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| Year : 2015 | Volume
: 58
| Issue : 2 | Page : 249-251 |
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| Autoimmune oophoritis: A rarely encountered ovarian lesion |
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Sunitha Jacob1, Murat Koc2
1 Department of Pathology, Bahrain Specialist Hospital, Juffair, Manama, Bahrain
2 Department of Gynecology, Bahrain Specialist Hospital, Juffair, Manama, Bahrain
Click here for correspondence address and email
| Date of Web Publication | 17-Apr-2015 |
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 Abstract | | |
Autoimmune oophoritis is a rare disorder causing ovarian failure clinically characterized by amenorrhea and infertility. It often occurs in a setting of autoimmune polyendocrine syndromes. A 38-year-old female presented with a 3 years history of secondary amenorrhea. She was on treatment for Hashimoto's thyroiditis and Addison's disease. The ovaries were cystic and histologically featured by folliculotropic lymphoplasmacytic inflammatory infiltrate concentrated in the theca interna layer of developing follicles, but sparing the primordial follicles. Keywords: Autoimmune disease, autoimmune oophoritis, ovary
How to cite this article:
Jacob S, Koc M. Autoimmune oophoritis: A rarely encountered ovarian lesion. Indian J Pathol Microbiol 2015;58:249-51 |
Autoimmune oophoritis (AIO) is a rare disorder with only about 30 cases documented in the literature. It is characterized by an autoimmune inflammation of ovaries resulting in their destruction, atrophy and fibrosis. It involves a complex interplay of humoral and cellular mechanisms, which provokes premature ovarian failure. [1],[2],[3],[4]
A 38-year-old female presented with a 3 years history of secondary amenorrhea associated with hot flushes. Her past medical history was significant for Hashimoto's thyroiditis and Addison's disease for which she was on treatment. Ultrasonography of the abdomen revealed a normal sized uterus with thin endometrial lining and bilateral thin walled cystic ovaries. The left ovary was enlarged, measured 6.8 cm × 4.6 cm × 4.2 cm with delicate intercystic septae. The right ovary measured 2.6 cm in maximum dimension. The serum FSH levels were mildly elevated, whereas LH and prolactin levels were normal. She was given an empirical course of oral contraceptives for 3 months, but failed to resume normal menstrual cycles.
Laparoscopic biopsies were taken from the left ovary. Microscopic examination revealed variable sized maturing cystic follicles and few primordial follicles. The eye-catching feature was the presence of broad winding trabeculae of folliculotropic lymphoplasmacytic inflammatory infiltrate around the maturing follicles [Figure 1] and [Figure 2].The inflammatory response was seen almost selectively attacking the theca interna layer [Figure 3] and [Figure 4]. Denser inflammation with a greater predominance of plasma cells and invasion of the granulosa cells was present in the more advanced follicles [Figure 5] and [Figure 6]. The involuting follicles displayed waning of the inflammatory reaction. The primordial follicles were devoid of any inflammation [Figure 7]. The intervening stroma was unremarkable. No perivascular inflammation was evident. A diagnosis of AIO was made. | Figure 1: Scanner view. Fragments of large cystic follicles laced by broad trabeculae of heavy inflammatory infiltrate (H and E, ×40)
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 | Figure 2: Maturing cystic follicles with intense folliculotropic inflammatory infiltrate (H and E, ×100)
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 | Figure 3: Antral follicle with early mild lymphocytic inflammation (arrows) confined to theca interna layer (H and E, ×200)
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 | Figure 4: Theca interna with dense lymphoplasmacytic inflammatory infiltrate. The granulosa layer is spared (H and E, ×200)
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 | Figure 5: Prominent folliculotropic lymphoplasmacytic inflammation with focal infiltration into the granulosa layer (H and E, ×200)
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 | Figure 6: Heavy inflammation with infiltration and destruction of luteinised granulosa cells (H and E, ×400)
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 | Figure 7: Primordial follicle devoid of inflammatory response, surrounded by unremarkable stroma (H and E, ×200)
