As nocardiosis is a rare disease, the choice of drug, its administration, and duration of treatment have not been well established in clinical trials.[43]Margalit I, Lebeaux D, Tishler O, et al. How do I manage nocardiosis? Clin Microbiol Infect. 2021 Apr;27(4):550-8.
https://www.clinicalmicrobiologyandinfection.com/article/S1198-743X(20)30779-5/fulltext
http://www.ncbi.nlm.nih.gov/pubmed/33418019?tool=bestpractice.com
Most of the recommendations are based on the results of basic research, animal models, and clinical expert opinions.
Although treatment should be guided by Nocardia speciation and susceptibility testing, clinicians usually start treatment empirically, when the results are still not available. Sulfonamides (e.g., trimethoprim/sulfamethoxazole) have been the agents of choice for the treatment of nocardiosis for more than 60 years. Depending on the clinical presentation, the evolution of the disease, and the involved body site, adjuvant surgical treatment may also be necessary.
Initial medical treatment for patients with severe disease
Severe disease is considered to be disseminated disease, central nervous system (CNS) involvement, or pulmonary disease with signs of extensive infection. Although sulfonamides have classically been the drug of choice, patients with severe disease are typically treated with a combination of two or three drugs. Combination therapy is used both to ensure adequate antimicrobial coverage and because of possible synergy between antibiotics.[62]Restrepo A, Clark NM, Infectious Diseases Community of Practice of the American Society of Transplantation. Nocardia infections in solid organ transplantation: guidelines from the Infectious Diseases Community of Practice of the American Society of Transplantation. Clin Transplant. 2019 Sep;33(9):e13509.
http://www.ncbi.nlm.nih.gov/pubmed/30817024?tool=bestpractice.com
For the treatment of severe disease, some experts recommend a triple regimen consisting of trimethoprim/sulfamethoxazole, amikacin, and carbapenem (e.g., imipenem/cilastatin). Alternatively, a two-drug regimen may be advised for severe disease not involving the CNS, such as trimethoprim/sulfamethoxazole plus amikacin or imipenem/cilastatin, or imipenem/cilastatin plus amikacin. Linezolid is another option to include in an initial regimen for severe disease.[43]Margalit I, Lebeaux D, Tishler O, et al. How do I manage nocardiosis? Clin Microbiol Infect. 2021 Apr;27(4):550-8.
https://www.clinicalmicrobiologyandinfection.com/article/S1198-743X(20)30779-5/fulltext
http://www.ncbi.nlm.nih.gov/pubmed/33418019?tool=bestpractice.com
Sulfonamides
Sulfonamides are considered a drug of choice for nocardiosis. Trimethoprim/sulfamethoxazole achieves good penetration into the CNS, and has good bioavailability when given orally.
However, these drugs are associated with a high incidence of adverse events such as allergic reactions, acute kidney injury, hyperkalemia, hematologic toxicity, and severe nausea.[63]Wallace RJ Jr, Septimus EJ, Williams TW Jr, et al. Use of trimethoprim-sulfamethoxazole for treatment of infections due to Nocardia. Rev Infect Dis. 1982 Mar-Apr;4(2):315-25.
http://www.ncbi.nlm.nih.gov/pubmed/6981158?tool=bestpractice.com
Monitoring for renal toxicity, electrolyte disturbances, and hematologic toxicity is recommended during treatment.
Resistance to sulfonamides is possible with some Nocardia species. For example, Nocardia farcinica is commonly resistant to trimethoprim/sulfamethoxazole.
Linezolid
Linezolid, an oxazolidinone antibiotic, has shown strong in-vitro activity against most of the Nocardia species and strains tested.[64]Brown-Elliott BA, Ward SC, Crist CJ, et al. In vitro activities of linezolid against multiple Nocardia species. Antimicrob Agents Chemother. 2001 Apr;45(4):1295-7.
https://journals.asm.org/doi/10.1128/aac.45.4.1295-1297.2001
http://www.ncbi.nlm.nih.gov/pubmed/11257051?tool=bestpractice.com
Additionally, linezolid has high oral bioavailability and can penetrate the CNS.[65]Mwandia G, Polenakovik H. Nocardia spp. pneumonia in a solid organ recipient: role of linezolid. Case Rep Infect Dis. 2018 Jan 30:2018:1749691.
