Coeliac disease associated with sarcoidosis and antiphospholipid syndrome: A case report

• Main Authors: Melek Kechida, Noel Lorenzo Villalba
• Format: Article
• Language: English
• Published: Elsevier 2017-07-01
• Series: Egyptian Rheumatologist
• Subjects: Sarcoidosis; Antiphospholipid syndrome; Celiac disease; Auto immunity; Pancreatitis
• Online Access: http://www.sciencedirect.com/science/article/pii/S1110116417300261

Background: Sarcoidosis is a clinically heterogenous disease of unknown etiology with a hallmark of the development and accumulation of non-caseating granulomas in any organ. The antiphospholipid syndrome (APS) is an autoimmune disorder characterized by an elevated risk for arterial and venous thrombosis and pregnancy-related morbidity. Celiac disease (CD) is a chronic, immune-mediated form of enteropathy that is now presenting later in life and often with extraintestinal manifestations. Co-existence of sarcoidosis, CD and APS is extremely rare. Case presentation: We describe a 27-year-old Tunisian woman with a history of non-explored superficial vein thrombosis and 4 successive miscarriages, who was explored for abdominal pain, vomiting, hypercalcemia, hepatic cytolyses and cholestasis and was complicated later with pancreatitis and anterior uveitis. Hepatic biopsy revealed noncaseating-granulomas, a high serum angiotensin converting enzyme activity was detected and the diagnosis of sarcoidosis was considered. A high titer of antiphopsholipid antibodies concluded the diagnosis of APS. Duodenal biopsies showed a total villous atrophy indicative of CD, confirmed by positivity of serum anti-endomisium and anti-transglutaminase antibodies. She had normocytic anemia (hemoglobin 9 g/dl) and elevated transaminases. Thorough investigation established the diagnosis of extra pulmonary sarcoidosis associated with CD and APS. Conclusion: Co-existence of sarcoidosis, CD and APS is extremely rare. APS should be recognized as an accompanying disorder of sarcoidosis and antiphospholipids measured especially when there is a history of thrombosis or miscarriages. CD should not be overlooked in association to sarcoidosis, given the shared immunological and genetic background, even in the absence of a typical presentation of the disease.