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Autoimmune oophoritis typically presents with oligomenorrhea or amenorrhea or symptoms relating to multiple follicular cysts including pelvic pain, adnexal torsion or estrogenic manifestations like abnormal bleeding. [5],[6] The patients' ages range between 17 and 48 years with a mean of 31 years. [7] Circulating anti-steroid or anti-ovarian antibodies may be detected in the patient's serum. [5],[6]
Autoimmune oophoritis often occurs in a setting of autoimmune polyendocrine syndromes and is commonly associated with Addison's disease and Hashimoto's thyroiditis. [2],[5] Other autoimmune diseases, which occur less commonly in these patients include rheumatoid arthritis, hypoparathyroidism, myasthenia gravis, diabetes mellitus, atrophic gastritis, pernicious anemia, ITP etc. [7]
The affected ovaries may be small or of normal size but in 1/3 rd of cases the ovaries are cystically enlarged. Cysts are more likely in the earlier phases of the disease and probably due to increased gonadotrophin levels. [5],[6] The generally low estradiol levels as a result of theca cell destruction will lead to impaired feedback with increased FSH levels, which overstimulates the viable granulosa cells. [2],[3]
The cardinal microscopic feature is the folliculotropic lymphoplasmacytic inflammatory infiltrate which selectively attacks the developing follicles and corpus luteum with sparing of the primordial follicles. [2],[4],[5] Immunohistochemical studies reveal a mix of B- and T-cells and polyclonal plasma cells. [1],[4] The more advanced the follicles, denser the inflammation with a greater preponderance of plasma cells. The theca cells are selectively destroyed with sparing or focal involvement of granulosa cells. [4] Extra-follicular infiltrates with medullary, and perivascular distribution may be seen. [4],[5] In some instances eosinophils, histiocytes and rarely sarcoid-like granulomas are present and may predominate. [5],[8] Nonspecific findings like abnormal dysplastic follicles and the superficial cortical fibrosis may be present. [5]
The differential diagnoses include acute and chronic salpingo-oophoritis and lymphoma; these conditions are featured by a more diffuse distribution of the lymphoid cells unlike the perifollicular predilection seen in AIO. [9]
Some patients with AIO may have intermittent ovarian follicle function and may ovulate spontaneously. Until date, there is no specific therapy. Immunosuppression with steroids has been tried but not with consistent results. The prognosis is difficult to evaluate given the rarity of this condition, but some patients may resume normal menses after therapy. [7],[9]
To conclude, although the condition is rare, AIO must be recognized histologically as it leads to ovarian failure and also indicates that the patient is at risk of developing other autoimmune diseases.
| References | |  |
| 1. | Sedmak DD, Hart WR, Tubbs RR. Autoimmune oophoritis: A histopathologic study of involved ovaries with immunologic characterization of the mononuclear cell infiltrate. Int J Gynecol Pathol 1987;6:73-81.  |
| 2. | Varras M, Anastasiadis A, Panelos J, Balassi E, Demou A, Akrivis CH. Autoimmune oophoritis: Clinical presentation of an unusual clinical entity. OA Case Rep 2013;2:7. |
| 3. | De Leon EM, Riddle N, Valente P. Eosinophilic perifolliculitis presenting as an incidental finding in a patient with leiomyomata with perinodular hydropic degeneration. Austin J Clin Pathol 2014;1:2. |
| 4. | Suh YL. Autoimmune oophoritis - a case report. J Korean Med Sci 1992;7:284-90. |
| 5. | Bannatyne P, Russell P, Shearman RP. Autoimmune oophoritis: A clinicopathologic assessment of 12 cases. Int J Gynecol Pathol 1990;9:191-207. |
| 6. | Lonsdale RN, Roberts PF, Trowell JE. Autoimmune oophoritis associated with polycystic ovaries. Histopathology 1991;19:77-81. |
| 7. | Irving JA, Clement PB. Nonneoplastic lesions of the ovary. In: Kurman RJ, Ellenson LH, Ronnett BM, editors. Blaustein's Pathology of the Female Genital Tract. 6 th ed. New York. London: Springer; 2011. p. 579-624. |
| 8. | Lewis J. Eosinophilic perifolliculitis: A variant of autoimmune oophoritis? Int J Gynecol Pathol 1993;12:360-4. |
| 9. | Amarosa EJ, Nascimento A, Hornstein MD, Crum CP. Benign conditions of the ovary. In: Crum CP, Nucci MR, Lee KR, editors. Diagnostic Gynecologic and Obstetric Pathology. 2 nd ed. Philadelphia: Elsevier Saunders; 2011. p. 679-716. |
Correspondence Address:
Dr. Sunitha Jacob
Department of Pathology, Bahrain Specialist Hospital, Juffair, Manama
Bahrain
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0377-4929.155335
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7] |
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