
- Acute Q fever
- The chest radiograph is variable (normal-to-widespread pneumonitis).
- The hepatic ultrasonogram may show granulomatous hepatitis.
- Chronic Q fever
- The echocardiogram most commonly depicts endocarditis of aortic valve. Abnormal and prosthetic valves are also prone to being affected.
- The hepatic ultrasonogram shows granulomatous hepatitis.
Procedures
- Perform lumbar puncture if Q fever meningoencephalitis is suspected.
Emergency Department Care
- Recognize disease and start appropriate antibiotic therapy.
- Provide supportive care.
Consultations
Consult infectious disease, especially if chronic Q fever is suspected.
Antibiotics are necessary for acute and chronic Q fever. However, in chronic Q fever, exact antibiotics recommended for use are in a state of flux. Consult infectious disease prior to starting antibiotics in this situation. Best studied are combinations of doxycycline plus an additional antibiotic (eg, fluoroquinolone, rifampin, trimethoprim-sulfamethoxazole).
Drug Category: Antibiotics
Therapy must cover all likely pathogens in the context of the clinical setting.
| Drug Name | Doxycycline (Doryx, Bio-Tab, Vibramycin) |
| Description | Interferes with bacterial cell wall synthesis during active multiplication, causing cell wall death, resulting in bactericidal activity against susceptible bacteria. For severe cases, administer IV; for outpatients, PO is preferred. |
| Adult Dose | 100 mg PO q12h |
| Pediatric Dose | <8 years: Not recommended > 8 years: 3 mg/kg/d PO q12h; not to exceed 200 mg/d |
| Contraindications | Documented hypersensitivity; severe hepatic dysfunction |
| Interactions | Bioavailability decreases with antacids containing aluminum, calcium, magnesium, iron, or bismuth subsalicylate; tetracyclines can increase hypoprothrombinemic effects of anticoagulants; tetracyclines can decrease effects of oral contraceptives, causing breakthrough bleeding and increased risk of pregnancy |
| Pregnancy | D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
|
| Precautions | Photosensitivity may occur with prolonged exposure to sunlight or tanning equipment; reduce dose in renal impairment; consider drug serum level determinations in prolonged therapy; tetracycline use during tooth development (last one half of pregnancy through age 8 y) can cause permanent discoloration of teeth; Fanconilike syndrome may occur with outdated tetracyclines |
| Drug Name | Chloramphenicol (Chloromycetin) |
| Description | Binds to 50S bacterial-ribosomal subunits and inhibits bacterial growth by inhibiting protein synthesis; is effective against gram-negative and gram-positive bacteria. In severe cases, administer IV; for outpatients, administer PO. |
| Adult Dose | 500-750 mg PO/IV q6h; not to exceed 4 g/d |
| Pediatric Dose | 50 mg/d PO/IV divided qid |
| Contraindications | Documented hypersensitivity |
| Interactions | Concurrently with barbiturates, chloramphenicol serum levels may decrease, while barbiturate levels may increase, causing toxicity; manifestations of hypoglycemia may occur with sulfonylureas; rifampin may reduce serum chloramphenicol levels, presumably through hepatic enzyme induction; may increase effects of anticoagulants; may increase serum hydantoin levels, possibly resulting in toxicity; chloramphenicol levels may be increased or decreased |
| Pregnancy | C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
|
| Precautions | Use only for indicated infections or as prophylaxis for bacterial infections; serious and fatal blood dyscrasias (aplastic anemia, hypoplastic anemia, thrombocytopenia, granulocytopenia) can occur; evaluate baseline and perform periodic blood studies approximately q2d while in therapy; discontinue upon appearance of reticulocytopenia, leukopenia, thrombocytopenia, anemia, or findings attributable to chloramphenicol; adjust dose in liver or kidney dysfunction; caution in pregnancy at term or during labor because of potential toxic effects on fetus (gray syndrome) |
Further Inpatient Care
- Valve replacement may be required, depending on cardiac status.
Further Outpatient Care
- For acute Q fever, antibiotic therapy should last 10-14 days.
- For chronic Q fever, antibiotic therapy lasts a minimum of 2 years.
Deterrence/Prevention
- Identify infections in domesticated animal populations.
- Birthing should take place in indoor facilities.
- Properly dispose of placentae, fetal membranes, and aborted material.
- Human Q fever vaccine is available in Australia and Eastern Europe but not in the US.
Prognosis
- Acute Q fever, if properly diagnosed and treated, has very low morbidity and mortality.
- Chronic Q fever requires long-term antibiotic therapy including close follow-up care with an infectious disease specialist.
Patient Education
Medical/Legal Pitfalls
- Obtain an occupational history from patients.
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CBRNE - Q Fever excerpt Article Last Updated: Sep 25, 2008
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