Consensus statement ularemia, a bacterial zoono

Clinical Manifestations

The primary clinical forms of tulare- mia vary in severity and presentation according to virulence of the infecting organism, dose, and site of inoculum. Primary disease presentations include ulceroglandular, glandular, oculoglan- dular, oropharyngeal, pneumonic, ty- phoidal, and septic forms.19,20,49,70,72,74,75 The term typhoidal tularemia has been used to describe illness in tularemia pa- tients with systemic infections mani- festing as fever and other constitu- tional signs without cutaneous or mucosal membrane lesions or re- gional lymphadenitis. Sometimes, these patients present with prominent gas- trointestinal manifestations, such as di- arrhea and pain. Confusion is created when typhoidal tularemia is used to de- scribe the illness in patients infected by

inhalation, especially when there are signs of pleuropneumonic disease; this usage can be misleading and has been discouraged.54,75

The onset of tularemia is usually

abrupt, with fever (38°C-40°C), head- ache, chills and rigors, generalized body aches (often prominent in the low back), coryza, and sore throat. A pulse- temperature dissociation has been noted in as many as 42% of patients.49 A dry or slightly productive cough and sub- sternal pain or tightness frequently oc- cur with or without objective signs of pneumonia, such as purulent sputum, dyspnea, tachypnea, pleuritic pain, or hemoptysis.7,19,26,70,74 Nausea, vomit- ing, and diarrhea sometimes occur. Sweats, fever and chills, progressive weakness, malaise, anorexia, and weight loss characterize the continuing ill- ness. Studies of volunteers have shown that F tularensis aerosol exposures can incapacitate some persons in the first 1 or 2 days of illness, and significant im- pairment in performing tasks can con- tinue for days after antibiotic treat- ment is begun.76 In untreated tularemia, symptoms often persist for several weeks and, sometimes, for months, usu- ally with progressive debility. Any form of tularemia may be complicated by he- matogenous spread, resulting in sec- ondary pleuropneumonia, sepsis, and, rarely, meningitis.74,77

Prior to the advent of antibiotics, the

overall mortality from infections with the more severe type A strains was in the range of 5% to 15%, and fatality rates as high as 30% to 60% were re- ported for untreated pneumonic and se- vere systemic forms of disease.72,78 Cur- rently, the overall case-fatality rate of reported cases in the United States is less than 2%.34,49 Type B infections are rarely fatal.

In ulceroglandular tularemia, the

form that typically arises from han- dling a contaminated carcass or follow- ing an infective arthropod bite, a local cutaneous papule appears at the inocu- lation site at about the time of onset of generalized symptoms, becomes pus- tular, and ulcerates within a few days of its first appearance. The ulcer is ten-

der, generally has an indolent charac- ter, and may be covered by an eschar. Typically, one or more regional affer- ent lymph nodes may become en- larged and tender within several days of the appearance of the papule. Even with antibiotic treatment, the affected nodes may become fluctuant and rup- ture. In oculoglandular tularemia, which follows direct contamination of the eye, ulceration occurs on the conjunctiva, accompanied by pro- nounced chemosis, vasculitis, and regional lymphadenitis. Glandular tu- laremia is characterized by lymphade- nopathy without an ulcer.

Oropharyngeal tularemia is acquired

by drinking contaminated water, ingest- ing contaminated food, and, some- times, by inhaling contaminated drop- lets or aerosols.14,20,36,43,50,51,79 Affected persons may develop stomatitis but more commonly develop exudative pharyn- gitis or tonsillitis, sometimes with ul- ceration. Pronounced cervical or retro- pharyngeal lymphadenopathy may occur (FIGURE 1).74,79

Tularemia pneumonia can be the

direct result of inhaling contaminated aerosols or be secondary to hematog- enous spread from a distal site. An aerosol release of F tularensis would be expected to result in acute illness with signs and symptoms of 1 or more of pharyngitis, bronchiolitis, pleuropneu- monitis, and hilar lymphadenitis, accompanied by various manifesta-