An Atypical Presentation of Extrapulmonary Sarcoidosis

Abstract

Sarcoidosis is an idiopathic, chronic, multisystem, granulomatous, inflammatory disease involving almost all organs. Sarcoidosis can occur with an atypical presentation of hepatosplenic involvement, like in the case of our patient. In this case report, we present a rare case of extrapulmonary sarcoidosis with isolated involvement of the liver and spleen in a 39-year-old Caucasian female. There is a possibility of this isolated involvement of an organ in the complete absence of pulmonary disease, which makes the diagnosis of sarcoidosis very difficult as it is usually not suspected. Ultrasound and CT are important in ruling out other differential diagnoses, but a definitive diagnosis is possible only on histological examination, differentiating sarcoid lesions from tuberculosis, primary biliary cirrhosis, metastasis, malignancy, and other granulomatous infections or diseases. Hence, the most credible criterion for diagnosis remains histology. After diagnosis, regular follow-up for systemic manifestations is recommended. Asymptomatic patients with hepatosplenic sarcoidosis have a good prognosis without any medical intervention, while patients with abnormal labs or symptoms must commence treatment.

The clinical management of hepatic sarcoidosis: A systematic review

“…The indication for therapy has been based on the presence of symptoms and biochemical liver derangement, with or without evidence of fibrosis/ cirrhosis on imaging. 9 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 The dosage of corticosteroids, particularly prednisolone, varies from 20 mg to 60 mg per day. 12 , 27 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 In our review, some of the studies used dosages of 0.5 mg to 1 mg/kg/day, 45 , 46 , 47 and none of the studies reported a prednisolone dose of greater than 1 mg/kg/day.…”

“…Despite this low quality evidence data, it can be summarized that corticosteroids are the main first‐line treatment in hepatic sarcoidosis. The indication for therapy has been based on the presence of symptoms and biochemical liver derangement, with or without evidence of fibrosis/ cirrhosis on imaging 9,27–36 . The dosage of corticosteroids, particularly prednisolone, varies from 20 mg to 60 mg per day 12,27,37–44 .…”

Sarcoidosis Presenting as Granulomatous Mastitis, Erythema Nodosum, and Arthritis Syndrome: A Case Report and Comprehensive Review of the Literature

“…Granulomatous mastitis treatment is widely variable depending on multiple factors and includes corticosteroids, surgery, methotrexate, and other immunosuppressive regimens. 16 A high success rate of remission has been demonstrated with the use of high-dose steroids. However, the undesirable side effects have limited its use.…”

“…However, the undesirable side effects have limited its use. 16 Conversely, studies have reported plausible outcomes and a lower recurrence rate of GM with the use of methotrexate as a single-drug regimen or in addition to low-dose corticosteroids.…”

Identification of Isolated Hepatic Sarcoidosis With 18F-FDG PET/CT and MRI

“…Hepatic involvement of systemic sarcoidosis is relatively common, and although it is usually asymptomatic, it can rarely lead to complications including cirrhosis and portal hypertension 1–5 . Isolated hepatic involvement of sarcoidosis is very rare 6–8 …”