Table 1.

Overview of previous PML reports in FAE-treated psoriasis patients.

Case reference	Age, sex	Underlying conditions with immunocompromising potential	Duration of FAE treatment prior to PML onset	Other immunosuppressive treatments within 5 years prior to PML onset	Grade of lymphopenia a at PML onset	Duration of lymphopenia prior to PML onset	PML treatment	Outcome
Present case report	52-year-old male	Psoriasis, hypopharyngeal carcinoma, and alcohol abuse	2 years	Chemotherapy with 5-fluorouracil and cisplatin (terminated 17 months prior to PML onset), nivolumab (last cycle 10 months prior to PML onset), and docetaxel (terminated 5 months prior to PML onset)	4	18 months	Discontinuation of FAE treatment and off-label use of mirtazapine and mefloquine	Partial recovery from clinical symptoms, remission of MRI lesions, decrease in CSF JCV DNA titer and in sNfL levels
van Oosten et al (2013) 27	42-year-old female	Psoriasis	5 years	None	3	5 years	Discontinuation of FAE treatment and off-label use of mirtazapine and mefloquine	Development of PML-IRIS followed by partial clinical recovery
Ermis et al (2013) 28	74-year-old male	Psoriasis	3 years	Methotrexate (terminated 3 years prior to PML onset)	3	2 years	Discontinuation of FAE treatment and off-label use of mirtazapine and mefloquine	Development of PML-IRIS, followed by partial remission of clinical signs and MRI findings
Buttmann et al (2013), Sweetser et al (2013)29,30	60-year-old female	Psoriasis, and pulmonary sarcoidosis	3 years	Prednisolone and methotrexate (terminated 3.5 years prior to PML onset)	2–3	20 months	No information available	Partial recovery with mild-to-moderate residual symptoms
Stoppe et al (2014), Sweetser et al (2013)15,30	Male patient, no information on age available	Psoriasis, and superficial spreading melanoma	3 years	Efalizumab (terminated 3 years prior to PML onset)	2–3	No information available	Discontinuation of FAE treatment and off-label use of mirtazapine, mefloquine, and immunoglobulins	Partial recovery
Bartsch et al (2015) 12	68-year-old male	Psoriasis, and adenocarcinoma of the rectum 8 years earlier (adjuvant radiochemotherapy with 5-fluorouracil)	2.5 years	None	2	16 months	Discontinuation of FAE treatment and off-label use of mirtazapine and mefloquine	Partial recovery
Nieuwkamp et al (2015) 13	64-year-old female	Psoriasis	2 years	None	2	1 month	Discontinuation of FAE treatment and off-label use of mirtazapine, mefloquine, and steroids	Deceased
Dammeier et al (2015) 31	53-year-old female	Psoriasis	1.5 years	None	2–3	No information available	Discontinuation of FAE treatment and off-label use of mirtazapine and mefloquine	Stable disease course with only mild residual deficits at 7-month follow-up
Hoepner et al (2015) 32	69-year-old male	Psoriasis, and monoclonal gammopathy	4 years	None	2–3	At least 18 months	Discontinuation of FAE treatment and off-label use of mirtazapine and mefloquine	Development of mild PML-IRIS followed by partial recovery
Elsner et al (2020) 33	58-year-old female	Psoriasis	4 years	No information available	No information available	No information available	Discontinuation of FAE treatment and antiviral treatment (not specified)	Persisting global aphasia and recurrent seizures
Case series by Gieselbach et al (2017) 14	57-year-old male	Psoriasis	4 years	No information available	1–3	4 years	No information available	Survived
Case series by Gieselbach et al (2017) 14	50-year-old female	Psoriasis	9 years	No information available	2–3	6 years	No information available	Survived
Case series by Gieselbach et al (2017) 14	71-year-old male	Psoriasis	1.5 years	No information available	3	No information available	No information available	Survived
Case series by Gieselbach et al (2017) 14	64-year-old female	Psoriasis, breast carcinoma (10 years prior to PML)	.5 years	Cyclophosphamide	3	No information available	No information available	Survived

Abbreviations: CSF: cerebrospinal fluid, DNA, desoxyribonucleic acid; FAE, fumaric acid ester; IRIS, immune reconstitution inflammatory syndrome; JCV, JC virus; PML, progressive multifocal leukoencephalopathy.

^aGrade 1 lymphopenia = 800–100 cells per mm³, Grade 2 lymphopenia = 500–800 cells per mm³, Grade 3 lymphopenia = 500–200 cells per mm³, Grade 4 lymphopenia <200 cells per mm³.