Case Report of Acute Q Fever with Hepatitis Progressing to Chronic Q Fever with Endocarditis.

A 35-year-old male greenhouse worker presented with myalgia, fatigue, and fever. Initially, he was thought to have an unspecified viral infection and was treated with conservative therapy. However, the patient's symptoms persisted, and he reported additional symptoms of mild abdominal pain and headaches. Laboratory evaluation was significant for elevated liver enzymes. Due to concern for acute hepatitis and persistent fever the patient was hospitalized. During his hospital course, no infectious etiology was found to explain his symptoms. After discharge from the hospital, additional testing showed positive serology for Q fever IgG phase II antibody (1:8192) and phase II antibody IgM (>1:2048). He was treated with doxycycline and had a good clinical response. Upon follow-up, he had worsening Phase I IgG serologies. Transesophageal echo demonstrated vegetations consistent with endocarditis.

Prerequisites, barriers and opportunities in care for Q-fever patients: a Delphi study among healthcare workers.

BACKGROUND: Q-fever is a zoonotic disease that can lead to illness, disability and death. This study aimed to provide insight into the perspectives of healthcare workers (HCWs) on prerequisites, barriers and opportunities in care for Q-fever patients. METHODS: A two-round online Delphi study was conducted among 94 Dutch HCWs involved in care for Q-fever patients. The questionnaires contained questions on prerequisites for high quality, barriers and facilitators in care, knowledge of Q-fever, and optimization of care. For multiple choice, ranking and Likert scale questions, frequencies were reported, while for rating and numerical questions, the median and interquartile range (IQR) were reported. RESULTS: The panel rated the care for Q-fever patients at a median score of 6/10 (IQR = 2). Sufficient knowledge of Q-fever among HCWs (36%), financial compensation of care (30%) and recognition of the disease by HCWs (26%) were considered the most important prerequisites for high quality care. A lack of knowledge was identified as the most important barrier (76%) and continuing medical education as the primary method for improving HCWs' knowledge (76%). HCWs rated their own knowledge at a median score of 8/10 (IQR = 1) and the general knowledge of other HCWs at a 5/10 (IQR = 2). According to HCWs, a median of eight healthcare providers (IQR = 4) should be involved in the care for Q-fever fatigue syndrome (QFS) and a median of seven (IQR = 5) in chronic Q-fever care. CONCLUSIONS: Ten years after the Dutch Q-fever epidemic, HCWs indicate that the long-term care for Q-fever patients leaves much room for improvement. Facilitation of reported prerequisites for high quality care, improved knowledge among HCWs, clearly defined roles and responsibilities, and guidance on how to support patients could possibly improve quality of care. These prerequisites may also improve care for patients with persisting symptoms due to other infectious diseases, such as COVID-19.

Rare case of chronic Q fever myocarditis in end stage heart failure patient: A case report.

BACKGROUND: Q fever myocarditis is a rare disease manifestation of Q fever infection caused by Coxiella burnetii. It is associated with significant morbidity and mortality if left untreated. Prior studies have reported myocarditis in patients with acute Q fever. We present the first case of chronic myocarditis in an end-stage heart failure patient with chronic Q fever infection. CASE SUMMARY: A 69-year-old male was admitted with dyspnea on exertion, hypotension and bilateral lower extremity edema for a few months. He has a past medical history of ischemic cardiomyopathy with left ventricular ejection fraction of 25%, implantable cardioverter defibrillator in place, bioprosthetic aortic valve and mitral valve replacement. He continued to have shortness of breath despite diuresis along with low grade fevers. Initial infectious work up came back negative. On further questioning, the patient was found to have close contact with farm animals and the recurrent fevers prompted the work-up for Q fever. Q fever serologies and cardiac positron emission tomography confirmed the diagnosis of chronic Q fever myocarditis. He was then successfully treated with doxycycline and hydroxychloroquine for 18 mo. CONCLUSION: Chronic Q fever myocarditis, if left untreated, carries a poor prognosis. It should be kept in differentials, especially in patients with recurrent fevers and contact with farm animals.

