Sarcoidosis is a heterogenous, multisystem disease that presents with noncaseating granulomas. It commonly affects the lymph nodes and pulmonary system, with 50% of cases having concurrent extrapulmonary involvement. Involvement of the reproductive tract has only been rarely reported in the literature [1]. We present a case of sarcoidosis involving the uterus and adnexal structures in a woman aged in her 60s, presenting with a 4-week history of progressive left visual changes.
A case presentation featuring the clinical progress, relevant investigations, and literature review.
Left-sided reduced visual acuity due to optic neuritis was identified on optometry, magnetic resonance imaging (MRI) brain, and (absent) visual evoked potentials. MRI spine was normal. Cerebrospinal fluid was negative for anti-aquaporin-4, anti-myelin oligodendrocyte glycoprotein, and anti-neuronal antibodies. Oligoclonal bands were matched. Widespread lymphadenopathy on MRI and computerized tomography imaging was fluorodeoxyglucose (FDG)-avid on positron emission tomography scan. Supraclavicular lymph node biopsies revealed necrotising noncaseating granulomatous inflammation. FDG-avidity in the endometrium and ovaries was histologically assessed and demonstrated granulomatous vasculitis. Grade 1 endometrioid cancer was also detected, and the patient had a subsequent hysterectomy and bilateral salpingo-oophorectomy. Microbiologic testing, including for M. tuberculosis and fungi, was negative. Other key results include a negative anti-neutrophil cytoplasmic antibody and raised C-reactive protein 13 mg/L, erythrocyte sedimentation rate 36 mm/hr, and angiotensin-converting enzyme 161 nM/mL/m. Initial treatment comprised corticosteroids and mycophenolate. Adalimumab was commenced due to mycophenolate toxicity and has proven to be effective in inducing and maintaining remission of disease.
This case highlights the rare involvement of the reproductive tract in sarcoidosis following exclusion of other immune-mediated and microbiologic causes of granulomatous disease.
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