Dados do Trabalho

Título

Disseminated histoplasmosis as a diagnostic challenge in an immunosuppressed patient

RESUMO

Case Report
A 38-year-old female patient with a history of systemic lupus erythematosus presented with spastic paraparesis, cognitive dysfunction and urinary incontinence. She underwent head CT and MRI, which showed communicating hydrocephalus. CSF analysis revealed monocytic pleocytosis (45 nc/µl), hypoglycorrhachia (12 mg/dL), significant increase in proteins (733 mg/dL) and was negative for cryptococcus, geneXpert, VDRL and bacterial and fungi cultures. Chest and abdomen CT evidenced calcified perihilar and abdominal lymph nodes, most indicative of tuberculosis (TB) or sarcoidosis. After full workup and multiple blood and CSF tests, TB was ruled out and the patient was treated with corticosteroids and cyclophosphamide, with no improvement. Despite regular immunosuppression, she continued to deteriorate. New CSF analysis kept same previous pattern. Blood radial immunodiffusion was positive for Histoplasma capsulatum. Liposomal amphotericin B was started, followed by oral itraconazole. After three months of treatment, she presents with mild cognitive deficit and discrete gait disturbance.
Discussion
Histoplasma capsulatum incidence rate has increased with the advance of immunosuppressive therapies in recent years, especially in endemic areas. Up to 10% of patients develop disseminated histoplasmosis via hematogenous dissemination, leading to meningitis, associated vasculitis, focal brain or spinal lesions, or hydrocephalus.
Diagnostic tools are mostly nonspecific, and diagnosis relies on strong clinical suspicion, which may be a challenge. For our patient, it came up after ruling out TB, sarcoidosis and lupus hydrocephalus. CSF analysis usually shows monocytic pleocytosis, elevated protein and low glucose. CSF culture has poor sensitivity, and antigen and antibody testing in CSF are not widely available in our scenario. However, urine and blood antigen testing are available, sensitive and highly specific.
Pharmacologic treatment should be maintained for at least 1 year, until CSF profile is resolved. If possible, it is ideal to stop immunosuppressive therapies. Relapses are frequent and may require prolonged treatment.
Final Comments
Untreated histoplasmosis is associated with high morbidity and mortality rates. Diagnosis is critical for adequate treatment implementation and better outcomes. It is even more important to accurately diagnose fungal infection in patients with autoimmune diseases, since treatment plans are opposite.

Palavras Chave

histoplasmosis; opportunistic infection; hydrocephalus