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Disseminated Coccidioidomycosis

Valley Fever

Disseminated coccidioidomycosis is the severe form of the fungal disease in which there is a hematogenous spread of the infection from the lungs to the bone, joints, central nervous system (CNS), skin, and other organs in susceptible patients. The clinical picture is reflective of affected organs. The diagnosis is based on the presentation, physical exam, and appropriate studies.


Presentation

Coccidioidomycosis can manifest in acute, chronic, and disseminated forms, of which the latter is the most severe [1]. Overall, it is caused by airborne transmission of Coccidioides immitis spores. This endemic disease occurs in the southwestern region of the United States, Central America, and South America [2]. Coccidioidomycosis is more common during the summer time and late fall [1]. Risk factors for disseminated coccidioidomycosis include AIDS and other immunosuppressed states as a consequence of chronic steroid therapy, chemotherapy, diabetes, and lymphoma [2].

The majority of patients with coccidioidomycosis have a mild, self-limiting respiratory infection [3] [4]. Symptomatic patients with a primary disease may exhibit fever, cough, dyspnea, malaise, arthralgia, chest pain, chills, weight loss, night sweats, and erythema nodosum [5]. Susceptible patients are at risk for developing disseminated disease weeks or months following the initial infection, in which it spreads hematogenously from the lungs to the bone, joints, CNS, and skin [1]. Extrapulmonary dissemination may occur even after a year of primary infection or following therapy. Those affected with disseminated infection report fever, dyspnea, weight loss, and night sweats.

Disseminated coccidioidomycosis can affect any organ. Musculoskeletal involvement results in lytic or sclerotic bone lesions and arthritis. Additionally, meningitis develops in nearly half of all patients with disseminated disease, which is associated with morbidity and mortality frequency [6]. Specifically, meningitis is characterized by headaches, photophobia, and nuchal rigidity. Also, if present, increased intracranial pressure (ICP) leads to altered mental status and emesis. Patients may even develop cranial nerve palsies, seizures, and coma [7]. Skin features in primary infection are commonly notable for diffuse maculopapular rashes which may evolve into erythema nodosum. Spread to the heart, which is rare, causes pericardial effusions and even tamponade [8].

Poor prognosis is associated with immunocompromised states and untreated individuals [9]. Coccidioidal fungemia is a rare occurrence but involves spread to CNS, liver, and spleen. Acute respiratory distress syndrome (ARDS) and shock are other serious complications.

Patients could present with fever, splenomegaly, and lymphadenopathy. The clinical features will reflect affected organs or systems. Meningeal and other neurologic signs may be apparent. Also, skin infection may be remarkable for papules, plaques, or nodules. If present, pulmonary findings could include rales, rhonchi, and decreased breath sounds.