A zoonotic cause of blood culture-negative infective endocarditis in Belgium: Case report and review of the literature on Q fever.

Q fever is a worldwide zoonotic infection caused by Coxiella burnetii. In Belgium, the disease must be notified, and the incidence is low. Human contamination is mostly due to sheep and goats. Herein, we report a case of chronic Q fever presenting as a prolonged fever in a patient with a history of valve prosthesis. Blood culture-negative endocarditis was diagnosed through an assessment including echocardiography and systematic serological testing. Despite the absence of travel abroad or obvious contact with domestic or wildlife animals, C. burnetii phase I and phase II IgG antibody titers were > 1:8192, and polymerase chain reaction performed on blood was positive for C. burnetii. Genotypic single nucleotide polymorphism (SNP) analysis of the pathogen strain identified a SNP-type 1 genomic group, which is associated with small ruminants in Belgium. The epidemiological investigation did not confirm the presence of positive C. burnetii cattle or sheep herds in the vicinity of the patient's workplace and home, nor in the pest animals surrounding the workplace. Patients with risk factors for chronic Q fever should be tested for C. burnetii infection in case of prolonged fever of unknown origin, osteomyelitis, abscess or blood culture-negative endocarditis, even in the absence of direct exposure to animals.

Targeted Screening for Chronic Q Fever, the Netherlands.

Early detection of and treatment for chronic Q fever might prevent potentially life-threatening complications. We performed a chronic Q fever screening program in general practitioner practices in the Netherlands 10 years after a large Q fever outbreak. Thirteen general practitioner practices located in outbreak areas selected 3,419 patients who had specific underlying medical conditions, of whom 1,642 (48%) participated. Immunofluorescence assay of serum showed that 289 (18%) of 1,642 participants had a previous Coxiella burnetii infection (IgG II titer >1:64), and 9 patients were suspected of having chronic Q fever (IgG I y titer >1:512). After medical evaluation, 4 of those patients received a chronic Q fever diagnosis. The cost of screening was higher than estimated earlier, but the program was still cost-effective in certain high risk groups. Years after a large Q fever outbreak, targeted screening still detected patients with chronic Q fever and is estimated to be cost-effective.

Fluorescence in situ hybridization for detecting Coxiella burnetii in tissue samples from chronic Q fever patients.

OBJECTIVE: Detection of the intracellular bacterium Coxiella burnetii, causative agent of chronic Q fever, is notoriously difficult. Diagnosis of and duration of antibiotic treatment for chronic Q fever is partly determined by detection of the bacterium with polymerase chain reaction (PCR). Fluorescence in situ hybridization (FISH) might be a promising technique for detecting C. burnetii in tissue samples from chronic Q fever patients, but its value in comparison with PCR is uncertain. We aim to assess the value of FISH for detecting C. burnetii in tissue of chronic Q fever patients. METHODS: FISH and PCR were performed on tissue samples from Dutch chronic Q fever patients collected during surgery or autopsy. Sensitivity, specificity, and overall diagnostic accuracy were calculated. Additionally, data on patient and disease characteristics were collected from electronic medical records. RESULTS: In total, 49 tissue samples from mainly vascular walls, heart valves, or placentas, obtained from 39 chronic Q fever patients, were examined by FISH and PCR. The sensitivity and specificity of FISH compared to PCR for detecting C. burnetii in tissue samples from chronic Q fever patients was 45.2% (95% confidence interval (CI), 27.3% - 64.0%) and 84.6% (95% CI, 54.6% - 98.1%), respectively. The overall diagnostic accuracy was 56.8% (95% CI, 42.2% - 72.3%). Two C. burnetii PCR negative placentas were FISH positive. Four FISH results (8.2%) were deemed inconclusive because of autofluorescence. CONCLUSION: With an overall diagnostic accuracy of 57.8%, we conclude that FISH has limited value in the routine diagnostics of chronic Q fever.

Open repair of a Coxiella burnetii-associated abdominal aortic endovascular stent graft infection with a cryopreserved allograft using visceral artery pump perfusion.