https://www.hindawi.com/journals/criid/2018/1749691
http://www.ncbi.nlm.nih.gov/pubmed/29666726?tool=bestpractice.com
[66]Myrianthefs P, Markantonis SL, Vlachos K, et al. Serum and cerebrospinal fluid concentrations of linezolid in neurosurgical patients. Antimicrob Agents Chemother. 2006 Dec;50(12):3971-6.
https://aac.asm.org/content/50/12/3971
http://www.ncbi.nlm.nih.gov/pubmed/16982782?tool=bestpractice.com
There is growing evidence to support the use of a linezolid-containing regimen in the treatment of nocardiosis, including among patients with CNS involvement, although some clinicians have raised concerns that the range of serious adverse effects might limit its widespread use.[62]Restrepo A, Clark NM, Infectious Diseases Community of Practice of the American Society of Transplantation. Nocardia infections in solid organ transplantation: guidelines from the Infectious Diseases Community of Practice of the American Society of Transplantation. Clin Transplant. 2019 Sep;33(9):e13509.
http://www.ncbi.nlm.nih.gov/pubmed/30817024?tool=bestpractice.com
[67]Moylett EH, Pacheco SE, Brown-Elliott BA, et al. Clinical experience with linezolid for the treatment of Nocardia infection. Clin Infect Dis. 2003 Feb 1;36(3):313-8.
https://academic.oup.com/cid/article/36/3/313/352803
http://www.ncbi.nlm.nih.gov/pubmed/12539073?tool=bestpractice.com
[68]Jodlowski TZ, Melnychuk I, Conry J. Linezolid for the treatment of Nocardia spp. infections. Ann Pharmacother. 2007 Oct;41(10):1694-9.
http://www.ncbi.nlm.nih.gov/pubmed/17785610?tool=bestpractice.com
[69]Ntziora F, Falagas ME. Linezolid for the treatment of patients with central nervous system infection. Ann Pharmacother. 2007 Feb;41(2):296-308.
http://www.ncbi.nlm.nih.gov/pubmed/17284501?tool=bestpractice.com
One 2019 retrospective study of 20 patients with moderate-to-severe nocardiosis found that initial empirical treatment with a linezolid-containing regimen was at least as safe and effective as other first-line agents. In this study, the most common adverse effects were thrombocytopenia and anemia; however, adverse effects necessitating discontinuation of treatment were more common in patients treated with trimethoprim/sulfamethoxazole than linezolid.[70]Davidson N, Grigg MJ, Mcguinness SL, et al. Safety and outcomes of linezolid use for nocardiosis. Open Forum Infect Dis. 2020 Mar 16;7(4):ofaa090.
https://academic.oup.com/ofid/article/7/4/ofaa090/5805505
http://www.ncbi.nlm.nih.gov/pubmed/32258209?tool=bestpractice.com
Another small study reported that 45% of patients developed myelosuppression and 18% developed neuropathy as an adverse effect of linezolid treatment for nocardiosis.[68]Jodlowski TZ, Melnychuk I, Conry J. Linezolid for the treatment of Nocardia spp. infections. Ann Pharmacother. 2007 Oct;41(10):1694-9.
http://www.ncbi.nlm.nih.gov/pubmed/17785610?tool=bestpractice.com
Other linezolid-associated adverse effects, reported in small series of patients receiving treatment for nocardiosis, include optic neuritis and lactic acidosis.[65]Mwandia G, Polenakovik H. Nocardia spp. pneumonia in a solid organ recipient: role of linezolid. Case Rep Infect Dis. 2018 Jan 30:2018:1749691.
https://www.hindawi.com/journals/criid/2018/1749691
http://www.ncbi.nlm.nih.gov/pubmed/29666726?tool=bestpractice.com
[67]Moylett EH, Pacheco SE, Brown-Elliott BA, et al. Clinical experience with linezolid for the treatment of Nocardia infection. Clin Infect Dis. 2003 Feb 1;36(3):313-8.
https://academic.oup.com/cid/article/36/3/313/352803
http://www.ncbi.nlm.nih.gov/pubmed/12539073?tool=bestpractice.com
The duration of treatment and the presence of preexisting factors (e.g., underlying neurologic disease, treatment with chemotherapy, diabetes, alcohol use disorder) appear to increase the risk of developing neurologic complications.[71]Bressler AM, Zimmer SM, Gilmore JL, et al. Peripheral neuropathy associated with prolonged use of linezolid. Lancet Infect Dis. 2004 Aug;4(8):528-31.
