Autoimmune Oophoritis: Clinical Presentation of an Unusual Clinical
Total Page:16
File Type:pdf, Size:1020Kb
Page 1 of 5 Case report Autoimmune oophoritis: clinical presentation of an unusual clinical entity Obstetrics & Gynecology M Varras1*, A Anastasiadis2, J Panelos3, E Balassi3, A Demou3, CH Akrivis4 Abstract infertility in women before the age and had regular menses on a 28-day Introduction of 40 years1-4. The aetiologies of this cycle. She had five full-term normal condition include chromosomal pregnancies without any miscar- anomalies (such as X chromosome riages. Autoimmune oophoritis is a rare co- monosomy, translocations or partial On pelvic bimanual examination, ndition, which provokes ovarian fail- deletions), genetic predisposition an anteverted uterus was palpated, ure with either primary amenorrhea (such as fragile X pre-mutations, while both fallopian tubes and ovaries or secondary amenorrhea and a sub- BMP15 or DIAPH2 mutations), infec- were impalpable. Pelvic ultrasonog- sequent loss of fertility and ovarian tious diseases, complications of chem- raphy disclosed normal-sized ovaries hormonal function. The purpose of t- otherapy, pelvic radiotherapy, surgical with bilateral multicystic structures, his report is to document the clinical interventions or surgery, enzymatic with the largest follicle measuring Casesfindings report from two patients with aut- disorders and endometriosis. Prema- 1.2 cm. The thickness of the endo- oimmune oophoritis. ture ovarian failure might also be idio- metrium was 3.5 mm. Initial inves- manuscript. nal pathic or autoimmune2. Among a total tigation showed her serum gonado- fi Two cases of autoimmune oophorit- of 266 patients with spontaneous POF, tropin concentrations to be elevated: is are presented whose histopathol- 4% were diagnosed to have autoim- follicle stimulating hormone (FSH) at ogical findings were consistent with mune oophoritis3,4. 40 mIU/ml and luteinizing hormone international literature. Both cases Ovarian autoimmunity was first (LH) at 56 mIU/ml. 17-beta oestra- were histopathologically characteri- reported and serologically docu- diol was at 30 pg/ml (normal range: sed by lymphocytic and plasmacytic mented by Vallotton and Forbes in 30–100 pg/ml). Serum prolactin inflammatory infiltrations around t- 19665. Autoimmune oophoritis is a levels were normal and there was he cystic follicles. The inflammation distinct clinical entity and one of the no evidence of hypoparathyroidism. Conclusionwas located both in the theca and gr- causes of POF, particularly in women Thyroid function tests were normal. anular layers. with secondary amenorrhea6-8. Auto- Also, kidneys and liver function tests immune oophoritis generally occurs were normal. Serum electrolytes Patients with autoimmune oophorit- in the setting of autoimmune poly- were normal. Proteinuria was nega- is should be recognised by the histo- endocrine syndromes and is asso- tive. Serum adrenocorticotropin pathology of the ovarian biopsies as ciated commonly with other major hormone levels were normal. The they are at an increased risk of deve- endocrine failures such as diabetes progesterone challenge test was loping other autoimmune disorders. mellitus, Addison’s disease, hypopar- negative. Introduction athyroidism or hypothyroidism8-10. A At exploratory laparoscopy, her wide clinical spectrum has also been ovaries appeared small and inactive. Premature ovarian failure (POF) is on of the manuscript, as well read and approved a condition characterised by amen- demonstrated11. There were no signs of abdominal or Ɵ orrhea, some hot flushes, elevated The aim of this was to pelvic inflammatory processes. Biop- serum gonadotropin levels and describe the clinical spectrum and sies from both ovaries were obtained hypo- oestrogenism with associated the interesting pathologicalreport findings and endometrial curettage was originating from small ovarian biop- performed as well. sies from two patients with autoim- Grossly, two specimens had been * Corresponding author Email: [email protected] mune oophoritis. obtained from the right ovary with none declared. ict of Interests: a whitish-grey colour, measuring fl 1 Third Department of Obstetrics and Gynecol- on, design, and prepara ogy, ‘Elena Venizelou’ General Maternity Hos- Case report 0.7 cm and 1.2 cm in their greatest Ɵ pital, Athens, Greece Case 1 dimension, respectively, and one 2 rules of disclosure. (AME) ethical Ethics Medical on for Fourth Department of Obstetrics and Gyne- A 33-year-old woman presented with specimen had been obtained from Ɵ cology, ‘Elena Venizelou’ General Maternity Hospital, Athens, Greece a 10-month history of secondary the left ovary with white tan to 3 Department of Pathology, ‘G. Chatzikosta’ amenorrhoea and hot flushes. grey colour measuring 1.6 cm in its General State Hospital, Ioannina, Greece Menarche had occurred at an age of 14 greatest dimension. Also, the spec- 4 Department of Obstetrics and Gynecology, ‘G. Chatzikosta’ General State Hospital, Ioannina, years, she had developed secondary imen from the endometrial biopsy Greece sex characteristics appropriately measured 3.5 × 3 × 0.2 cm. At micro- Licensee