Medicina, Vol. 57 No. 3, 2021.
Recenzija, Prikaz slučaja
https://doi.org/10.21860/medflum2021_261195
Hypertrophic pachymeningitis and aortitis as manifestations of immunoglobulin G4-related disease, successfully treated with rituximab: case report
Ana Šević
; Sveučilište u Rijeci, Medicinski fakultet, Rijeka, Hrvatska
Dragan Javoran
; Klinički zavod za radiologiju, Klinički bolnički centar Rijeka, Rijeka, Hrvatska
Felina Anić
; Sveučilište u Rijeci, Medicinski fakultet, Rijeka, Hrvatska; Zavod za reumatologiju i kliničku imunologiju, Klinički bolnički centar Rijeka, Rijeka, Hrvatska
Srđan Novak
orcid.org/0000-0001-8870-4704
; Sveučilište u Rijeci, Medicinski fakultet, Rijeka, Hrvatska; Zavod za reumatologiju i kliničku imunologiju, Klinički bolnički centar Rijeka, Rijeka, Hrvatska
Sažetak
Aim: To present a patient with immunoglobulin G4-related disease (IgG4-RD), diagnostic challenges associated with it and therapeutic options with emphasis on rituximab treatment. Case report: A 60-year-old patient was admitted for hospital treatment after an examination at the gastroenterology clinic he was admitted to due to irregular stools, weakness and loss of 20 kg within two months. The patient had a lesion of the right facial nerve, ptosis, lagophthalmos and difficult swallowing. The examination confirmed lesions of the right oculomotor, facial and trigeminal nerve, and paresis of the right vocal cord. Laboratory findings showed anemia and elevated inflammatory markers. An ultrasound-detected liver lesion was defined as hemangioma. Computed tomography (CT) and magnetic resonance imaging (MRI) of the brain showed hypertrophic pachymeningitis in the middle and posterior cranial fossa, along with the right tentorium, cavernous sinus, Meckel’s pit, and upper orbital fissure. CT aortography showed infrarenal aortitis. Serum IgG4 antibody levels were elevated, IgG4-RD criteria were met and methylprednisolone therapy was initiated. The following year he was re-hospitalized for reevaluation of the disease, MRI of the brain showed regression of hypertrophic dura tissue, but CT-aortography incomplete regression of the disease and inflammatory parameters were still high. Rituximab treatment was introduced which led to remission. Conclusion: IgG4-RD is a rare and complex disease that often mimics others and presents a major diagnostic challenge, so the knowledge of this disease would contribute to its early detection and better therapeutic outcomes. Although glucocorticoids are standard therapy, they often do not give the desired results, and rituximab treatment shows excellent efficacy.
Ključne riječi
aortitis; hypertrophic pachymeningitis; IgG4-RD; immunoglobulin G4-related disease; rituximab
Hrčak ID:
261195
URI
Datum izdavanja:
1.9.2021.
Posjeta: 1.332 *