Coxiella burnetii, the causative organism of Q fever, has been increasingly reported to be associated with infections of abdominal aortic aneurysms and endovascular stent grafts. We have added to the current literature by presenting a case of the surgical management of chronic Q fever that had infected a prior aortic endovascular stent graft placed for a contained rupture of an infrarenal aortic aneurysm in a 68-year-old woman. We presented our case of the surgical management of the excision and explantation of the infected aorta and stent graft, with reconstruction of the aorta using a cryopreserved aortic graft and visceral artery pump perfusion.

Incidence of chronic Q fever and chronic fatigue syndrome: A 6 year follow-up of a large Q fever outbreak.

Acute Q fever is a generally self-limiting infection caused by the intracellular gram-negative bacterium Coxiella burnetii. For yet unknown reasons, a subset of patients develops chronic infection. Furthermore, chronic fatigue syndrome (CFS) as post-acute Q fever sequelae has been described. We here investigated the rates of chronic Q fever and incidences of CFS 6 years after one of the largest European Q fever outbreaks that occurred in Jena, Germany in 2005 with 331 reported cases, who lived in proximity of a grazing flock of sheep. A total of 80 patients and their 52 non-diseased household members from the former outbreak, were enrolled 6 years after the outbreak. Blood samples were collected and tested for chronic Q fever which was determined by seroprevalence using referenced immunofluorescence assays. Also, the presence of CFS was assessed using the Short Form Symptom Inventory developed by the Centers (United States) for Disease Control and Prevention (SF CDC- SI). In 80 out of 132 (60.6%) study participants, previous Q fever infection was confirmed serologically, while no previous infection was detected in the 52 household members. None of the participants fulfilled the serological criteria of chronic Q fever. The evaluation of the CDC-SI did not show any differences between the two groups. Also, there was no difference between both groups regarding fulfillment of CFS-defining criteria (n = 3 (3.8%; sero-positive) versus n = 2 (3.8%; sero-negative), p = 0.655). Our 6-year follow-up study of a large Q fever outbreak did not find evidence of chronic Q fever or post Q fever CFS. There was no asymptomatic sero-positivity in household members of Q fever patients.

Polymicrobial Infections Among Patients with Vascular Q Fever, France, 2004-2020.

We report 5 cases of vascular Q fever complicated by polymicrobial superinfection in patients who had no risk factors for acute Q fever. Q fever was diagnosed by serologic and molecular assays for Coxiella burnetii. We confirmed additional infections using conventional graft cultures.

Still New Chronic Q Fever Cases Diagnosed 8 Years After a Large Q Fever Outbreak.

BACKGROUND: Chronic Q fever usually develops within 2 years after primary infection with Coxiella burnetii. We determined the interval between acute Q fever and diagnosis of chronic infection, assessed what factors contribute to a longer interval, and evaluated the long-term follow-up. METHODS: From 2007 to 2018, patients with chronic Q fever were included from 45 participating hospitals. The interval between acute and chronic infection was calculated in patients with a known day of first symptoms and/or serological confirmation of acute Q fever. Chronic Q fever-related complications and mortality were assessed by 2 investigators based on predefined criteria. RESULTS: In total, 313 (60.3%) proven, 81 (15.6%) probable, and 125 (24.1%) possible chronic Q fever patients were identified. The date of acute Q fever was known in 200 patients: in 45 (22.5%), the interval was longer than 2 years, with the longest observed interval being 9.2 years. Patients in whom serological follow-up was performed after acute Q fever were diagnosed less often after this 2-year interval (odds ratio, 0.26; 95% confidence interval, 0.12-0.54). Chronic Q fever-related complications occurred in 216 patients (41.6%). Chronic Q fever-related mortality occurred in 83 (26.5%) of proven and 3 (3.7%) of probable chronic Q fever patients. CONCLUSIONS: Chronic Q fever is still being diagnosed and mortality keeps occurring 8 years after a large outbreak. Intervals between acute Q fever and diagnosis of chronic infection can reach more than 9 years. We urge physicians to perform microbiological testing for chronic Q fever even many years after an outbreak or acute Q fever disease.

The prognostic value of serological titres for clinical outcomes during treatment and follow-up of patients with chronic Q fever.