OA Publishing London 2013. Creative Commons Attribution Licence (CC-BY) none declared. Con ng interests: Ɵ F : Varras M, Anastasiadis A, Panelos J, Balassi E, Demou A, Akrivis CH. Autoimmune oophoritis: All authors abide by the Associa All authors contributed to the concep to All authors contributed clinical presentation of an unusual clinical entity. OA Case Reports 2013 Jan 31;2(1):7. Compe Page 2 of 5 Case report Figure 1: Case 1: dense inflammation of Figure 3: Case 1: primordial folli- Figure 5: Case 1: T8 positive lympho- the ovary (haematoxylin-eosin × 100). cles are spared by the inflammation cytes (CD8 stain × 200). (haematoxylin-eosin × 400). nal manuscript. nal fi Figure 2: Case 1: dense inflamma- Figure 6: Case 1: CD138 positive tory infiltrate destroys the follicle Figure 4: Case 1: T4 positive lympho- plasma cells (× 100). (haematoxylin-eosin × 200). cytes (CD4 stain × 200). history of hypothyroidism; she was was i dentified in the endometrium. scopic examination, the ovarian biop- stable on thyroxine 0.1 mg daily. The final pathological diagnosis was sies contained primordial, developing On pelvic bimanual examination, autoimmune oophoritis. and atretic follicles. Dense inflamma- an anteverted uterus was palpated The patient was diagnosed as tion of the ovary was found (Figure and a painful cystic mass in the having POF due to autoimmune 1). The most striking feature was right adnexa was palpated as well. oophoritis, and she was started on the destruction of the developing Pelvic ultrasonography revealed oestrogen and progesterone replace- follicles by lymphocytic and plasma- a well-defined cystic mass with a ment therapy. Laboratory tests were cytic infiltration (Figure 2), while the hypoechogenic appearance in the negative for rheumatoid factor, anti- primordial follicles were apparently right adnexa uteri, measuring 8 cm nuclear antibodies (ANA), compo- and entirely spared from this process in its maximum diameter and a on of the manuscript, as well read and approved nents 3 and 4 of the complement (C3 Ɵ (Figure 3). The inflammation was small multicystic structure in the left and C4), immunoglobulin (Ig) G and located both in the theca and granular adnexa. Free liquid in the posterior IgM alpha cardiolipin antibodies and layers. The ovarian stroma was unre- space of Douglas was not found. C-reactive protein (CRP). The patient markable. Immunohistochemically, Initial investigation showed was missed from further follow-up. the inflammatory population was normal blood cell counts, as well composed mainly from T-lympho- Case 2 as normal function tests for liver, cytes (CD4+, CD8+) (Figures 4 A 32-year-old woman presented with kidneys and thyroid. The INR and none declared. ict of interests: and 5) and plasma cells (CD138+) acute lower abdominal pain. Last activated partial thromboplastin fl on, design, and prepara (Figure 6). The endometrium had menstrual period was three months time ratios were normal. Elevated Ɵ an oedematous appearance with previous to the date of presentation. levels of serum carbohydrate antigen rules of disclosure. (AME) ethical Ethics Medical on for focal haemorrhagic infiltration of the Menarche had occurred at the age (CA)-19.9 (81 IU/ml, normal range Ɵ stroma. The endometrial glands were of 15 years and her menstrual cycle <37) and CA-125 (39.7 U/ml, normal small, round, coated with cylinder had been regular previously on a range: <37) were found, while the epithelium and without any remark- 29-day cycle. She had two previous serum levels of CA-15.3, carcinoem- able mitotic activity. No inflammation caesarean sections and a medical bryonic antigen and alpha fetoprotein Licensee OA Publishing London 2013. Creative Commons Attribution Licence (CC-BY) none declared. Con ng interests: Ɵ F : Varras M, Anastasiadis A, Panelos J, Balassi E, Demou A, Akrivis CH. Autoimmune oophoritis: All authors abide by the Associa All authors contributed to the concep to All authors contributed clinical presentation of an unusual clinical entity. OA Case Reports 2013 Jan 31;2(1):7. Compe Page 3 of 5 Case report Figure 7: Case 2: severe inflamma- Figure 9: Case 2: characteristic Figure 11: Case 2: PanT (CD45RO) tory infiltrate destroys the follicle destruction of the developing follicle (haematoxylin- eosin × 200). by the inflammatory infiltration stain × 100. (haematoxylin-eosin × 400). virus. Also, the serum Ig concentra- tions were negative for IgM (162 mg/ dl; normal range: 46–304 mg/dl) and IgA (165 mg/dl; normal range: 82–453 mg/dl). Positive serum labo- ratory tests were found for antistrep- nal manuscript. nal tolysin O (333 IU/ml; normal range: fi 0–116 IU/ml) and IgG (1730 mg/dl; normal: 751–1560 mg/dl). Also, the titres of ANA were positive. During the six-month follow-up Figure 8: Case 2: dense inflamma- postoperatively, her serum gonado- tory infiltrations around the follicle tropins were slightly elevated with (haematoxylin-eosin × 200). Figure 10: Case 2: layers of granu- FSH levels of 15.8 mIU/ml and losa cells surround an unremark- LH levels of 18.4 mIU/ml. 17-beta able oocyte in a secondary follicle were in normal ranges. The patient (haematoxylin-eosin × 400).