| Popis: |
Sarcoidosis and the overlap syndrome of autoimmune hepatitis and primary biliary cholangitis (PBC) share common clinical, biological, and histological features. The simultaneous occurrence of these diseases have been reported in few cases and suggests that a common pathway which may contribute to granuloma formation in both conditions.We report the cases of two female patients having an association of sarcoidosis and inflammatory liver diseases.The first case is of a 61-year-old woman had been monitored for an overlap syndrome of PBC and autoimmune hepatitis (AIH). Therefore, treatment with azathiprine has been initiated associated with ursodeoxycholic acid (UDCA). Azathioprine had to be discontinued due to digestive intolerance, specifically chronic diarrhea and abdominal pain. The patient remained clinically stable on UDCA and her liver function tests were stable for years, until she developed symptoms of progressive dyspnea without any other associated signs. Chest computed tomography (CT) revealed mediastinal enlargement, bilateral pulmonary nodules, and symmetrical adenomegalies in the mediastinum. The bronchoalveolar lavage (BAL) revealed increased cellularity, with a notable elevation in lymphocyte count (48 %) and a CD4/CD8 ratio of 4. The patient underwent mediastinoscopy; a biopsy of the right laterotracheal (4R) adenomegaly was performed. Histological examination of the lymph node showed epithelioid and giant-cell tuberculoid lymphadenitis without necrosis, compatible with sarcoidosis. Ophthalmological and cardiac assessments were normal. Plethysmography test was normal and there was no need for corticosteroid treatment; a surveillance was planned. Treatment with UDCA was pursued. The second case is of a 50-year-old woman with no medical history presented symptoms including dry eyes and mouth, inflammatory-type polyarthralgia affecting knees and wrists, bilateral Raynaud's phenomenon, right hypochondrium pain, and worsening dyspnea over six months. Liver analysis showed elevation of alkaline phosphatase (ALP) to three times upper limit of normal (ULN) and gamma-glutamyltransferase (GGT) to 5 times ULN. This cholestasis was associated with an increase in transaminase activity to 5 times ULN for over six months. Immunological tests revealed positive anti-nuclear antibodies (ANA), anti-Ro52, anti-M23E, and anti-centromere antibodies. Chest-CT showed multiple bilateral bronchiolar parenchymal micronodules mostly in the upper and posterior regions without any mediastinal adenomegaly. Bronchial endoscopy was normal, and biopsies indicated chronic inflammation. The BAL revealed increased cellularity, characterized by a high lymphocyte count (51.7 %) and a CD4/CD8 ratio of 2.8. Biopsy of minor salivary gland revealed grade 4 lymphocytic sialadenitis. Skin biopsy revealed an epithelioid granuloma without caseous necrosis. Liver biopsy performed in the presence of cytolysis and moderate hepatic insufficiency, revealed granulomatous hepatitis and cholangitis lesions along with septal fibrosis suggestive of PBC. The diagnosis of cutaneous and pulmonary sarcoidosis with PBC and Sjögren's syndrome was retained. The spirometry and diffusing capacity for carbon monoxide value were normal. Treatment involved UDCA, corticosteroids, and azathioprine, leading to clinical and biological improvement.Sarcoidosis shares some clinical manifestations with autoimmune liver diseases, primarily PBC. A hepatic granuloma with a different appearance and location can guide the diagnosis. Early diagnosis and appropriate management can avoid serious complications and improve prognosis. |
