Nervous system dysfunction in Henoch–Schönlein syndrome: systematic review of the literature

• Published in: Rheumatology (Oxford, England) Vol. 48; no. 12; pp. 1524 - 1529
• Main Authors: Garzoni, Luca, Vanoni, Federica, Rizzi, Mattia, Simonetti, Giacomo D., Simonetti, Barbara Goeggel, Ramelli, Gian P., Bianchetti, Mario G.
• Format: Journal Article
• Language: English
• Published: Oxford Oxford University Press 01.12.2009
• Subjects: Adolescent; Adult; Age Distribution; Biological and medical sciences; Central Nervous System Diseases - diagnosis; Child; Child, Preschool; Dermatology; Diseases of the osteoarticular system; Female; Henoch–Schönlein syndrome; Humans; Hypertension - complications; IgA Vasculitis - diagnosis; Infant; Infant, Newborn; Kidney Diseases - complications; Male; Medical sciences; Nervous System Diseases - diagnosis; Nervous System Diseases - etiology; Peripheral Nervous System Diseases - diagnosis; Peripheral neuropathy; Posterior reversible encephalopathy syndrome; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Stroke; Vascular disorders of the skin; Vasculitis; Young Adult; Skin disease; Nervous system diseases; Stroke; Diseases of the osteoarticular system; Rheumatology; Cardiovascular disease; Nervous system; Henoch-Schönlein syndrome; Review; Peripheral neuropathy; Cerebral disorder; Vascular disease; Vasculitis; Posterior reversible encephalopathy syndrome; Dysfunction; Central nervous system disease; Henoch-Schönlein purpura; Capillary vessel disease; Peripheral nerve disease; Cerebrovascular disease; Bibliographic review
• DOI: 10.1093/rheumatology/kep282
• ISSN: 1462-0324; 1462-0332

Objective. CNS or peripheral nervous system dysfunction sometimes occurs in Henoch–Schönlein patients. Methods. We review all Henoch–Schönlein cases published after 1969 with CNS dysfunction without severe hypertension and neuroimaging studies (n = 35), cranial or peripheral neuropathy (n = 15), both CNS and peripheral nervous system dysfunction without severe hypertension (n = 2) or nervous system dysfunction with severe hypertension (n = 2). Forty-four of the 54 patients were <20 years of age. Results. In patients with CNS dysfunction without or with severe hypertension the following presentations were observed in decreasing order of frequency: altered level of consciousness, convulsions, focal neurological deficits, visual abnormalities and verbal disability. Imaging studies disclosed the following lesions: vascular lesions almost always involving two or more vessels, intracerebral haemorrhage, posterior subcortical oedema, diffuse brain oedema and thrombosis of the superior sagittal sinus. Following lesions were noted in the subjects with cranial or peripheral neuropathy without severe hypertension: peroneal neuropathy, peripheral facial palsy, Guillain–Barré syndrome, brachial plexopathy, posterior tibial nerve neuropathy, femoral neuropathy, ulnar neuropathy and mononeuritis multiplex. Persisting signs of either CNS (n = 9) or peripheral (n = 1) nervous system dysfunction were sometimes reported. Conclusions. In Henoch–Schönlein syndrome, signs of nervous system dysfunction are uncommon but clinically relevant. This review helps clinicians managing Henoch–Schönlein syndrome with nervous system dysfunction.