Brill-Zinsser disease is a relapse of epidemic typhus, which preserves the clinical manifestations of the primary disease, but proceeds mainly in a mild form. A characteristic clinical symptom is an abundant roseolous-petechial rash on the skin, mucous membranes. Systemic manifestations are fever, changes in consciousness, a tendency to tachycardia and hypotension. Confirmatory diagnostic methods include the detection of antibodies to typhoid rickettsia and the pathogen itself in the body. Treatment of recurrent typhus is carried out with the help of etiotropic antibiotics, as well as symptomatic drugs.
ICD 10
A75.1 Recurrent typhus [Brill-Zinsser disease]
General information
Brill’s disease (Brill-Zinsser disease, recurrent typhus) is an acute infectious disease that occurs with damage to the skin, mucous membranes, less often cardiovascular, nervous systems. The disease was first described in 1910 by the American physician N. Brill and supplemented in 1934 by the epidemiologist H. Zinsser. Scientists associated the symptoms of pathology with previously transferred typhus, which in ser. The 20th century was confirmed by the discovery of rickettsias in the lymph nodes of those who died from a relapse of typhus, first suffered more than 20 years before death. The disease has no clear seasonality, is sporadic, does not imply mandatory recent contact with lice or patients with pediculosis.
Causes
The causative agents of infection are Provacek’s rickettsias, which are located in the lymph nodes and other organs of a person who has suffered from epidemic typhus in the past. Rickettsia are obligate intracellular microorganisms that die when boiled, using standard solutions of disinfectants. The source of infection for himself is the sick person himself, for others he is not contagious only in the absence of lice. In the presence of lice, a patient with Brill-Zinsser disease can infect others with epidemic typhus with the help of vector-borne transmission of infection.
Traditionally, only people over 45 years of age were included in the risk group, but due to the high prevalence of HIV infection in the world, patients with the immunodeficiency virus may be more susceptible to Brill-Zinsser disease than the general population. The trigger factors of relapse are usually acute respiratory infections, exhaustion, prolonged stressful situations, hypothermia, radiation exposure, exacerbations of chronic diseases, injuries, climate change, work in hazardous production, severe immunodeficiency conditions, etc. Children under 18 do not suffer from this pathology.
Pathogenesis
After suffering epidemic lice typhus, rickettsias persist for a long time in lymph nodes, parenchymal organs (most often in the liver, kidneys) and lungs. An important role in the pathogenesis of recurrent infection is played by the number of rickettsias (usually insignificant), which correlates with the severity of clinical and morphological manifestations. In the presence of appropriate conditions, rickettsias penetrate into the systemic circulation, while the main target for the pathogen is the vascular endothelium. Rickettsia multiplies in endotheliocytes, inflammatory changes can capture the entire thickness of the vessel, leading to necrosis of the vascular wall. However, specific typhoid granulomas (Popov nodules) are rarely formed.
The disease is characterized by so-called warty endocarditis with damage to the heart valves. As a result of a circular or segmental vascular lesion, blood clots are formed, with the localization of thrombosis (or thromboembolism) in the vessels of the brain, ischemic damage to the nervous tissue is possible. Vascular lesions cause skin manifestations of the disease. An important feature of pathology is non-sterile immunity, therefore, with complete elimination of the pathogen, a new infection is possible. However, there are practically no repeated cases of this pathology in the world. This is probably due to the morphological features of rickettsias, their intracellular parasitism, which allows them to be inaccessible to the cells of the body’s immune system for a long time.
Symptoms
The incubation period of the disease lasts in some cases from 3 to 50 years. The first clinical manifestations may begin 5-7 days after exposure to the provoking factor. The onset of relapse is acute, with a sharp rise in body temperature to high numbers (above 39 ° C), chills, pronounced weakness, fatigue, severe headache, decreased appetite. Patients are excited, sleep disorders, euphoria, depersonalization, hyperesthesia of the ocular, auditory, olfactory and other analyzers may occur. There is a decrease in blood pressure (below 120/80 mm Hg). There is an unexpressed hyperemia of the face, injection of the vessels of the sclera, from the first day spots of Chiari-Avtsyn (single red-orange elements with a fuzzy contour on the conjunctiva, cartilage of the upper eyelid) and Rosenberg’s enanthema (small petechiae on the shell of the soft palate, the arches of the tonsils) are determined.
From 3-4 days of the onset of relapse, the appearance of a roseolous-petechial rash is noted on the skin, mainly concentrated on the body, the lateral surfaces of the trunk, upper extremities; much less often elements are found on the face, palms and soles. The rash does not cause itching, peeling, passes without a trace (roseoli) or leaves areas of depigmentation (petechiae). An unfavorable prognostic sign is the predominance of petechial rashes, however, with this nosology, this is extremely rare, as is the course without the appearance of exanthema. An infrequent, but formidable symptom is inflammation of the substance of the brain, manifested by a violation of consciousness, paralysis, paresis.