OBJECTIVES: We assessed the prognostic value of phase I IgG titres during treatment and follow-up of chronic Q fever. METHODS: We performed a retrospective cohort study to analyse the course of phase I IgG titres in chronic Q fever. We used a multivariable time-varying Cox regression to assess our primary (first disease-related event) and secondary (therapy failure) outcomes. In a second analysis, we evaluated serological characteristics after 1 year of therapy (fourfold decrease in phase I IgG titre, absence of phase II IgM and reaching phase I IgG titre of ≤1:1024) with multivariable Cox regression. RESULTS: In total, 337 patients that were treated for proven (n = 284, 84.3%) or probable (n = 53, 15.7%) chronic Q fever were included. Complications occurred in 190 (56.4%), disease-related mortality in 71 (21.1%) and therapy failure in 142 (42.1%) patients. The course of phase I IgG titres was not associated with first disease-related event (HR 1.00, 95% CI 0.86-1.15) or therapy failure (HR 1.02, 95% CI 0.91-1.15). Similar results were found for the serological characteristics for the primary (HR 0.97, 95% CI 0.62-1.51; HR 1.12, 95% CI 0.66-1.90; HR 0.99, 95% CI 0.57-1.69, respectively) and secondary outcomes (HR 0.86, 95% CI 0.57-1.29; HR 1.37, 95% CI 0.86-2.18; HR 0.80, 95% CI 0.48-1.34, respectively). DISCUSSION: Coxiella burnetii serology does not reliably predict disease-related events or therapy failure during treatment and follow-up of chronic Q fever. Alternative markers for disease management are needed, but, for now, management should be based on clinical factors, PCR results, and imaging results.

Single nucleotide polymorphism (SNP) rs3751143 in P2RX7 is associated with therapy failure in chronic Q fever while rs7125062 in MMP1 is associated with fewer complications.

OBJECTIVES: Chronic Q fever is a persistent infection with the intracellular bacterium Coxiella burnetii. Development of chronic Q fever is associated with single nucleotide polymorphisms (SNPs) in genes encoding for pattern recognition receptors, for phagolysosomal pathway components and for matrix metalloproteinases (MMPs). We evaluated the association of SNPs in these innate-immunity and MMP genes with clinical outcomes. METHODS: SNPs were selected from previous association studies and analysed in a cohort of patients with chronic Q fever. The primary outcome was all-cause mortality; secondary outcomes were therapy failure and chronic Q fever-related complications. Subdistribution hazard ratios (SHR) were calculated. RESULTS: Nineteen SNPs were analysed in 134 patients with proven and 29 with probable chronic Q fever. In multivariable analysis, none of the selected SNPs was associated with all-cause mortality. However, SNP rs3751143 located in P2RX7 appeared to be associated with therapy failure (SHR 2.42; 95% confidence interval, 1.16-5.05; p 0.02), which is in line with other reports, showing that a loss of function of the P2X7 receptor leads to inefficient killing of intracellular organisms. In addition, SNP rs7125062 located in MMP1, involved in the cleavage of extracellular matrix, was associated with fewer chronic Q fever-related complications such as acute aneurysms (SHR 0.49; 95% confidence interval, 0.29-0.83; p 0.008). CONCLUSIONS: A polymorphism in P2RX7, known to lead to loss of function of the receptor and inefficient killing of intracellular organisms, and a polymorphism in MMP1 were respectively associated with more therapy failures and fewer complications such as acute aneurysms in patients with chronic Q fever.

A systematic review on the health outcomes associated with non-endocarditis manifestations of chronic Q fever.

