Nerve granulomas and vasculitis in sarcoid peripheral neuropathy: A clinicopathological study of 11 patients

• Published in: Brain (London, England : 1878) Vol. 125; no. Pt 2; pp. 264 - 275
• Main Authors: SAID, Gérard, LACROIX, Catherine, PLANTE-BORDENEUVE, Violaine, LE PAGE, Laurence, PICO, Fernando, PRESLES, Olivier, SENANT, Jacques, REMY, Pascal, RONDEPIERRE, Philippe, MALLECOURT, Jacques
• Format: Journal Article
• Language: English
• Published: Oxford Oxford University Press 01.02.2002
• Subjects: Adult; Aged; Aged, 80 and over; Biological and medical sciences; Biopsy; Capillaries - ultrastructure; Electrophysiology; Female; Granuloma - pathology; Guillain-Barre Syndrome; Humans; Male; Medical sciences; Middle Aged; Muscle, Skeletal - pathology; Muscle, Skeletal - physiopathology; Muscle, Skeletal - ultrastructure; Neural Conduction; Peripheral Nerves - pathology; Peripheral Nerves - physiopathology; Peripheral Nerves - ultrastructure; Peripheral Nervous System Diseases - etiology; Peripheral Nervous System Diseases - pathology; Peripheral Nervous System Diseases - physiopathology; Retrospective Studies; Sarcoidosis - pathology; Sarcoidosis - physiopathology; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Vasculitis - pathology; Human; Granuloma; Nervous system diseases; Necrotizing vasculitis; Sarcoidosis; Cardiovascular disease; Peripheral neuropathy; Vascular disease; Electrodiagnosis; Pathology; Nerve conduction; Systemic disease; Adult; Complication; Motor nerve; Peripheral nerve disease; Diagnosis
• ISSN: 0006-8950
• DOI: 10.1093/brain/awf027

Peripheral neuropathy is a rare, yet treatable manifestation of sarcoidosis, a multisystem disorder characterized by the presence of non-caseating granulomas that are seldom found in nerve biopsy specimens. In order to learn more about the subject, we reviewed our clinical and pathological findings in a series of 11 patients (six men and five women aged 26-83 years) with symptomatic neuropathy associated with characteristic granulomas in nerve biopsy specimens. Only two patients were known to have sarcoidosis before the occurrence of the neuropathy. The neuropathy was focal or multifocal in six patients, including one with a multifocal neuropathy associated with conduction blocks, and one with a multifocal axonal motor deficit. Four patients had a distal symmetrical deficit and one patient had a Guillain-Barré-like syndrome with facial diplegia and respiratory failure. Serum angiotensin-converting enzyme concentration was elevated in only two patients. Epineurial granulomas and perineuritis were present in all nerve specimens. The inflammatory infiltrates invaded the endoneurium, following connective tissue septae and blood vessels, in five patients. Multinucleated giant cells were found in eight patients and necrotizing vasculitis in seven. Inflammatory lesions were associated with variable, asymmetrical involvement of nerve fascicles and axon loss. A muscle specimen was sampled during the same procedure in 10 patients. It showed inflammatory infiltrates and granulomas in nine patients and necrotizing vasculitis in two. Immunolabelling showed a mixed inflammatory infiltrate of T cells (predominantly CD4+ cells) and macrophages, in keeping with a delayed hypersensitivity reaction. In addition to nerve involvement, all patients had at least one other tissue or organ affected, including muscle in nine patients, lungs and/or intrathoracic lymph nodes in eight, skin in three, arthritis in two, and peripheral lymph nodes, stomach and eye in one patient each. Most patients improved on corticosteroids. Two patients remain free of symptoms after 7 years. Severe side-effects of long-term treatment with corticosteroids occurred in two patients, leading to death in one. This study illustrates the wide range of clinical manifestations of sarcoid neuropathy and the frequent association of granulomatous inflammatory infiltrates with necrotizing vasculitis and with silent or symptomatic involvement of other organs.