Neurological Manifestations in Sjögren Syndrome
Abstract & Commentary
By John J. Caronna, MD, Vice-Chairman, Department of Neurology, Cornell University Medical Center, Professor of Clinical Neurology, NewYork-Presbyterian Hospital. Dr. Caronna reports no financial relationship relevant to this field of study.
Synopsis: Approximately one half of patients who meet current criteria for the diagnosis of primary Sjögren syndrome have clinical or laboratory evidence for peripheral neuropathy.
Source: Gøransson, LG, et al. Peripheral Neuropathy in Primary Sjögren Syndrome. A Population-Based Study. Arch Neurol. 2006;63:1612-1615.
Primary Sjögren Syndrome (PSS), an auto-immune disease that affects the exocrine glands, is characterized clinically by keratoconjunctivitis sicca and xerostomia. The histologic hallmark of PSS is focal infiltration of the salivary glands by mononuclear lymphoid cells. More than half of patients develop extraglandular manifestations such as myalgias, arthralgias, and pulmonary and gastrointestinal involvement. Gøransson and associates investigated the prevalence and pattern of peripheral nerve involvement in PSS patients selected using new, more stringent criteria.1
The authors reviewed the medical records of all patients diagnosed with PSS between 1980 and 2004 at a university hospital in Norway. Of 67 patients who fulfilled the revised international classification criteria for PSS, 62 (8 men and 54 women) agreed to participate in the study (Table 1). Their mean + SD duration of disease was 12 + 10 years (range 0-48 years). Twenty-eight of 62 were not receiving medication for PSS. Among those receiving medication for PSS, 24 were taking antimalarial drugs, 14 were taking corticosteroids, and 6 were taking immunosuppressant drugs.
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Seventeen patients (27%) had signs of peripheral neuropathy (PN) on clinical examination. The results of nerve conduction studies (NCS) were abnormal in 34 patients (55%): 19 patients (31%) had motor neuropathy, 8 (13%) had sensory neuropathy, and 7 (11%) had sensorimotor neuropathy. In 15 patients the only abnormal finding was an increased F-wave latency in 2 or more nerves. Ten patients had unilateral (n = 8) or bilateral (n = 2) carpal tunnel syndrome, and 3 had abnormal NCS due to local nerve injuries unrelated to PSS.
No significant differences were found on skin biopsy in the density of intraepidermal nerve fibers (IENF) between the calf and the thigh. Mean IENF densities in the leg were significantly lower in PSS patients compared with normative values2 and values in patients with systemic lupus erythematosus.3 Two PSS patients had IENF densities less than 3.4 fibers/mm., thereby meeting the morphologic criteria for small-diameter nerve fiber neuropathy. NCS results were normal in these 2 patients. No associations between abnormal clinical findings and IENF densities in the leg or thigh were detected.
Commentary
The authors investigated peripheral nervous system involvement in patients with PSS. They applied the new classification criteria1 to ensure that only patients with true autoimmune PSS were included and patients with SICCA syndromes due to other causes were excluded. Using clinical, electrophysiologic, and morphometric examinations, they identified PN in about one-half of the PSS patients studied (34/62). In half of these (17/34), the PN was subclinical.
Several mechanisms could underlie the demyelinating neuropathy in PSS. Previous authors have reported vascular and perivascular inflammatory infiltrates involving the vasa nervorum in peripheral nerve biopsy specimens.4,5 The present study has given new impetus to the search for antibodies with reactivity against the proximal regions of sensory and motor nerves and neurons.
References
1. Vitali C. et al. Ann Rheum Dis. 2002;61:554-558.
2. Gøransson LG, et al. Neurology. 2004;62:774-777.
3 Gøransson LG, t al. Arch Neurol. 2006;63:1410-1413.
4 Mellgen, SI, et al. Neurology. 1989;39:390-394.
5. Griffin, JW, et al. Ann Neurol. 1990;27:304-315.
Approximately one half of patients who meet current criteria for the diagnosis of primary Sjögren syndrome have clinical or laboratory evidence for peripheral neuropathy.You have reached your article limit for the month. Subscribe now to access this article plus other member-only content.
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