Paracoccidioidomycosis overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Danitza Lukac
Overview
Paracoccidioidomycosis (also known as Lutz-Splendore-Almeida disease or Brazilian blastomycosis) is a mycosis caused by the fungus Paracoccidioides brasiliensis and Paracoccidioides lutzii. Sometimes called South American blastomycosis, paracoccidioidomycosis is caused by a different fungus than that which causes blastomycosis. Lutz-Splendore-de Almeida disease is named for the physicians Adolfo Lutz, Alfonso Splendore, and Floriano Paulo de Almeida, who first characterized the disease in Brazil in the early 20th century. [1] Paracoccidioidomycosis may be classified according to Franco et al. in 1987 into: paracoccidioidomycosis infection, paracoccidioidomycosis disease, paracoccidioidomycosis associated with immunosupression and residual form (sequela). Based on the duration of symptoms, paracoccidioidomycosis disease may be classified into: acute, subacute or chronic.[2][3]Spores of Paracoccidioides spp. are commonly transmitted via the respiratory route to the human host. Following transmission, Paracoccidioides spp. particles invade the terminal bronchioles and alveoli where granulomas are formed, but can be inactive for approximately 40 years. [3] On microscopic histopathological analysis, a pilot's wheel or Mickey mouse ears-like appearance are a characteristic finding of PMC. [4] [5] [6]Common risk factors in the development of paracoccidioidomycosis disease are: age, gender, poor hygiene, occupation, malnutrition, tabacco and alcohol consumption. [2] [7] In paracoccidioidomycosis disease, the majority of infected patients do not develop any symptoms.[3] The acute form affects 5% of the patients, and it has a more rapid and severe evolution. [8][9] Meanwhile, the chronic form which represents 90% of the patients, has a more slow evolution. Chronic PMC most frequently develops pulmonary symptoms which can leave severe sequela. [10] [11] Complications that can develop as a result of PMC are: chronic obstructive pulmonary disease (COPD), pulmonary fibrosis, bullae, pulmonary hypertension, dyspnea, adrenal gland insufficiency, dysphonia, laryngeal lesions (such as glottis estenosis), microstomia, seizures and motor deficiency. [11] [12] [9] The prognosis of paracoccidioidomycosis is good with treatment.[13] Patients with acute paracoccidioidomycosis usually have an ill appearance, and chronic PMC patients can appear healthy at early stages. Physical examination of patients with juvenile PMC is usually remarkable for node swelling, hepatomegaly, and splenomegaly. The presence of pulmonary abnormalities and skin lesions on physical examination is suggestive of adult PMC. [10] Pharmacologic medical therapy is indicated in paracoccidioidomycosis. The preferred regimens for both mild and moderate-to-severe include antifungals either azoles (such as itraconazole, ketoconazole, vorizonazole) or amphotericin B and antimicrobials such as trimetropin-sulfamethoxazole. [14] Surgery is not the first-line treatment option for patients with paracoccidioidomycosis. Different surgical procedures are usually reserved for patients with PMC sequelae. There are no primary preventive measures available for paracoccidioidomycosis.
Historical Perspective
Lutz-Splendore-de Almeida disease is named for the physicians Adolfo Lutz, Alfonso Splendore, and Floriano Paulo de Almeida, who first characterized the disease in Brazil in the early 20th century. [15]
Classification
Paracoccidioidomycosis may be classified according to Franco et al. in 1987 into: paracoccidioidomycosis infection, paracoccidioidomycosis disease, paracoccidioidomycosis associated with inmunosupression and residual form (sequela). Based on the duration of symptoms, paracoccidioidomycosis disease may be classified into: acute, subacute or chronic. The chronic form can be subclassified into: unifocal and multifocal.[2][3]Paracoccidioidomycosis tends to affect agriculture workers from southern Mexico to northern Argentina. Paracoccidioidomycosis is prevalent in Brazil, Colombia, Venezuela, and Argentina, and is classically associated with individuals from rural areas. The typical patient is a man aged 30 to 50 years. [16] PMC disease affects men, more commonly than women. However, PMC infection can affect anyone. [3]
Pathophysiology
Spores of Paracoccidioides spp. are commonly transmitted via the respiratory route to the human host. Following transmission, Paracoccidiodes spp. particles invade the terminal bronchioles and alveoli where granulomas are formed, but can be inactive for approximately 40 years. [3] On microscopic histopathological analysis, a pilot's wheel or Mickey mouse ears-like appearance are a characteristic finding of PMC. [17] [5] [6]
Causes
Paracoccidioidomycosis may be caused by either Paracoccidioides brasiliensis or Paracoccidioides lutzii.
