Diagnostic and prognostic relevance of neuromuscular biopsy in primary Sjögren's syndrome-related neuropathy.

Abstract

OBJECTIVE:

To evaluate the clinicobiologic presentation in patients with primary Sjögren's syndrome (SS)-related peripheral neuropathy, the histologic results of neuromuscular biopsy (NMB), and clinical outcome, and to identify prognostic factors.

METHODS:

We retrospectively studied clinical and biologic presentation of 40 patients with primary SS-related neuropathy who underwent NMB. Prognostic factors of clinical outcome were assessed by univariate and multivariate analysis.

RESULTS:

Patients with vasculitis (lymphocytic [n = 8] or necrotizing [n = 14]) had a higher prevalence of acute-onset neuropathy, multiple mononeuropathy, sensorimotor involvement, vascular purpura, general symptoms, increased C-reactive protein level, positivity for rheumatoid factor, hypocomplementemia, and monoclonal gammopathy compared with those without vasculitis (n = 18). Comparison between patients with necrotizing or lymphocytic vasculitis did not reveal significant differences in clinical or biologic presentation except for the presence of general symptoms and rheumatoid factor. Regarding clinical evolution, the results of NMB (P < 0.0001), in particular the presence of necrotizing vasculitis (P < 0.001), an acute neuropathy onset (P < 0.0001), general symptoms (P < 0.0001), multiple mononeuropathy (P = 0.0007), presence of sensorimotor involvement (P = 0.002), and increased C-reactive protein level (P = 0.008), were significantly associated with a better outcome in univariate analysis. In multivariate analysis, NMB resulting in the identification of patients with necrotizing vasculitis was the only variable that remained significantly associated with a better outcome (P = 0.01).

CONCLUSION:

NMB is necessary to identify patients with necrotizing vasculitis, who have a better response to immunosuppressive therapy. NMB might therefore have both a diagnostic and prognostic relevance in primary SS-related neuropathy.

• Authors: Benjamin Terrier, Catherine Lacroix, Loïc Guillevin, Pierre-Yves Hatron, Robin Dhote, François Maillot, Elisabeth Diot, Françoise Sarrot-Reynauld, Christelle Sordet, Odile Dubourg, Laurence Meyer, Xavier Mariette, Jacques-Eric Gottenberg, Club Rhumatismes et Inflammation
• Journal: Arthritis and rheumatism (Arthritis Rheum) Vol. 57 Issue 8 Pg. 1520-9 (Dec 15 2007) ISSN: 0004-3591 [Print] United States
• PMID: 18050172 (Publication Type: Journal Article, Multicenter Study)

Chemical References

• Adrenal Cortex Hormones
• Immunosuppressive Agents
• C-Reactive Protein

Topics

• Adrenal Cortex Hormones (therapeutic use)
• Adult
• Aged
• Biopsy
• C-Reactive Protein (metabolism)
• Disease Progression
• Female
• Humans
• Immunosuppressive Agents (therapeutic use)
• Male
• Middle Aged
• Multivariate Analysis
• Muscle, Skeletal (pathology)
• Peripheral Nervous System Diseases (diagnosis, etiology, pathology)
• Prognosis
• Retrospective Studies
• Sjogren's Syndrome (blood, complications, drug therapy)
• Sural Nerve (pathology)
• Vasculitis (diagnosis, etiology, pathology)