title: Choroidal vascular occlusion in a child with a connective tissue disease and complement C4 deficiency.
abstract: OBJECTIVE
  To report the histopathologic findings in the eyes of a patient with a connective tissue disease and complement deficiency.
  
  
  METHODS
  Human postmortem ocular histopathologic study.
  
  
  METHODS
  A 15-year-old female died from complications of a connective tissue disease of uncertain etiology, particularly acute respiratory distress syndrome. Abnormalities seen in the eyes at autopsy were consistent with complement activation, granulocyte aggregation, and leukocyte embolization.
  
  
  METHODS
  Both eyes were examined by light microscopy.
  
  
  RESULTS
  Some choroidal vessels were occluded by platelet-fibrin thrombi and occasionally by aggregates of granulocytes and fibrin. Serous retinal detachment involving the macula and peripheral retina was present in both eyes.
  
  
  CONCLUSIONS
  This is a report of the ocular histopathologic findings in a patient with connective tissue disease and complement C4 deficiency. The light microscopy findings were consistent with complement activation with granulocyte aggregation and leukocyte embolization and may represent another mechanism to explain the clinical findings in patients with connective tissue disease, particularly systemic lupus erythematosus.
authors.affiliation: The Eye Pathology Laboratory, Wilmer Institute, The Johns Hopkins Medical Institutions, 600 North Wolfe Street, Baltimore, MD 21287-9248 USA.
authors.forename: Nicola G
authors.initials: NG
authors.lastname: Ghazi
authors.forename: Stephen A
authors.initials: SA
authors.lastname: Gollance
authors.forename: W Richard
authors.initials: WR
authors.lastname: Green
mesh_terms: D000293:Adolescent; D002829:Choroid / Q000098:blood supply* / Q000473:pathology; D003167:Complement Activation; D003181:Complement C4 / Q000172:deficiency*; D003240:Connective Tissue Diseases / Q000150:complications* / Q000175:diagnosis; D003251:Constriction, Pathologic; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D016491:Peripheral Vascular Diseases / Q000209:etiology* / Q000473:pathology; D012128:Respiratory Distress Syndrome / Q000150:complications; D012163:Retinal Detachment / Q000209:etiology / Q000473:pathology
chemical_list: D003181:Complement C4