Differential Diagnosis
Paracoccidioidomycosis must be differentiated from tuberculosis, histoplasmosis and metastasis. [18]
Epidemiology and Demographics
Paracoccidioidomycosis has been reported as an autochthonous disease, that tends to affect agriculture workers from southern Mexico to northern Argentina. Paracoccidioidomycosis is prevalent in Brazil, Colombia, Venezuela, and Argentina, and is classically associated with individuals from rural areas. The typical patient is a man aged 30 to 50 years. [19] PMC disease affects men, more commonly than women. However, PMC infection can affect anyone. [3]
Risk Factors
Common risk factors in the development of paracoccidioidomycosis disease are: age, gender, poor hygiene, occupation, malnutrition, tabacco and alcohol consumption. [2] [7]
Natural History, Complications and Prognosis
In paracoccidioidomycosis disease, the majority of infected patients do not develop any symptoms.[3] The acute form affects 5% of the patients, and it has a more rapid and severe evolution. [8][9] Meanwhile, the chronic form which represents 90% of the patients, has a more slow evolution. Chronic PMC most frequently develops pulmonary symptoms which can leave severe sequela. [10] [11] Complications that can develop as a result of PMC are: chronic obstructive pulmonary disease (COPD), pulmonary fibrosis, bullae, pulmonary hypertension, dyspnea, adrenal gland insufficiency, dysphonia, laryngeal lesions (such as glottis estenosis), microstomia, seizures, motor deficiency. [11] [12] [9] The prognosis of paracoccidioidomycosis is good with treatment. Without treatment, PMC will result in death due to disease complications. The presence of late diagnosis and sequelae is associated with a particularly poor prognosis among patients with PMC. [13]
History and Symptoms
Primary infection is thought to be autolimited and almost asymptomatic as histoplasmosis or Valley Fever. In young people, there is a progressive form of the disease (akin of tuberculous septicemia in tuberculous priminfection) with high prostrating fever, generalized lymphadenopathy and pulmonary involvement with milliary lesions. This juvenile form has a more severe prognosis even with treatment. The most common form is the so called adult form of paracoccidioidomycosis that is almost certainly a reactivation of the disease. Chronic PMC has mainly pulmonary symptoms. [20]
Physical Examination
Patients with acute paracoccidioidomycosis usually have an ill appearance, and chronic PMC patients can appear healthy at early stages. Physical examination of patients with juvenile PMC is usually remarkable for node swelling, hepatomegaly, and esplenomegaly. The presence of pulmonary abnormalities and skin lesions on physical examination is suggestive of adult PMC. [10]
Laboratory Findings
Laboratory findings consistent with the diagnosis of acute PMC include anemia, Hypergammaglobulinemia, Eosinophilia, Hypoalbulinemia, Mild increase of AST and ALT and conjugated hyperbilirrubinemia. [9]
Imaging Findings
Common chest x-ray findings in chronic PMC include bilateral and symmetric opacities, butterfly wing pattern, architectural distorsion, paracicatricial emphysema an traction bronchiectasis. There are not many chest x-ray in acute PMC, but it is characterized by mediastinal and hiliar lymphadenopathy and pleural effusions.[8]. On thoraxic CT scan, chronica paracoccidioidomycosis is characterized by ground-glass attenuation, airspace consolidations, interlobular septal thickening, nodular pattern, fibrotic pattern, cavitary lesions, halo sign and reversed halo sign. [21] [22] [23] [8]
Medical Therapy
Sulphonamides are the traditional remedies to paracoccidiodomycosis. The most used sulfa drugs in this infection are sulfadimethoxime, sulfadiazine and co-trimoxazole. This treatment is generally safe but several adverse effects can appear, the most severe of which are the Stevens Johnson Syndrome and agranulocytosis. It must be continued for up to 3 years to obtain cure and relapse and treatment failures aren't unusual. Antifungal drugs like Amphotericin B or Ketoconazole are also effective in clearing the infection but they are very expensive compared with sulphonamides.[24]
Surgery
Surgery is not the first-line treatment option for patients with paracoccidioidomycosis. Different surgical procedures are usually reserved for patients with PMC sequelae.
Prevention
There are no primary preventive measures available for paracoccidioidomycosis.
References
- ↑ Paracoccidioidomycosis. Wikipedia.https://en.wikipedia.org/wiki/Paracoccidioidomycosis. Accessed on January 12, 2016
- ↑ 2.0 2.1 2.2 2.3 de Oliveira HC, Assato PA, Marcos CM, Scorzoni L, de Paula E Silva AC, Da Silva Jde F; et al. (2015). "Paracoccidioides-host Interaction: An Overview on Recent Advances in the Paracoccidioidomycosis". Front Microbiol. 6: 1319. doi:10.3389/fmicb.2015.01319. PMC 4658449. PMID 26635779.
- ↑ 3.0 3.1 3.2 3.3 3.4 3.5 3.6 3.7 Fortes MR, Miot HA, Kurokawa CS, Marques ME, Marques SA (2011). "Immunology of paracoccidioidomycosis". An Bras Dermatol. 86 (3): 516–24. PMID 21738969.