Complications
Early treatment for medical help, correct diagnosis, timely treatment allow you to transfer the disease without complications. Patients with chronic systemic diseases are most often susceptible to the development of moderate and complicated forms of Brill-Zinsser disease. The most serious consequences of relapse of rickettsiosis are thromboembolic syndrome, thrombophlebitis, infectious and toxic shock, secondary bacterial infections (hypostatic pneumonia, pyelonephritis, sepsis). Purulent complications are possible due to invasive medical therapeutic and diagnostic manipulations (intravenous infusions, vascular catheterization, intramuscular injections and others).
Diagnostics
To confirm or exclude the diagnosis of recurrent typhus, consultation of an infectious disease specialist, cardiologist, dermatovenerologist is required. If signs of central nervous system damage appear, a neurologist’s examination is required. Diagnosis of nosology is carried out by carefully collecting an epidemiological history, correct interpretation of examination data, results of laboratory and instrumental studies:
- Physical examination. In an objective examination of the patient, moderate psychomotor agitation attracts attention, sometimes tremor of the fingers of the hands and push–like pushing of the tongue forward (Govorov-Godelier symptom). Roseolous and petechial rashes are observed on the skin, conjunctiva of the eyes, soft palate. Palpation of the abdomen can detect an increase in the liver, spleen (hepatosplenomegaly). Positive symptoms of tourniquet and pinching are characteristic (artificial rash causing with skin compression).
- Blood and urine tests. In the general clinical analysis, the presence of neutrophilic leukocytosis with a rod-shaped shift to the left, lymphocytopenia, eosinophilopenia, acceleration of ESR is noted. Biochemical studies show an increase in creatinine, hyperglobulinemia, and hypoalbuminemia. In the general analysis of urine – an increase in density, albuminuria, cylindruria, less often microhematuria.
- Identification of infectious agents. Mandatory for diagnosis is a serological blood test (ELISA, RSC) with the detection of class G immunoglobulins (IgG) to Provacek rickettsia. The presence of the pathogen in the body can be proved by PCR. Bacteriological examination of blood is a time-consuming and expensive process, if necessary, it is carried out only in laboratories of particularly dangerous infections.
- Cardiodiagnostics. Electrocardiography reveals tachycardia, signs of myocardial hypoxia, less often – insufficiency of the affected valves. With ECHO-CG, vegetations with a shaded contour, an inhomogeneous echographic signal, thickening of valve flaps, sometimes the formation of valve insufficiency, aggravation of regurgitation are detected.
Differential diagnosis is carried out with endemic typhus, rubella, measles, syphilis, sepsis, Rocky Mountain spotted fever, influenza, typhoid fever, paratyphs A and B, hemorrhagic fever with renal syndrome, meningococcal infection (meningococcemia and meningococcal meningitis), leptospirosis. Taking into account the heart lesions caused by both the relapse of typhus itself and often concomitant cardiac pathology, it is necessary to differentiate the disease with infectious endocarditis, rheumatic heart defects, cardiomyopathies. Psychomotor agitation or inhibition, as well as possible episodes of hallucinations, delusions may be caused by alcohol, narcotic intoxication or be the debut of mental disorders or senile dementia.
Treatment
Patients with suspected of this pathology are subject to inpatient treatment. Bed rest mode, it is necessary to maintain a predominantly horizontal position until 5-6 days of normal temperature. Then short–term movements are allowed under the supervision of medical workers, then independently within the department. Regular monitoring of blood pressure is necessary. The use of compression knitwear for the prevention of deep vein thrombosis of the lower extremities and PE may have some effectiveness. It is recommended to increase the intake of liquids, adhere to a diet with the exception of alcohol, fried and fatty dishes.
Etiotropic drugs for the treatment of Brill-Zinsser disease are tetracycline, rifamycin antibiotics, as well as macrolides (for example, doxycycline, tetracycline, rifampicin or azithromycin). The use of vitamins C and P with vasoprotective properties is pathogenetically justified. Symptomatic therapy includes antipyretic, detoxifying drugs (chlosol, acesol, Hartman’s, Ringer’s solution, glucose-salt and succinate-containing infusion solutions), sedatives (barbiturates, diazepam and others). All patients, especially the elderly, are recommended to use anticoagulants (heparin).
Prognosis and prevention
The prognosis of the condition is most often favorable, the mortality rate is 1-2%. The course of infection can be influenced by comorbid conditions and the age of the patient, most often the febrile stage of the disease lasts about 7-8 days, full recovery takes up to 2-3 weeks. About 70% of cases of the disease occur in mild or moderate form.
Specific prevention of the disease consists in the use of vaccines for epidemiological indications. The most common practice is vaccination with a live typhoid vaccine and its analogue – a dry chemical containing killed rickettsias. The use of specific prophylaxis is indicated for persons who are temporarily or permanently in contact with patients with pediculosis and typhus, as well as living together with a patient with Brill-Zinsser disease in unfavorable sanitary and epidemiological conditions.
The main method of nonspecific prevention of pathology is the fight against lice (preventive examinations in educational institutions and work collectives), sanitary and educational work with the population, instilling hygienic skills in children. A full-fledged disinfection of places of residence, clothing, personal belongings of patients with pediculosis is necessary. Early detection, isolation, rational antibiotic therapy of patients with epidemic typhus is important.