The aim of this study was to systematically review the non-endocarditis manifestations of chronic Q fever and understand the significance of non-specific symptoms like pain and fatigue in chronic endovascular, osteomyelitis and abscess due to chronic Q fever. We performed a systematic review using Pub Med (the National Library of Medicine (NLM)) and Scopus databases. All studies in English on chronic Q fever that listed clinical manifestations other than infective endocarditis (IE) and chronic fatigue syndrome (CFS). Meta-analysis was carried out to investigate the effects of patient's health outcomes (pain, fatigue, the need for surgery and mortality) on vascular infections, osteomyelitis and abscess. Among cases not presenting as IE or CFS, vascular infections and osteomyelitis were the most common chronic Q fever disease manifestations. There were distinct regional patterns of disease. Compared with infective endocarditis, these are significantly associated with increased risk of pain: osteomyelitis (relative risk (RR) = 4.13, 95% confidence interval (CI) 3.36-5.07), abscess (RR = 3.59, 95% CI 3.28-3.93) and vascular infection (RR = 2.46, 95% CI 1.99-3.03). The strongest significant association was observed between osteomyelitis and pain. There was no significant association between fatigue and these manifestations. Clinicians have to be aware of uncommon manifestations of chronic Q fever as they present with non-specific symptoms and are significantly associated with increased risk of morbidity and mortality. The findings emphasise the need to investigate patients with positive chronic Q fever serology presenting with acute or chronic pain for possible underlying complications.

Chronic Q fever associated with systemic sclerosis.

BACKGROUND: After the Q fever outbreak in the Netherlands between 2007 and 2010, more than 300 patients with chronic Q fever have been identified. Some patients were also diagnosed with systemic sclerosis, a rare immune-mediated disease. We aimed to increase awareness of concomitant chronic Q fever infection and systemic sclerosis and to give insight into the course of systemic sclerosis during persistent Q fever infection. MATERIALS AND METHODS: Chronic Q fever patients were identified after the Dutch Q fever outbreak in 2007-2010. Systemic sclerosis was diagnosed by a scleroderma expert and patients fulfilled the 2013 Classification Criteria for Systemic Sclerosis. RESULTS: Four cases presented with chronic Q fever, persistent Coxiella burnetii infection, shortly preceded or followed by the diagnosis of limited cutaneous systemic sclerosis. The three male patients of 60 years or older developed a relatively mild systemic sclerosis, which did not require immunosuppressive therapy during adequate treatment of the chronic Q fever infection. The 58-year-old female patient used immunosuppressives for her newly diagnosed systemic sclerosis at the time she likely developed a chronic Q fever infection. CONCLUSIONS: In this case series, chronic Q fever preceding systemic sclerosis was associated with a mild course of systemic sclerosis without the necessity of immunosuppressive drugs, while chronic Q fever development due to immunocompromised state was associated with a more deteriorating course of systemic sclerosis.

Explaining the long-term impact of chronic Q fever and Q fever fatigue syndrome on psychosocial functioning: A comparison with diabetes and the general population.

OBJECTIVE: After Q fever infection, 1-5% of patients develop chronic Q fever, while about 20% develops Q fever fatigue syndrome (QFS). This study examines whether these two conditions have a long-term impact on psychosocial functioning compared to the general population and patients with type 2 diabetes (DM) and investigate which mediating factors influence outcomes. METHODS: Cross-sectional study was performed, measuring psychosocial functioning including quality of life (depression and satisfaction with life), anxiety, social functioning and relationship satisfaction in patients with proven or probable chronic Q fever or QFS, 5-9 years after acute Q fever infection. Multivariate linear regression was used to analyse differences between groups, correct for confounders and identify relevant mediators (fatigue, physical or cognitive functioning, illness perception). RESULTS: Quality of life and social functioning of chronic Q-fever and QFS patients was significantly lower and anxiety significantly higher compared to DM patients and the general population. The impact was completely mediated by fatigue in both Q fever groups. Physical and cognitive functioning and illness perception partially mediated the impact. CONCLUSIONS: Health care workers need to be aware of the long-term impact of chronic Q fever and QFS on psychosocial functioning of patients in order to provide proper guidance.

Genetic variations in innate immunity genes affect response to Coxiella burnetii and are associated with susceptibility to chronic Q fever.