- ↑ Paracoccidioidomycosis. Wikipedia.https://en.wikipedia.org/wiki/Paracoccidioidomycosis. Accessed on January 12, 2016
- ↑ 5.0 5.1 Manns B.J, Baylis B.W, Urbanski S.J, Gibb A.P, Rabin H.R. Paracoccidioidomycosis: Case Report and Review. CID. 1996; 23: 1026-1032
- ↑ 6.0 6.1 Vargas J, Vargas R. Paracoccidiodomicosis. Rev. enferm. infecc. trop.2009(1):49-56
- ↑ 7.0 7.1 Magalhães EM, Ribeiro Cde F, Dâmaso CS, Coelho LF, Silva RR, Ferreira EB; et al. (2014). "Prevalence of paracoccidioidomycosis infection by intradermal reaction in rural areas in Alfenas, Minas Gerais, Brazil". Rev Inst Med Trop Sao Paulo. 56 (4): 281–5. PMC 4131811. PMID 25076426.
- ↑ 8.0 8.1 8.2 8.3 Barreto MM, Marchiori E, Amorim VB, Zanetti G, Takayassu TC, Escuissato DL; et al. (2012). "Thoracic paracoccidioidomycosis: radiographic and CT findings". Radiographics. 32 (1): 71–84. doi:10.1148/rg.321115052. PMID 22236894.
- ↑ 9.0 9.1 9.2 9.3 9.4 Brummer E, Castaneda E, Restrepo A. Paracoccidioidomycosis: An Update. 'Clin. Microbiol. Rev.1993;Vol 6(2):89-117
- ↑ 10.0 10.1 10.2 10.3 Vargas J, Vargas R. Paracoccidiodomicosis. Rev. enferm. infecc. trop.2009(1):49-56
- ↑ 11.0 11.1 11.2 11.3 Wanke B, Aidê M. Chapter 6 - Paracoccidioidomycosis. J. bras. pneumol. 2009; 35(12):1245-1249
- ↑ 12.0 12.1 Francesconi F, da Silva MT, Costa RL, et al. Long-term outcome of neuroparacoccidioidomycosis treatment. Rev Soc Bras Med Trop. 2011;44(1):22-25
- ↑ 13.0 13.1 Martinez, R.Epidemiology of Paracoccidioidomycosis. Rev. Inst. Med. trop. S. Paulo. 2015;57(19), 11-20
- ↑ Marques SA (2013). "Paracoccidioidomycosis: epidemiological, clinical, diagnostic and treatment up-dating". An Bras Dermatol. 88 (5): 700–11. doi:10.1590/abd1806-4841.20132463. PMC 3798345. PMID 24173174.
- ↑ Paracoccidioidomycosis. Wikipedia.https://en.wikipedia.org/wiki/Paracoccidioidomycosis. Accessed on January 12, 2016
- ↑ Paracoccidioidomycosis. Wikipedia.https://en.wikipedia.org/wiki/Paracoccidioidomycosis. Accessed on January 12, 2016
- ↑ Paracoccidioidomycosis. Wikipedia.https://en.wikipedia.org/wiki/Paracoccidioidomycosis. Accessed on January 12, 2016
- ↑ Manns B.J, Baylis B.W, Urbanski S.J, Gibb A.P, Rabin H.R. Paracoccidioidomycosis: Case Report and Review. CID. 1996; 23:1026-1032
- ↑ Paracoccidioidomycosis. Wikipedia.https://en.wikipedia.org/wiki/Paracoccidioidomycosis. Accessed on January 12, 2016
- ↑ Paracoccidioidomycosis. Wikipedia.https://en.wikipedia.org/wiki/Paracoccidioidomycosis. Accessed on January 12, 2016
- ↑ Marchiori E, Valiante PM, Mano CM, Zanetti G, Escuissato DL, Souza AS; et al. (2011). "Paracoccidioidomycosis: high-resolution computed tomography-pathologic correlation". Eur J Radiol. 77 (1): 80–4. doi:10.1016/j.ejrad.2009.06.017. PMID 19608361.
- ↑ Funari M, Kavakama J, Shikanai-Yasuda MA, Castro LG, Bernard G, Rocha MS; et al. (1999). "Chronic pulmonary paracoccidioidomycosis (South American blastomycosis): high-resolution CT findings in 41 patients". AJR Am J Roentgenol. 173 (1): 59–64. doi:10.2214/ajr.173.1.10397100. PMID 10397100.
- ↑ Souza AS, Gasparetto EL, Davaus T, Escuissato DL, Marchiori E (2006). "High-resolution CT findings of 77 patients with untreated pulmonary paracoccidioidomycosis". AJR Am J Roentgenol. 187 (5): 1248–52. doi:10.2214/AJR.05.1065. PMID 17056912.
- ↑ Paracoccidioidomycosis. Wikipedia.https://en.wikipedia.org/wiki/Paracoccidioidomycosis. Accessed on January 12, 2016