ABSTRACT
INTRODUCTION AND IMPORTANCE: Endometriomas are the most common presenting subtype of endometriosis. Although most endometriomas are asymptomatic, patients can rarely present acutely with spontaneous rupture causing diffuse peritonitis and severe systemic inflammatory response. CASE PRESENTATION: Here we describe a case of ruptured endometriomas in a 26-year-old nulligravid female with a history of heavy menses, progressive abdominal distension, and a recent urinary tract infection. The patient presented to the emergency department with upper abdominal pain radiating to her back with associated nausea. Computed tomography (CT) scan demonstrated diffuse ascites with a large, multilobulated, and multicystic septated mass arising in the right pelvis and extending into the lower abdomen. Findings were concerning for peritoneal carcinomatosis and the patient was admitted for evaluation. She developed progressive signs of sepsis and was emergently brought to the operating room for surgical exploration on hospital day (HD) number two. She was found to have ruptured pelvic cysts arising from both ovaries with diffuse contamination of the abdomen by cyst contents and bilateral salpingo-oophorectomy (BSO) was performed. Final pathology demonstrated benign bilateral endometriomas. CLINICAL DISCUSSION: Endometrioma rupture is extremely rare and imaging findings may appear to represent disseminated peritoneal malignancy. CT findings demonstrating a pelvic mass with concurrent ascites should raise clinical suspicion for ruptured endometrioma, particularly in younger patients. CONCLUSION: Prompt surgical exploration and complete resection of pathologic tissue may be necessary for diagnosis and treatment in some patients with clinical deterioration related to perforated endometriomas. Combined oral contraceptives are recommended in the postoperative period.
ABSTRACT
BACKGROUND: Current first-line anti-proteinuric treatments for nephrotic syndrome (NS) do not produce an effective response in all patients and are not tolerated by some patients. Additional effective and tolerable treatment options in NS are strongly needed. This retrospective case series is the largest to date to examine Acthar gel (adrenocorticotropic hormone, ACTH) in patients with varied-etiology NS. METHODS: This multicenter retrospective case series included adult patients with NS (N = 44) treated with Acthar gel at 6 clinical practices. NS etiologies included idiopathic focal segmental glomerulosclerosis (FSGS, 15), idiopathic membranous nephropathy (iMN, 11), IgA nephropathy (IgAN, 5), diabetic nephropathy (DN, 4), systemic lupus erythematosus class V membranous lupus nephritis (MLN, 2), minimal change disease (MCD, 2), membranoproliferative glomerulonephritis (MPGN, 1), fibrillary glomerulonephritis (FGN, 1), and unbiopsied NS (3). Proteinuria response was assessed as percent reduction from baseline and percent of patients meeting complete remission (final proteinuria <500 mg/d), partial remission (≥50 % reduction in proteinuria from baseline and final proteinuria 500-3500 mg/d), clinical response (≥30 % reduction in proteinuria from baseline that did not meet criteria for complete or partial remission), and no response (failed to meet remission or clinical response criteria) following Acthar gel therapy. Safety and tolerability were examined using adverse event (AE) frequency reported by patients or treating nephrologists and frequency of early discontinuation of treatment due to AEs. RESULTS: 68.2 % (30/44) of patients had received prior NS treatment with immunosuppressive or cytotoxic therapies. Thirty-seven patients completed Acthar gel treatment. Seven patients (15.9 %) had early termination due to AEs, including weight gain (2), hypertension (2), edema (1), fatigue (1), seizures (1) and for reasons not stated (2). Proteinuria reduction ≥30 % was shown in 81.1 % (30/37) of patients and 62.2 % (23/37) showed ≥50 % proteinuria reduction. Proteinuria responses were greatest in MCD (n = 2/2 complete remission), MLN (n = 2/2 partial remission), MPGN (n = 1/1 partial remission), FSGS (n = 12/15 [80.0 %] partial remission or clinical response), and iMN (n = 8/11 [72.7 %] complete remission, partial remission, or clinical response). CONCLUSIONS: Acthar gel may meet an important treatment need in patients with treatment-resistant NS in response to first-line therapies, patients unable to tolerate first-line therapies, and in patients with advanced disease.