Decreased Breath Sounds
  • If present, pulmonary findings could include rales, rhonchi, and decreased breath sounds.[symptoma.com]
Fever
  • The patient was a 21-year-old, apparently immunocompetent, pregnant black woman who had fever of unknown origin for 6 weeks. She had recently moved from Arizona.[ncbi.nlm.nih.gov]
  • Symptomatic patients with a primary disease may exhibit fever, cough, dyspnea, malaise, arthralgia, chest pain, chills, weight loss, night sweats, and erythema nodosum.[symptoma.com]
  • The scientific name for Valley fever is “coccidioidomycosis,” and it’s also sometimes called “San Joaquin Valley fever” or “desert rheumatism.”[cdc.gov]
  • The director of the University of Arizona Valley Fever Center for Excellence, John N.[deptmedicine.arizona.edu]
  • Overview Valley fever is a fungal infection caused by coccidioides (kok-sid-e-OY-deze) organisms. It can cause fever, chest pain and coughing, among other signs and symptoms. Two species of coccidioides fungi cause valley fever.[mayoclinic.org]
Weight Loss
  • A 50-year-old man presented with a 2-3 month history of left lower quadrant abdominal pain, right periorbital headache, blurry vision, tinnitus, polydipsia, right elbow pain, and a 32 kg weight loss over the past year.[ncbi.nlm.nih.gov]
  • Symptomatic patients with a primary disease may exhibit fever, cough, dyspnea, malaise, arthralgia, chest pain, chills, weight loss, night sweats, and erythema nodosum.[symptoma.com]
  • Soon after the primary infection, dissemination (extrapulmonary spread) could be first suspected in those patients with a history of weight loss, prolonged duration of generalized symptoms (malaise, FULL TEXT[annals.org]
  • Patients develop chronic productive cough, haemoptysis, weight loss and chest pains.[life-worldwide.org]
Chills
  • Symptomatic patients with a primary disease may exhibit fever, cough, dyspnea, malaise, arthralgia, chest pain, chills, weight loss, night sweats, and erythema nodosum.[symptoma.com]
  • The major signs and symptoms exhibited by Disseminated Coccidioidomycosis are fever and chills, cough, muscle stiffness and aches, altered mental status, and swelling of the joints.[dovemed.com]
  • Patients with mild or self-limiting infections will present with: Low-grade fever with chills and night sweats Fatigue Pain (headaches, sore throat, chest pain) Cough, with possible sputum production and hemoptysis Lower limb/foot swelling Loss of appetite[dermnetnz.org]
  • When the infection is in your lungs, it can cause flu-like symptoms, such as: a cough chest pain a fever chills night sweats fatigue joint aches a red, spotty rash shortness of breath This type of infection is commonly known as valley fever.[healthline.com]
  • They tend to resemble those of the flu, and can range from minor to severe, including: Fever Cough Chest pain Chills Night sweats Headache Fatigue Joint aches Red, spotty rash The rash that sometimes accompanies valley fever is made up of painful red[mayoclinic.org]
Malaise
  • We report a case of disseminated coccidioidomycosis in an immunocompetent black male with cutaneous lesions, a flocculent neck mass, bilateral hilar adenopathy, persistent fever, night sweats and malaise.[ncbi.nlm.nih.gov]
  • Soon after the primary infection, dissemination (extrapulmonary spread) could be first suspected in those patients with a history of weight loss, prolonged duration of generalized symptoms (malaise, FULL TEXT[annals.org]
  • Symptomatic patients with a primary disease may exhibit fever, cough, dyspnea, malaise, arthralgia, chest pain, chills, weight loss, night sweats, and erythema nodosum.[symptoma.com]
  • Primary infection is often complicated by prolonged cough, pleuritic chest pain, malaise, and fatigue, which can be prolonged and debilitating.[clinicaladvisor.com]
Polydipsia
  • A 50-year-old man presented with a 2-3 month history of left lower quadrant abdominal pain, right periorbital headache, blurry vision, tinnitus, polydipsia, right elbow pain, and a 32 kg weight loss over the past year.[ncbi.nlm.nih.gov]
  • A 50-year-old man presented with a 2–3 month history of left lower quadrant abdominal pain, right periorbital headache, blurry vision, tinnitus, polydipsia, right elbow pain, and a 32 kg weight loss over the past year.[nature.com]
Neck Mass
  • He subsequently developed a large neck mass and was found to have mediastinal and hilar adenopathy highly suspicious of a neoplastic process. Fine needle aspiration biopsy of the neck mass, followed by flexible bronchoscopy, was performed.[ncbi.nlm.nih.gov]
  • Fine needle aspiration (FNA) of the neck mass provided the primary diagnosis of coccidioidomycosis. It was confirmed by culture of FNA-obtained fluid and a subsequent biopsy of a cutaneous lesion.[ncbi.nlm.nih.gov]
  • There are case reports of neck masses due to cervical lymphadenopathy representing the only manifestation of disseminated disease. 37 Abdomen and pelvis The abdomen and pelvis are other potential sites for dissemination.[appliedradiology.com]
Dark Urine
  • A young man from California presented with acute onset of cough, fevers, night sweats and pruritus with dark urine. Laboratory studies were notable for moderate transaminitis with elevated bilirubin and eosinophilia.[ncbi.nlm.nih.gov]

Workup

The diagnosis is based on the clinical manifestations, history including risk factors such as a recent travel to endemic areas and the patient's immunocompetence, physical exam, and the appropriate studies.

Laboratory studies should include complete blood count (CBC), which may be normal or significant for elevated lymphocytes, eosinophils, and monocytes. Additionally, there is an increase in erythrocyte sedimentation rate (ESR).

Diagnostic studies include serologic testing such as IgM and IgG antibodies as well as complement fixation techniques for IgG. Polymerase chain reaction (PCR), skin testing, blood cultures, and the urine antigen test are all helpful. Also, visualization of the fungus in body fluids such as blood, sputum, urine, or cerebrospinal fluid (CSF) is beneficial.

Patients with disseminated coccidioidomycosis could exhibit hilar and mediastinal adenopathy as well as the miliary pattern on chest X-ray. Other findings may also be present. Computed tomography (CT) scan reveals the morphology of the lesions more effectively [10].

Patients with suspected meningitis or other neurologic manifestations warrant assessment with CT and magnetic resonance imaging (MRI) of the brain, with the latter demonstrating better sensitivity for coccidioidal meningitis. Frequently observed abnormalities include basilar meningitis, vascular occlusion, and hydrocephalus.