http://www.ncbi.nlm.nih.gov/pubmed/15288827?tool=bestpractice.com
The risk of thrombocytopenia has also been reported to increase when linezolid is used for more than 10 days.[72]Attassi K, Hershberger E, Alam R, et al. Thrombocytopenia associated with linezolid therapy. Clin Infect Dis. 2002 Mar 1;34(5):695-8.
https://academic.oup.com/cid/article/34/5/695/319032
http://www.ncbi.nlm.nih.gov/pubmed/11803505?tool=bestpractice.com
The risk of hematologic and neurologic complications might limit the use of linezolid to monitored hospital settings and for initial, empirical treatment. Careful monitoring for these complications is recommended with treatment.
Amikacin
Amikacin, an aminoglycoside antibiotic, is often included in initial empiric treatment regimens due to high susceptibility rates across Nocardia species. However, its use is associated with potential nephrotoxicity and ototoxicity, including vestibular dysfunction and irreversible hearing loss.
Careful monitoring of serum amikacin levels and for signs of nephrotoxicity and ototoxicity, are recommended when treating with amikacin, especially if the treatment duration is prolonged.
Amikacin achieves poor CNS penetration, therefore a two-drug regimen containing this drug may be insufficient for the treatment of CNS disease.
Initial medical treatment for patients with nonsevere pulmonary disease
Nonsevere pulmonary disease may be treated with trimethoprim/sulfamethoxazole monotherapy, or with a two-drug regimen consisting of trimethoprim/sulfamethoxazole plus another agent such as ceftriaxone, ciprofloxacin, moxifloxacin, linezolid, or minocycline.[21]Martínez R, Reyes S, Menendez R. Pulmonary nocardiosis: risk factors, clinical features, diagnosis and prognosis. Curr Opin Pulm Med. 2008 May;14(3):219-27.
http://www.ncbi.nlm.nih.gov/pubmed/18427245?tool=bestpractice.com
[43]Margalit I, Lebeaux D, Tishler O, et al. How do I manage nocardiosis? Clin Microbiol Infect. 2021 Apr;27(4):550-8.
https://www.clinicalmicrobiologyandinfection.com/article/S1198-743X(20)30779-5/fulltext
http://www.ncbi.nlm.nih.gov/pubmed/33418019?tool=bestpractice.com
Choice of antibiotic therapy is guided by the treating clinician and depends on whether the infection is mild or moderate.
Initial medical treatment for patients with cutaneous disease
Cutaneous nocardiosis may be treated with trimethoprim/sulfamethoxazole monotherapy or combination therapy with trimethoprim/sulfamethoxazole plus another agent (e.g., moxifloxacin, ciprofloxacin, imipenem/cilastatin, linezolid) is recommended for mycetoma or if there is deep tissue involvement or extension to the bone.[12]Inamadar AC, Palit A. Primary cutaneous nocardiosis: a case study and review. Indian J Dermatol Venereol Leprol. 2003 Nov-Dec;69(6):386-91.
https://ijdvl.com/view-pdf/?article=0cab24b21adc6a06d5d89ee6f633826aeHhbkI9oVMVa6w==
http://www.ncbi.nlm.nih.gov/pubmed/17642947?tool=bestpractice.com
[43]Margalit I, Lebeaux D, Tishler O, et al. How do I manage nocardiosis? Clin Microbiol Infect. 2021 Apr;27(4):550-8.
https://www.clinicalmicrobiologyandinfection.com/article/S1198-743X(20)30779-5/fulltext
http://www.ncbi.nlm.nih.gov/pubmed/33418019?tool=bestpractice.com
[73]Maraki S, Scoulica E, Alpantaki K, et al. Lymphocutaneous nocardiosis due to Nocardia brasiliensis. Diagn Microbiol Infect Dis. 2003 Sep;47(1):341-4.
http://www.ncbi.nlm.nih.gov/pubmed/12967747?tool=bestpractice.com
[74]Negroni R, Lopez Daneri G, Arechavala A, et al. Clinical and microbiological study of mycetomas at the Muniz hospital of Buenos Aires between 1989 and 2004 [in Spanish]. Rev Argent Microbiol. 2006 Jan-Mar;38(1):13-8.
http://www.ncbi.nlm.nih.gov/pubmed/16784127?tool=bestpractice.com
Imipenem/cilastatin has shown to be effective for actinomycetomas due to Nocardia refractory to sulfonamides.[75]Ameen M, Arenas R, Vásquez del Mercado E, et al. Efficacy of imipenem therapy for Nocardia actinomycetomas refractory to sulfonamides. J Am Acad Dermatol. 2010 Feb;62(2):239-46.
