breast mass has many differential diagnosis, one of which is autoimmune, idiopathic granulomatosis mastitis.

1. Idiopathic Granulomatous
Mastitis (IGM)
Marwa Abo Elmaaty Besar
Lecturer Of Internal Medicine
(Rheumatology Immunology Unit)
(Pediatric Rheumatology)

3. Idiopathic granulomatous mastitis
(IGM)
 IGM is a rare benign chronic inflammatory condition of the breast.
 It is a rare breast pseudotumor.
 IGM characterised by non-caseating granulomas in the breast, often
with abscess formation.
 First described by Kessler and Wolloch in 1972.
 GM is rare, and incidence and prevalence have been difficult to
estimate.
 Incidence:
 2.4 per 100,000 women
 0.37% in the US, Europe is less.

4.  High risk group:
 non-white women.
 women of childbearing age, median age 30 years.
 Pregnant, first five year of pregnancy.
 lactation or hyperprolactinemia previous 5-6 years.
 Female using oral contraceptive pill.

5. The aetiology:-
 The exact etiology of GM is still unknown,
 A possible reason for GM is an inflammatory autoimmune response to epithelial damage,
although the trigger for this damage is still unknown.
 Hormonal changes:
 A correlation with breastfeeding and childbirth
 Might occur in response to extravasated secretions from the lobules.
 Bacterial infection;
 Corynebacterium kroppenstedtii has often been cultivated and may therefore play
a fundamental role in inducing this disease.
 lipophilic gram-positive rod, named ‘cystic neutrophilic granulomatous mastitis’

6. The aetiology:-
 Medication;
 Hyperprolactinemia provoked by antipsychotic medication
 Promote ductal ectasias and milk stagnation lead to inflammation.
 Alpha-1 antitrypsin deficiency
 Evidence of it is a potential risk factor for inducing GM
 Is an autosomal codominant disorder
 Is most prevalent in Caucasians of European or North American
descent.

7. Presenting Symptoms:-
 A very obscure presentation.
 The leading symptom of GM is a painful mass or as a hard irregular mass,
 Up to 50% of patients, erythema and swelling (inflammation of the involved
breast).
 Other symptoms; hyperemia, areolar retraction, fistula, and ulceration.
 Around 37% patients signs of an abscess.
 Lymphadenopathy is present in up to 15% of patients.
 Most lesions occur unilaterally.
 The lesion may occur in any quadrant of the breast but is mostly in the retro
areolar region from where it extends radially.

9. Differential Diagnosis:
 The mass may clinically mimic a bacterial abscess and/or breast cancer by
inducing skin or nipple retraction.
1. Granulomatous conditions
o Tuberculosis,
o Sarcoidosis,
o Wegener’s granulomatosis.
2. Other infection commonly involve breast:-
o histoplasmosis,
o actinomycosis,
3. Foreign body reaction, fat necrosis.
4. IgG4-RD mastitis,
5. Inflammatory breast cancer

10. Diagnosis:
 The diagnosis and treatment of a patient with GM still remains a
challenge
 The unspecific symptoms are often misleading in the diagnostic process.
 The patient suffer a protracted disease course with a significant impact on
quality of life.
 Different causes of mastitis, and most importantly malignancy, have to be
excluded before the diagnosis of GM
 The time period from the onset of symptoms to the exact diagnosis can
therefore be several months up to years.
 Radiographic images (Ultrasound, mammography, and magnetic
resonance imaging (MRI)) are nonspecific

11.  Imaging:-
 Ultrasound;
o Multiple contiguous hypoechoic masses with posterior acoustic shadowing or posterior acoustic enhancement.
o Advanced cases present with fluid collections and cavities in association with skin fistulas.
o hypervascularity which can be detected by Doppler imaging
o 15–55% of all cases show ipsilaterally enlarged reactive axillary lymph nodes
 Mammography:
o Unilateral focal or regional asymmetry as the most frequent pattern,
o 24% normal finding.
o Lesions were mammographically occult in 15 out of 45 women, because of an overlying dense breast pattern seen in
most women.
 MRI findings are also variable
o Heterogeneous ill-defined masses
o Non-mass enhancement, abscess formation, depending on the severity of the inflammation.
Breast Care 2018;13:413–418

12.  Laboratory Findings:-
o No blood-related factors to reliably support IGM.
o RF, ANA, ANCA (C,P), anti-dsDNA antibodies, interleukin 2 receptor, or angiotensin-
converting enzyme; NORMAL.
o C-reactive protein levels; unspecific marker of infection.
o The levels of carcinoembryonic antigen and cancer antigen (CA) within normal limit.