Fluorodeoxyglucose positron emission tomography (FDG-PET) scans demonstrate diffuse intake reflective of disseminated disease and active infection. Also, bone scans are indicated in patients with osteomyelitis or suspected bone disease [11]. MRI can also display affected bone and tissue.

Lumbar puncture is necessary for assessment of meningitis [12]. Common findings in the CSF include lymphocytic and eosinophilic pleocytosis, increased protein, and low glucose levels. Complement-fixating antibodies in the CSF typically reveal elevated titers, which confirms the diagnosis of coccidioidal meningitis.

Pericardial Effusion
  • Spread to the heart, which is rare, causes pericardial effusions and even tamponade. Poor prognosis is associated with immunocompromised states and untreated individuals.[symptoma.com]
  • In addition, disseminated coccidioidomycosis can cause pericardial effusions resulting in the development of cardiac tamponade or restrictive pericarditis. 8 Extrapulmonary manifestations Central nervous system Central nervous system involvement is the[appliedradiology.com]
  • Sometimes patients present with a pericardial effusion resulting from a focus of disease adjacent to the pericardium.[clinicaladvisor.com]
Bilateral Hilar Adenopathy
  • We report a case of disseminated coccidioidomycosis in an immunocompetent black male with cutaneous lesions, a flocculent neck mass, bilateral hilar adenopathy, persistent fever, night sweats and malaise.[ncbi.nlm.nih.gov]
Anergy
  • Reversal of coccidioidal anergy in vivo by dendritic cells from patients with disseminated coccidioidomycosis. J Immunol. 2002;169:2020-2025. (20.) Delgado N, Xue J, Yu J, Hung CY, Cole G.[biomedsearch.com]

Treatment

  • To determine the efficacy and safety of fluconazole as treatment for coccidioidomycosis. This was a multicenter, open-label, single-arm study.[ncbi.nlm.nih.gov]
  • Six had received treatment with amphotericin B previously and five of these patients could be evaluated for the efficacy of the treatment.[ncbi.nlm.nih.gov]
  • The abscess in the vertebral bodies also responded to treatment, but a small lesion was still left in the 10th vertebral body after 2 years of treatment.[ncbi.nlm.nih.gov]
  • The combination should be considered in the treatment of disseminated coccidioidomycosis with central nervous system involvement.[ncbi.nlm.nih.gov]
  • Amphotericin B is an important part of the therapy and treatment of invasive and life-threatening mycoses.[ncbi.nlm.nih.gov]

Prognosis

  • Aspergillus was the second most common offender, and disseminated infection was associated with a very grave prognosis for the transplant recipient. Rare infections with Mucor and Cryptococcus neoformans are described in the literature.[ncbi.nlm.nih.gov]
  • The prognosis of the acute form is excellent, since it is self-limiting Who gets Disseminated Coccidioidomycosis? (Age and Sex Distribution) Any individual may be affected by coccidioidomycosis.[dovemed.com]
  • Poor prognosis is associated with immunocompromised states and untreated individuals. Coccidioidal fungemia is a rare occurrence but involves spread to CNS, liver, and spleen.[symptoma.com]
  • Treatment and prognosis The mainstay of medical treatment is with fluconazole 7 . Surgical opinion may also be sought for CNS involvement or complications of pulmonary coccidioidomycosis if necessary 7 . Promoted articles (advertising)[radiopaedia.org]
  • Outlook and prognosis Coccidioidal meningitis needs to be treated lifelong, as it is incurable. Complications including communicating hydrocephalus.[life-worldwide.org]

Etiology

  • The expression of PTHrP is a property of infectious granulomas regardless of etiology or the tissue involved, suggesting that PTHrP expression is part of the normal granulomatous immune response.[ncbi.nlm.nih.gov]
  • Because all tests yielded negative results, the known history of disseminated coccidioidomycosis was presumed to be the etiology of this iritis.[ncbi.nlm.nih.gov]
  • Both patients were later found to have disseminated Coccidioidomycosis, a rare diagnosis that is often mistaken for other etiologies. Both patients responded well to fluconazole therapy.[content.iospress.com]
  • April 06, 2015 ; 84 (14 Supplement) April 23, 2015 Rahul Shah , Bing Liao , Elena Shanina , Glenn Smith First published April 8, 2015, Abstract OBJECTIVE: To emphasize Coccidioidomycosis as an unusual etiology for brachial plexitis BACKGROUND: Coccidioidomycosis[neurology.org]
  • This report is intended to increase clinician awareness of the possibility of underlying immunodeficiency syndromes in patients who present with disseminated fungal disease with an unclear etiology and may provide insight in diagnosing and further managing[frontiersin.org]

