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CNS (Central Nervous System) Paracoccidioidomycosis as a differential diagnosis of IgG4 – disease related : case report.

RESUMO

Male,38 years old, healthy, motoboy,from Belo Horizonte,MG.In Mid 2020,he presented headache,xeroftalma and perceived two small bilateral cervical nodules in addition to intermittent paresthesias in fingers,hands and face and seizures.He also had type B symptoms(weight loss, frequent sweating and fever spikes).He looked for a neurologist who requested an MRI,EEG and referred him to hospital.He received pulse therapy with1g methylprednisolone for five days, with partial improvement in his neurological condition.A single dose of IV cyclophosphamide(1g)was prescribed.The laboratory showed an increase in IgG4(above 8000),with a brain MRI report suggesting Igg4-related disease.The Rheumatology evaluation ruled out other rheumatologic diseases.The biopsy of cervical lymph node revealed paracoccidioidomycosis(PCM), and treatment with amphotericin B deoxylate was initiated.After treatment, there was significant clinical and radiological improvement,with a drop in serum IgG4 levels.DISCUSSION:The IgG4-related disease has various clinical manifestations, with possible involvement of the pancreatobiliary tract, retroperitoneum, head and neck organsand glands. It could mimic neoplastic, inflammatory and infectious conditions.Histopathology remains the key to diagnosis because reliable biomarkers are lacking.IgG4-related disease is treated with glucocorticoids but can even lead to death if not recognized.However, to get accurate diagnosis of IgG4-related disease, it is mandatory to exclude infectious, neoplastic and rheumatologic diseases. On the other side, PCM, a systemic infection caused by the fungus Paracoccidioides brasiliensis, affects individuals in all age groups.The lung is the most affected organ, and it may present on the CNS.Santos et al,2012, evaluated the levels of IgG, IgG 1, IgG 2, IgG 3 and IgG4 in 54 patients with PCM during and after treatment.Only the igg4 subtype showed a negative correlation between its levels and clinical improvement during treatment.Overall, 65% of patients showed reactivity against IgG 4 when Mexo antigen was used and this reactivity decreased over the course of treatment.The antigen can be used in the measurement of total igg levels and its subclasses to monitor patients during treatment.Conclusion:it's imperative to identify and exclude differential diagnosis on suspecting IgG4-related disease,due its lack of other biomarkers and the clinical and laboratory charachteriustics may be mimicked by other pathologies.