13. Histopathological assessment:
 The gold standard for diagnosing GM is core needle biopsy of the
lesion with a sensitivity of 96%,
o A non-necrotizing granuloma in combination with a localized infiltrate of
multi-nucleated giant cells, epithelioid histiocytes, lymphocytes, and
plasma cells.
o Organized sterile micro-abscesses occur with neutrophilic infiltrates.
o Inflammation that extends into adjacent lobules can indicate a higher
severity.
o The involved parenchyma mostly shows loss of acinar structures and
damaged ducts

14. Management:
 No consensus on disease management exists.
 A blind antibiotic therapy;
o Mastitis,
o without any microbiological proof of a bacterial infection.
o Therapy usually fails.
o The lowest efficacy with improvement rates ranging from 6 to 21%
 Drainage and aspiration
o Abscess puncture, drainage, or incision depending on the size of the
lesion
o Aspiration can fail because the abscess-like mass often has necrotic
tissue in the center that makes the aspirate thick and hard to extract.
Breast Care 2018;13:413–418

15.  Conservative strategy involving medical therapy with corticosteroids versus a surgical
approach.???????
 Medical therapy:
1. Corticosteroid:
o A high-dose corticosteroid therapy with prednisolone 30 mg/day for at least 2
months
o leads to a decrease in the diameter of the lesion.
o The duration of steroid varied between 2 and 6 months.
o a success rate of between 66 und 72%.
o a recurrence rate of 15% was reported.
o Despite these side effects, this approach became standard of care.

16. 1. Methotrexate
 Indication: mainly for patients
o who have failed corticosteroid therapy.
o who have relapsed or
o who do not tolerate high-dose corticosteroid therapy
 low-dose regimen, 7.5–25 mg a weekly dose combined with folic acid applied daily or once a
week
 S/E;
o Ulcerative stomatitis, leukopenia,
o Nausea, abdominal distress,
o Undue fatigue, chills and fever, dizziness,
o Decreased resistance to infection.
 The evidence for this approach is limited and based on only a few case reports,
 Other immunosuprresion; azathioprine

17.  Surgical treatment: wide excision of the lesion
 The decision at what stage of the disease surgery is performed depends on the
individual clinical appraisal of the patient.
 The choice of surgical technique which varies from wide excision to mastectomy.
 Problem;
o Scars, asymmetry,
o Unsatisfactory esthetic results,
o Problems with breastfeeding.

18. Which is good?
 The decision whether surgery or medical treatment is preferred depend on
o Divergent regional resources,
o The patient’s expectations,
o Surveillance opportunities.
 Some authors primarily perform surgery mostly as a wide excision, others
start with a conservative regimen and switch to surgery when the medical
treatment fails.
 Surgery alone or in combination with corticosteroids seems to have the
lowest recurrence rates of 6.8 and 4%, respectively.
 There is a great variety of findings related to the risk of recurrence for the
different therapeutic approaches.

19. The local standards of the breast unit of ‘kliniken essen-mitte

20. Home message
 Idiopathic granulomatous mastitis (IGM) is a rare benign chronic
inflammatory condition of the breast but occur.
 IGM has clinical and imaging features similar to inflammatory breast
carcinoma.
 A definite diagnosis of idiopathic granulomatous mastitis always
requires a histopathological analysis .
 The treatment of choice for IGM has not yet been established,
corticosteroid or surgical should be individualized, expert opinion.

21. Reference:-
 Hovanessian Larsen LJ, Peyvandi B, Klipfel N, et al: Granulomatous lobular mastitis: imaging,
diagnosis and treatment. AJR Am J Roentgenol 2009; 193: 574–581.
 Akbulut S, Arikanoglu Z, Senol A, et al: Is methotrexate an acceptable treatment in the
management of idiopathic granulomatous mastitis? Arch Gynecol Obstet 2011; 284: 1189–1195.
 Bashir MU, Ramcharan A, Alothman SB, et al: The enigma of granulomatous mastitis: a series.
Breast Dis 2017; 37: 17–20.
 Calis H, Karabeyoglu SM: Follow-up of granulomatous mastitis with monitoring versus surgery.
Breast Dis 2017; 37: 69–72.
 Emre A, Akbulut S, Sertkaya M, et al: Idiopathic granulomatous mastitis: overcoming this important
clinical challenge. Int Surg 2017;Epub ahead of print.
 Huyser M, Kieran J, Myers S, et al: Review of idiopathic granulomatous mastitis in the Southwest
Native American population. Presentation at The American Society of Breast Surgeons, 19th Annual
Meeting 2018, Orlando.
 Moris D, Damaskos C, Davakis S, et al: Is idiopathic granulomatous mastitis a surgical disease? The
jury is still out ATM. Ann Transl Med 2017; 5: 309.
 Wong SCY, Poon RWS, Chen JHK, et al: Corynebacterium kroppenstedtii is an emerging cause of
mastitis especially in patients with psychiatric illness on antipsychotic medication. Open Forum
Infect Dis 2017; 4:ofx096.