Epidemiology

  • The epidemiology, diagnosis, and management of coccidioidomycosis are discussed.[ncbi.nlm.nih.gov]
  • Chapter 6: Epidemiology of coccidioidomycosis, pages 199-238. IN: McGinnis MR (editor). Current Topics in Medical Mycology, Volume 2. Springer-Verlag. 1988. Rosenstein NE, Emery KW, et al.[meducator3.net]
  • Epidemiologic, clinical, and diagnostic aspects of coccidioidomycosis. J Clin Microbiol . 2007;45:26-30. Williams PL. Coccidioidal meningitis. Ann NY Acad Sci . 2007; 1111:377-384. Davis LE, Porter BS.[appliedradiology.com]
  • Epidemiological, clinical, and diagnostic aspects of coccidioidomycosis . J. Clin. Microbiol. 45 , 26–30 (2007). 4. Hector, R. F. & Laniado-Laborin, R. Coccidioidomycosis—a fungal disease of the Americas . PLoS Med. 2 , e2 (2005). 5. Crum, N.[nature.com]
  • "Epidemiologic, clinical and diagnostic aspects of coccidioidomycosis". J Clin Microbiol. vol. 45. 2007. pp. 26-30. Valdivia, L, Nix, D, Wright, M. "Coccidioidomycosis as a common cause of community-acquired pneumonia".[clinicaladvisor.com]
Sex distribution
Age distribution

Prevention

  • Cell-mediated immunity, which appears to be the relevant host defense mechanism, is impaired by the immunosupressive agents used to prevent allograft rejection.[ncbi.nlm.nih.gov]
  • Early treatment of CM with oral antifungal agents may prevent severe pulmonary disease in groups considered to be at high risk, such as elderly individuals, persons with diabetes, and smokers.[ncbi.nlm.nih.gov]
  • They may also help in the development of preventative vaccines.[grantome.com]
  • SOURCE: Centers for Disease Control and Prevention (CDC) Impacts and Issues Coccidioidomycosis has plagued humans for many years. A cure has long been sought, but not yet attained.[encyclopedia.com]
  • Professor of Microbiology at Emory University School of Medicine (EUSM), Atlanta, Georgia, and Research Microbiologist, Molecular Typing Unit, Mycotic Diseases Branch, Division of Bacterial and Mycotic Diseases in the Centers for Disease Control and Prevention[books.google.com]

References

Article

  1. Cummings KC, McDowell A, Wheeler C, et al. Point-source outbreak of coccidioidomycosis in construction workers. Epidemiol Infect. 2010;138(4):507-511.
  2. Kirkland TN, Fierer J Coccidioidomycosis: a reemerging infectious disease. Emerg Infect Dis. 1996;2(3):192–199.
  3. Galgiani JN. Coccidioides Species. In: Mandell G, Bennett JE, eds. Principles and Practices of Infectious Diseases Vol 2. 6th ed. Philadelphia, PA: Elsevier; 2005:3040-3051.
  4. Richardson MD, Warnock DW. Coccidioidomycosis. In: Fungal Infection Diagnosis and Management. Boston: MA: Blackwell Scientific Publication; 1993: 134-142.
  5. Centers for Disease Control and Prevention (CDC). Increase in Coccidioidomycosis - California, 2000-2007. MMWR Morb Mortal Wkly Rep. 2009;58(5):105-109.
  6. Blair JE, Mayer AP, Currier J, Files JA, Wu Q. Coccidioidomycosis in elderly persons. Clin Infect Dis. 2008; 47(12):1513-1518.
  7. Blair JE. Coccidioidal meningitis: update on epidemiology, clinical features, diagnosis, and management. Curr Infect Dis Rep. 2009;11(4):289-295.
  8. Arsura EL, Bobba RK, Reddy CM. Coccidioidal pericarditis: a case presentation and review of the literature. Int J Infect Dis. 2005;9(2):104-109.
  9. Keckich DW, Blair JE, Vikram HR. Coccidioides fungemia in six patients, with a review of the literature. Mycopathologia. 2010;170(2):107-115.
  10. Kim KI, Leung AN, Flint JD, Müller NL. Chronic pulmonary coccidioidomycosis: computed tomographic and pathologic findings in 18 patients. Can Assoc Radiol J. 1998;49(6):401-407.
  11. Taljanovic MS, Adam RD. Musculoskeletal coccidioidomycosis. Semin Musculoskelet Radiol. 2011;15(5):511-526.
  12. Adam RD, Elliott SP, Taljanovic MS. The spectrum and presentation of disseminated coccidioidomycosis. Am J Med. 2009; 122(8):770-777.

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Last updated: 2018-06-21 18:21