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Aim of the work: To detect the frequency of secondary amenorrhea in a cohort of Egyptian systemic lupus erythematosus (SLE) patients and to identify potentially related key-players. Patients and methods: The medical records of 140 adult SLE patients above 18 and less than 40 years were revised regarding clinical characteristics, immunological features and medications received. The SLE disease activity index (SLEDAI) and damage index (SLICC-DI) were recorded. Patients were divided into two groups: patients who developed secondary amenorrhea and those having normal menstrual cycles. Results: The mean age of the patients was 26.8 ± 5.7 years, the median disease duration was 3.5 years. The patients had renal involvement in 69.3%, neuropsychiatric in 23.6% and cardiac in 37.9%. Cyclophosphamide (CYC) was received by 52.1% and steroids by 139 patients. Secondary amenorrhea was found in 44 patients (31.4%); 36 (81.8%) of them were ≤32 years old. Patients with secondary amenorrhea had a higher frequency of positive anti-double stranded deoxyribonucleic acid (ds-DNA) antibodies (75% vs 50%; p = 0.04) and a shorter disease duration (median 2 years vs 4.5 years; p = 0.005). Lupus nephritis, renal failure, neuropsychiatric manifestations, cardiac involvement and receiving CYC and high-dose steroids tended to be more frequent in those with secondary amenorrhea compared to those without. The median SLICC-DI was higher in those with amenorrhea compared to those without (p = 0.2) Conclusion: Cyclophosphamide is not the only key-player for the development of secondary amenorrhea in SLE; other factors as the shorter disease duration, presence of autoantibodies and major organ involvements may be implicated. Keywords: Systemic lupus erythematosus, Secondary amenorrhea, Egyptian, Disease duration, Anti ds-DNA

Highlights • Vesicouterine fistula is pathological communication between the bladder and uterus or cervix. • Symptoms of VUF were secondary amenorrhea and cyclic hematuria with history of previous cesarean section (concluded as Youssef’s syndrome). • Surgical approach is contemplated as definitive management of the VUF following caesarean section. • A group of women was able to have normal period after fistula repair., Vesicouterine fistula (VUF) is an abnormal communication between the bladder and uterus, occurring 1–4% of all urogenital fistulas. Diagnosis is still a challenge because symptoms may appear late or fistula may be missed even after repeated examination. A 37-year old woman who has two children born through caesarean section complained of the absence of menstruation for the past three years. At the same time point, she experienced cyclic haematuria and amenorrhea. The diagnosis was made through ultrasonography, cystoscopy and hysteroscopy. She was then managed with laparoscopic bladder fistula repair continued with total laparoscopic hysterectomy. The VUF can present as an undesirable consequence of caesarean section.

The “resistant ovary syndrome” or “Savage syndrome” is a rare cause of hypergonadotropic hypogonadism, in which the ovaries are resistant to follicle-stimulating hormone (FSH), leading to a primary or secondary amenorrhea with high gonadotropin levels (FSH and Luteinizing hormone (LH)) despite a normal ovarian follicle pool.

Failure of germ cell development is associated with complete ovarian failure, resulting in lack of secondary sexual pubertal development (primary amenorrhea). A decreased number but not a total absence of germ cells is more likely associated with premature ovarian failure, presenting with infertility or secondary amenorrhea. Yet complete and premature ovarian failure may be different manifestations of the same underlying pathogenic and etiologic processes. Many different genetic mechanisms are pertinent to the processes m chromosomal abnormalities, Mendelian mutations of autosomal or X-linked genes, and polygenic/multifactorial factors. In the absence of the Y chromosome, the indifferent embryonic gonad always develops into an ovary. Germ cells exist in 45,X human fetuses. Oocyte development initially exists even in 46,XY phenotypic females, such as in infants with XY gonadal dysgenesis or the genito-palatocardiac syndrome. Oocyte development in the presence of a Y chromosome is also well documented in mice. Thus, the pathogenesis of germ cell failure in humans can be deduced to be increased germ cell attrition. If two intact X chromosomes are not present, ovarian follicles in 45,X individuals usually degenerate by birth.

Functional hypothalamic anovulation or amenorrhea is the cessation of menses and ovulation in women with no identifiable organic cause for amenorrhea. This common and, in theory, reversible condition accounts for 15–35% of secondary amenorrhea.

Background Burkitt's lymphoma (BL) occurs mostly in children. Isolated bilateral ovarian involvement presenting with amenorrhea is extremely rare in young adults. Case A 24-year-old female presented with secondary amenorrhea. Bilateral adnexal masses were identified on physical examination and abdominal computed tomography (CT). She underwent total abdominal histerectomy and bilateral salpingoophorectomy. Histopathologic evaluation yielded a diagnosis of BL of ovaries. Combined chemotherapy was administered. After complete remission an autologous bone marrow transplantation (ABMT) was performed. She died 35 days after ABMT. Conclusion Although rare, BL should be kept in mind when isolated ovarian tumors are detected in young patients.

Publisher Summary Amenorrhea means absence of menstruation. Primary amenorrhea means that menstruation has never occurred. The term secondary amenorrhea is usually used to mean that menstruation has occurred in the past but not for at least 6 months. Amenorrhea may be physiological or pathological. If a girl presents at the age of 14 with primary amenorrhea, a full history should be taken and a general and vulval examination should be performed. Height and weight should be noted and the ponderal index should be calculated. If there are no signs of puberty, investigation and management for delayed puberty should be initiated. This chapter discusses primary amenorrhea at 16 years of age. If a girl presents at the age of 16 with primary amenorrhea, detailed investigation is indicated because there is an increased risk of malignancy if the gonads are not removed in a girl with a Y chromosome. If the gonads fail to develop, raised gonadotrophin levels will be found in an untreated teenager or adult although they may be low in mid-childhood. An abnormality of the sex chromosomes will usually be found.