http://www.ncbi.nlm.nih.gov/pubmed/20005007?tool=bestpractice.com
Follow-up treatment
After empiric therapy is initiated, treatment should be guided by the results of Nocardia speciation and susceptibility testing.[43]Margalit I, Lebeaux D, Tishler O, et al. How do I manage nocardiosis? Clin Microbiol Infect. 2021 Apr;27(4):550-8.
https://www.clinicalmicrobiologyandinfection.com/article/S1198-743X(20)30779-5/fulltext
http://www.ncbi.nlm.nih.gov/pubmed/33418019?tool=bestpractice.com
When the results of typing and sensitivity tests are available, treatment should be targeted toward that isolate. After the patient has clinically improved, oral agents can be considered for follow-up therapy. Potentially useful drugs, according to the species involved, are cephalosporins (e.g., ceftriaxone), other beta-lactams (e.g., amoxicillin/clavulanate), trimethoprim/sulfamethoxazole, minocycline, fluoroquinolones (e.g., ciprofloxacin, moxifloxacin), linezolid, and carbapenems (e.g., meropenem, imipenem/cilastatin, ertapenem). Most Nocardia farcinica isolates are resistant to the cephalosporins, but cephalosporins are highly active against N asteroides type VI, N nova, and N abscessus. The latter species is more frequently resistant to imipenem/cilastatin. N cyriacigeorgica has also been reported to be resistant to imipenem/cilastatin.[46]Hagerman A, Rodríguez-Nava V, Boiron P, et al. Imipenem-resistant Nocardia cyriacigeorgica infection in a child with chronic granulomatous disease. J Clin Microbiol. 2011 Mar;49(3):1185-7.
https://journals.asm.org/doi/10.1128/jcm.02073-10
http://www.ncbi.nlm.nih.gov/pubmed/21177900?tool=bestpractice.com
[76]Conville PS, Witebsky FG. Organisms designated as Nocardia asteroides drug pattern type VI are members of the species Nocardia cyriacigeorgica. J Clin Microbiol. 2007 Jul;45(7):2257-9.
https://journals.asm.org/doi/10.1128/jcm.00133-07
http://www.ncbi.nlm.nih.gov/pubmed/17475753?tool=bestpractice.com
Since susceptibility to different carbapenems changes for different Nocardia species, there may be utility in testing Nocardia for imipenem/cilastatin, meropenem, and ertapenem sensitivity.[77]Brown-Elliott BA, Killingley J, Vasireddy S, et al. In vitro comparison of ertapenem, meropenem, and imipenem against isolates of rapidly growing mycobacteria and Nocardia using broth microdilution and e-tests. J Clin Microbiol. 2016 Jun;54(6):1586-92.
https://journals.asm.org/doi/10.1128/jcm.00298-16
http://www.ncbi.nlm.nih.gov/pubmed/27053677?tool=bestpractice.com
Fluoroquinolones are active against most N farcinica and N brasiliensis strains. N brasiliensis is also frequently susceptible to amoxicillin/clavulanate.[8]Brown-Elliott BA, Brown JM, Conville PS, et al. Clinical and laboratory features of the Nocardia spp. based on current molecular taxonomy. Clin Microbiol Rev. 2006 Apr;19(2):259-82.
https://journals.asm.org/doi/10.1128/cmr.19.2.259-282.2006
http://www.ncbi.nlm.nih.gov/pubmed/16614249?tool=bestpractice.com
However, because resistance patterns within the same species may differ, treatment will be individual to each patient and cannot be standardized.
Duration of treatment and secondary prophylaxis
Duration of therapy is variable and depends on the location of the lesions and the immune status of the patient. Primary cutaneous nocardiosis can be treated for 3-6 months. However, mycetoma requires more prolonged therapy. Pulmonary and disseminated nocardiosis without CNS involvement should be treated for at least 6 months.[20]Corti ME, Villafane-Fioti MF. Nocardiosis: a review. Int J Infect Dis. 2003 Dec;7(4):243-50.