OBJECTIVES: Chronic Q fever is a persistent infection, mostly of aortic aneurysms, vascular prostheses or damaged heart valves, caused by the intracellular bacterium Coxiella burnetii. Only a fraction of C. burnetii-infected individuals at risk develop chronic Q fever. In these individuals, a defective innate immune response may contribute to the development of chronic Q fever. We assessed whether genetic variations in genes involved in the killing machinery for C. burnetii by macrophages, contribute to the progression to chronic Q fever. METHODS: The prevalence of 66 single nucleotide polymorphisms (SNPs) in 31 genes pivotal in phagolysosomal maturation, bacterial killing and autophagy, was determined in 173 chronic Q fever patients and 184 controls with risk factors for chronic Q fever and serological evidence of a C. burnetii infection. Associations were detected with univariate logistic regression models. To assess the effect of these SNPs on innate responses to C. burnetii, the C. burnetii-induced cytokine production and basal reactive oxygen species production of healthy volunteers was determined. RESULTS: RAB7A (rs13081864) and P2RX7 loss-of-function SNP (rs3751143) were more common in chronic Q fever patients than in controls. RAB5A (rs8682), P2RX7 gain-of-function SNP (rs1718119), MAP1LC3A (rs1040747) and ATG5 (rs2245214) were more common in controls. In healthy volunteers, RAB7A (rs13081864) and MAP1LC3A (rs1040747) influenced the C. burnetii-induced cytokine production. RAB7A (rs13081864) modulated basal reactive oxygen species production. CONCLUSIONS: RAB7A (rs13081864) and P2RX7 (rs3751143) are associated with the development of chronic Q fever, whereas RAB5A (rs8682), P2RX7 (rs1718119), MAP1LC3A (rs1040747) and ATG5 (rs2245214) may have protective effects.

Viable Coxiella burnetii Induces Differential Cytokine Responses in Chronic Q Fever Patients Compared to Heat-Killed Coxiella burnetii.

Cytokine responses of chronic Q fever patients to the intracellular bacterium Coxiella burnetii have mostly been studied using ex vivo stimulation of immune cells with heat-killed C. burnetii due to the extensive measures needed to work with viable biosafety level 3 agents. Whether research with heat-killed C. burnetii can be translated to immune responses to viable C. burnetii is imperative for the interpretation of previous and future studies with heat-killed C. burnetii Peripheral blood mononuclear cells (PBMCs) of chronic Q fever patients (n = 10) and healthy controls (n = 10) were stimulated with heat-killed or viable C. burnetii of two strains, Nine Mile and the Dutch outbreak strain 3262, for 24 h, 48 h, and 7 days in the absence or presence of serum containing anti-C. burnetii antibodies. When stimulated with viable C. burnetii, PBMCs of chronic Q fever patients and controls produced fewer proinflammatory cytokines (interleukin-6 [IL-6], tumor necrosis factor alpha, and IL-1ß) after 24 h than after stimulation with heat-killed C. burnetii In the presence of Q fever seronegative serum, IL-10 production was higher after stimulation with viable rather than heat-killed C. burnetii; however, when incubating with anti-C. burnetii antibody serum, the effect on IL-10 production was reduced. Levels of adaptive, merely T-cell-derived cytokine (gamma interferon, IL-17, and IL-22) and CXCL9 production were not different between heat-killed and viable C. burnetii stimulatory conditions. Results from previous and future research with heat-killed C. burnetii should be interpreted with caution for innate cytokines, but heat-killed C. burnetii-induced adaptive cytokine production is representative of stimulation with viable bacteria.

Autoimmunity and B-cell dyscrasia in acute and chronic Q fever: A review of the literature.

Q fever infection can lead to chronic Q fever, a potentially lethal disease occurring in 1-5% of patients infected with Coxiella burnetii, characterized by the persistence of this intracellular bacterium. It usually presents as endocarditis, infected vascular aneurysms, or infected vascular prostheses. This systematic review of the literature discusses the various autoimmune syndromes and B-cell dyscrasias in acute and chronic Q fever patients, that may interfere with or impede recognition and diagnosis of Q fever. Reportedly, high concentrations of anti-cardiolipin antibodies may be found in acute Q fever patients, while specifically cardiac muscle antibodies have been reported during chronic Q fever. Systemic lupus erythematosus and antiphospholipid syndrome are the most frequently reported autoimmune syndromes, followed by neuromuscular disorders and vasculitis. B-cell dyscrasia, mostly cryoglobulinaemia, is predominantly described in chronic Q fever patients with endocarditis. We conclude that immunological (epi)phenomena are not rare during Q fever and may obscure the infectious etiology of the disease.

