ABSTRACT
Behçet's disease (BD) is a multisystemic disorder that involves vessels of all sizes. Superior vena cava (SVC) thrombosis is a rare complication that can lead to the development of various collateral pathways. A 31-year-old man presented with SVC syndrome. He had a history of recurrent genital aphthosis. Computed tomography revealed extensive thrombosis of the right internal jugular, axillary, and subclavian veins with collateral circulation. The patient was diagnosed with BD, and he was started on anticoagulation and immunosuppressive therapy. One week later, he presented with haematemesis. Upper gastrointestinal endoscopy disclosed varices in the upper third of the oesophagus with stigmata of recent bleeding. Portal hypertension was ruled out. Anticoagulation therapy was discontinued. He was discharged on immunosuppressive therapy. Bleeding from downhill oesophageal varices should be suspected in any patient presenting with upper gastrointestinal bleeding and a history of SVC syndrome due to BD.
Subject(s)
Behcet Syndrome/complications , Behcet Syndrome/drug therapy , Esophageal and Gastric Varices/etiology , Hematemesis/etiology , Superior Vena Cava Syndrome/complications , Adult , Anticoagulants/therapeutic use , Behcet Syndrome/diagnosis , Hematemesis/physiopathology , Hematemesis/therapy , Humans , Immunosuppressive Agents/therapeutic use , Male , Rare Diseases , Risk Assessment , Severity of Illness Index , Superior Vena Cava Syndrome/diagnosis , Treatment OutcomeSubject(s)
Chylous Ascites/etiology , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/diagnosis , Adrenal Cortex Hormones/therapeutic use , Antirheumatic Agents/therapeutic use , Chylous Ascites/diagnosis , Chylous Ascites/drug therapy , Diagnosis, Differential , Diuretics/therapeutic use , Drug Therapy, Combination , Female , Humans , Lupus Erythematosus, Systemic/drug therapy , Middle Aged , Sjogren's Syndrome/etiology , Treatment OutcomeABSTRACT
Exceptionally, acute pancreatitis and reactive hemophagocytic syndrome (RHS) are observed in the course of systemic lupus erythematosus (SLE). However, the association of the two conditions has never been reported before. A 31-years-old woman with a 7-year history of SLE was admitted for abdominal pain and fever. Elevated serum amylase and pancreatic enlargement on computerized tomography confirmed the diagnosis of pancreatitis. Laboratory examinations revealed pancytopenia, abnormal hepatic tests, and elevation of serum LDH and triglyceride levels. Bone marrow aspiration showed hemophagocytosis. The patient responded well to high dose corticosteroids. About eighty cases of pancreatitis have been reported in patients with SLE. The mechanisms are still unclear: SLE as the primary etiologic factor, drug toxicity, especially steroids which play a controversial role, or infection. About 40 cases of RHS have been reported in patients with SLE, sometimes associated with active infection. Overall mortality is 38.5%. When RHS occurs as an initial manifestation of SLE, or in the course of active SLE, it responds well to immunosuppressive therapy.