Granulomatosis with Polyangiitis-Mimicking Advanced Gynecological Cancer: A Case Report and Systematic Review of the Literature

Abstract

(1) Background: Granulomatosis with polyangiitis (GPA) is a necrotizing vasculitis that mimics gynecologic cancer. In GPA patients, the genitourinary system is affected in <1%. The objective of the study was to provide a systematic review of the literature of GPA patients with gynecological involvement. (2) Methods: PubMed and Embase were searched from inception to July 2021 for GPA patients with gynecological involvement Medical Subject Headings (MeSH) and free-text terms. Exclusion criteria were other language, review articles, pregnancy, fertility, or male patients. Data were extracted on clinical evolution, symptoms, examinations findings, diagnosis delay, treatment, outcome, patient status, and follow-up. (3) Results: Seventeen studies included data from patients with GPA and primary or relapsed gynecological involvement. 68% of the authors of this review thought the patient had cancer. The main gynecological symptom is bleeding, but exclusive gynecologic symptomatology is rare (ENT: 63%, lungs: 44%, kidneys-urinary tract: 53%). GPA could affect all areas of the genital tract, but the most frequent location is the uterine cervix. Medical treatment for GPA is effective. (4) Conclusions: GPA of the female genital tract must be considered when biopsies of an ulcerated malignant-appearing cervical or vaginal mass are negative for malignancy even when they are unspecific. Rheumatology consultation is indicated.

Not Everything Is as It Seems: A Case Series and Overview of Diseases Mimicking Antineutrophil Cytoplasmic Antibody-Associated Vasculitis

“…Diagnosing AAV may be straightforward in patients with typical clinical features, such as a pulmonary-renal syndrome in the presence of ANCA and/or histological confirmation of small-vessel vasculitis. However, establishing the diagnosis can be challenging in patients with atypical and/or rare disease manifestations, for instance cerebral vasculitis, gynecological or myocardial involvement, as we have encountered in our clinic and has been reported by others [4][5][6]. Positive ANCA serology or histologic confirmation in these cases are considered essential.…”

Rare cause of diffuse alveolar haemorrhage and subconjunctival haemorrhage rescued by extracorporeal membrane oxygenation and rituximab

“…It can affect all kinds of small blood vessels. The diversiform characteristics of GPA can involve almost all body systems, particularly the lungs and kidneys 6. Pulmonary haemorrhagic nephritis syndrome is one of the most frequent cause of acute respiratory failure, and supervision in ICU with advanced respiratory support and renal replacement treatment is extremely useful to help those patients recuperate 7 8.…”

An Unusual Case of Vulvar Involvement in the Course of Granulomatosis with Polyangiitis

“…Genitourinary involvement in GPA occurs in less than 1% of cases [ 9 ]. In most of the described cases involving genitourinary involvement, gynecologic malignancy was considered the first possible diagnosis [ 10 ]. It is worth noting that more frequently than GPA of the vulva, cancer in this area is observed.…”