The effect of intravenous immunoglobulin (IVIG) treatment on patients with dermatomyositis: a 4-year follow-up study.

OBJECTIVES: The aim of this study is to evaluate the short- and long-term outcome of patients with dermatomyositis treated with IVIG. METHODS: Forty-two dermatomyositis patients (43 ± 19 yrs, 40.5% males) were studied; 24 of them received IVIG as an add-on treatment, while the rest received conventional immunosupression. The first follow-up point was 6 months following the initiation of treatment. Muscular and cutaneous involvement, as well as demographical and baseline data of the IVIG treated patients, were documented for a median period of 76 months (1st, 3rd quartiles 48, 108). RESULTS: Muscular remission rate was higher for IVIG treated patients at 6 months after the onset of treatment (p=0.007). During long-term follow-up, IVIG treated patients presented with low muscular and cutaneous involvement, as well as low percentages of muscular relapses. The total number of muscular relapses was inversely associated with the number of pulses (p=0.03). CONCLUSIONS: This study is a retrospective one, consisting of a small patient sample, and both muscle and skin involvement scores were developed on the basis of the clinical data provided in the patients' records. Nevertheless, it manages to demonstrate that IVIG may improve the short-term prognosis of dermatomyositis patients as compared to the classical therapies. During long-term follow-up, IVIG treated patients experienced relapses, but their muscular and cutaneous involvement scores were significantly better than their pre-treatment ones. A larger number of IVIG infusions could maintain disease remission for a longer period of time, reducing the total number of muscular relapses.

Peripheral neuropathies in Sjögren's syndrome: a critical update on clinical features and pathogenetic mechanisms.

Sjögren's syndrome is a systemic autoimmune disease that, apart from exocrine glands, may affect every organ or system. Involvement of different sections of the peripheral nervous system results in a wide spectrum of neuropathic manifestations. Based on distinct clinical, electrophysiological and histological criteria, the types of neuropathies seen in Sjögren's syndrome include: a) pure sensory which presents with distal symmetric sensory loss due to axonal degeneration of sensory fibers; sensory ataxia due to loss of proprioceptive large fibers (ganglionopathy); or with painful dysethesias (small fiber sensory neuropathy) due to degeneration of cutaneous axons. The latter appears to be the most common neuropathy in Sjögren's syndrome and requires skin biopsy for diagnosis to document loss or reduction of nerve fiber density; b) sensorimotor polyneuropathy affecting sensory and motor axons, often associated with severe systemic or pro-lymhomatous manifestations, such as palpable purpura and cryoglobulinemia, and c) rare types that include autoimmune demyelinating neuropathy, mononeuropathy, mononeuropathy multiplex and autonomic neuropathy. In this review, the frequency, prevalence and diagnostic criteria for each neuropathy subset are discussed and possible pathogenetic mechanisms are outlined.