Primary and secondary arterial fistulas during chronic Q fever.

OBJECTIVE: After primary infection with Coxiella burnetii, patients may develop acute Q fever, which is a relatively mild disease. A small proportion of patients (1%-5%) develop chronic Q fever, which is accompanied by high mortality and can be manifested as infected arterial or aortic aneurysms or infected vascular prostheses. The disease can be complicated by arterial fistulas, which are often fatal if they are left untreated. We aimed to assess the cumulative incidence of arterial fistulas and mortality in patients with proven chronic Q fever. METHODS: In a retrospective, observational study, the cumulative incidence of arterial fistulas (aortoenteric, aortobronchial, aortovenous, or arteriocutaneous) in patients with proven chronic Q fever (according to the Dutch Chronic Q Fever Consensus Group criteria) was assessed. Proven chronic Q fever with a vascular focus of infection was defined as a confirmed mycotic aneurysm or infected prosthesis on imaging studies or positive result of serum polymerase chain reaction for C. burnetii in the presence of an arterial aneurysm or vascular prosthesis. RESULTS: Of 253 patients with proven chronic Q fever, 169 patients (67%) were diagnosed with a vascular focus of infection (42 of whom had a combined vascular focus and endocarditis). In total, 26 arterial fistulas were diagnosed in 25 patients (15% of patients with a vascular focus): aortoenteric (15), aortobronchial (2), aortocaval (4), and arteriocutaneous (5) fistulas (1 patient presented with both an aortocaval and an arteriocutaneous fistula). Chronic Q fever-related mortality was 60% for patients with and 21% for patients without arterial fistula (P < .0001). Primary fistulas accounted for 42% and secondary fistulas for 58%. Of patients who underwent surgical intervention for chronic Q fever-related fistula (n = 17), nine died of chronic Q fever-related causes (53%). Of patients who did not undergo any surgical intervention (n = 8), six died of chronic Q fever-related causes (75%). CONCLUSIONS: The proportion of patients with proven chronic Q fever developing primary or secondary arterial fistulas is high; 15% of patients with a vascular focus of infection develop an arterial fistula. This observation suggests that C. burnetii, the causative agent of Q fever, plays a role in the development of fistulas in these patients. Chronic Q fever-related mortality in patients with arterial fistula is very high, in both patients who undergo surgical intervention and patients who do not.

Pulmonary manifestations of Q fever: analysis of 38 patients.

BACKGROUND: Lung involvement in both acute and chronic Q fever is not well described with only a few reported cases of pseudotumor or pulmonary fibrosis in chronic Q fever. The aim of this study was to better understand the pulmonary manifestations of Q fever. METHODS: We conducted a retrospective cohort study of patients with diagnosis of Q fever at Mayo Clinic Rochester. A total of 69 patients were initially identified between 2001 and 2014. Thirty-eight patients were included in this study as 3 were pediatric patients, 20 did not meet serologic criteria for Q fever, and 8 did not have imaging available at time of initial diagnosis. Descriptive analysis was conducted using JMP software. RESULTS: The median age was 57 years [interquartile range (IQR) 43, 62], 84% from the Midwest, and 13% worked in an occupation involving animals. The most common presentation was fevers (61%). Respiratory symptoms, such as cough, were noted in only 4 patients (11%). Twelve patients (29%) had abnormal imaging studies attributed to Q fever. Three patients (25%) with acute Q fever had findings of consolidation, lymphadenopathy, pleural effusions, and nonspecific pulmonary nodules. Radiographic findings of chronic Q fever were seen in 9 patients (75%) and included consolidation, ground-glass opacities, pleural effusions, lymphadenopathy, pulmonary edema, and lung pseudotumor. CONCLUSIONS: Our results demonstrate that pulmonary manifestations are uncommon in Q fever but include cough and consolidation for acute Q fever and radiographic findings of pulmonary edema with pleural effusions, consolidation, and pseudotumor in those with chronic Q fever.