https://www.ijidonline.com/article/S1201-9712(03)90102-0/pdf
http://www.ncbi.nlm.nih.gov/pubmed/14656414?tool=bestpractice.com
For patients with CNS involvement, treatment duration depends on clinical and imaging responses, but treatment should be continued for 1 year or longer. In HIV-infected patients with low CD4 T-cell count and patients who have received transplants, secondary prophylaxis with an active oral agent needs to be maintained until the immune status improves.[30]Biscione F, Cecchini D, Ambrosioni J, et al. Nocardiosis in patients with human immunodeficiency virus infection [in Spanish]. Enferm Infecc Microbiol Clin. 2005 Aug-Sep;23(7):419-23.
http://www.ncbi.nlm.nih.gov/pubmed/16159542?tool=bestpractice.com
Surgical treatment
Some locations of nocardiosis may require adjunctive surgical treatment, particularly for patients with brain abscesses. However, the optimal treatment approach has not been established, and Nocardia brain abscesses have been managed either conservatively, with aspirations, or with open craniotomy and enucleation.[54]Cecchini D, Ambrosioni JC, Gomez A, et al. Disseminated nocardiosis caused by Nocardia abscessus in an HIV-infected patient: first reported case. AIDS. 2005 Aug 12;19(12):1330-1.
https://journals.lww.com/aidsonline/fulltext/2005/08120/disseminated_nocardiosis_caused_by_nocardia.15.aspx
http://www.ncbi.nlm.nih.gov/pubmed/16052092?tool=bestpractice.com
[78]Valarezo J, Cohen JE, Valarezo L, et al. Nocardial cerebral abscess: report of three cases and review of the current neurosurgical management. Neurol Res. 2003 Jan;25(1):27-30.
http://www.ncbi.nlm.nih.gov/pubmed/12564122?tool=bestpractice.com
[79]Dahan K, El Kabbaj D, Venditto M, et al. Intracranial Nocardia recurrence during fluorinated quinolones therapy. Transpl Infect Dis. 2006 Sep;8(3):161-5.
http://www.ncbi.nlm.nih.gov/pubmed/16913975?tool=bestpractice.com
Thus, every case needs a careful multidisciplinary evaluation by the primary physician, the infectious diseases specialist, and the surgeon. Surgery or bronchoscopy can also be considered for draining pulmonary abscesses or empyemas.[7]Yildiz O, Doganay M. Actinomycoses and Nocardia pulmonary infections. Curr Opin Pulm Med. 2006 May;12(3):228-34.
http://www.ncbi.nlm.nih.gov/pubmed/16582679?tool=bestpractice.com
Cutaneous lesions and mycetoma may sometimes require surgical treatment, as well as some ocular infections and rare cases with other body site involvement.[80]Devi KR, Singh LR, Devi NT, et al. Subcutaneous nocardial abscess in a post-renal transplant patient. Indian J Med Microbiol. 2007 Jul;25(3):279-81.
http://www.ncbi.nlm.nih.gov/pubmed/17901652?tool=bestpractice.com
[81]Lichon V, Khachemoune A. Mycetoma: a review. Am J Clin Dermatol. 2006;7(5):315-21.
http://www.ncbi.nlm.nih.gov/pubmed/17007542?tool=bestpractice.com
[82]Clare G, Mitchell S. Iris root abscess and necrotizing sclerokeratitis caused by Mycobacterium abscessus and presenting as hemorrhagic anterior uveitis. Cornea. 2008 Feb;27(2):255-7.
http://www.ncbi.nlm.nih.gov/pubmed/18216592?tool=bestpractice.com
[83]Gokhale NS, Garg P, Rodrigues C, et al. Nocardia infection following phacoemulsification. Indian J Ophthalmol. 2007 Jan-Feb;55(1):59-61.
https://www.ijo.in/article.asp?issn=0301-4738;year=2007;volume=55;issue=1;spage=59;epage=61;aulast=Gokhale
http://www.ncbi.nlm.nih.gov/pubmed/17189890?tool=bestpractice.com
[84]Gates JD, Warth JA, McGowan K. Nocardia asteroides-infected aneurysm of the aorta: case report and review of the literature. Vascular. 2006 May-Jun;14(3):165-8.
http://www.ncbi.nlm.nih.gov/pubmed/16956490?tool=bestpractice.com
[85]Shah HR, Zamboni WA, Khiabani KT. Nocardial septic arthritis of the wrist diagnosed and treated by arthroscopy. Scand J Plast Reconstr Surg Hand Surg. 2005;39(4):252-4.
http://www.ncbi.nlm.nih.gov/pubmed/16208791?tool=bestpractice.com