Choroidal vascular occlusion in a child with a connective tissue disease and complement C4 deficiency.
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.
Ghazi
Nicola G
NG
The Eye Pathology Laboratory, Wilmer Institute, The Johns Hopkins Medical Institutions, 600 North Wolfe Street, Baltimore, MD 21287-9248 USA.
Gollance
Stephen A
SA
Green
W Richard
WR
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
D003181:Complement C4