Chronic inflammatory demyelinating polyradiculoneuropathy associated with Sjögren`s syndrome in a child.

BACKGROUND: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a peripheral nervous system disease associated with polyautoimmunity. CASE: We report a previously healthy 13-year old boy who was referred to our outpatient clinic with gait disturbance and distal lower limb weakness that had been increasing for six months. The patient had decreased deep tendon reflexes in the upper extremities and absence in the lower extremities, reduced muscle strength in the distal and proximal lower extremities, muscle atrophy, drop foot, and normal pinprick sensations. The patient was diagnosed with CIDP as a result of clinical findings and electrophysiological studies. Autoimmune diseases and infectious agents were investigated in terms of triggering CIDP. Although there was no clinical sign other than polyneuropathy, he was also diagnosed with Sjögren`s syndrome due to positive antinuclear antibodies and antibodies against Ro52, and with autoimmune sialadenitis. After six months of monthly intravenous immunoglobulin and oral methylprednisolone treatments, the patient was able to dorsiflex his left foot and walk without support. CONCLUSIONS: To our knowledge, our case is the first pediatric case with the coexistence of Sjögren`s syndrome and CIDP. Therefore, we suggest investigating children with CIDP in terms of underlying autoimmune diseases such as Sjögren`s syndrome.

Impaired aortic function in patients with coeliac disease.

BACKGROUND AND AIM: We aimed to investigate the association between aortic function (aortic stiffness index, aortic strain, and aortic distensibility), which is a predictor of atherosclerosis, and coeliac disease (CD). METHODS: Thirty-six patients with CD and 35 control subjects were included in the study. Serological screening was performed to determine the levels of auto-immune markers, including anti-gliadin immunoglobulin (Ig)A and IgG, and anti-tissue transglutaminase antibodies. Aortic distensibility, aortic strain, and aortic stiffness index were calculated using echocardiography. RESULTS: Aortic strain and aortic distensibility were significantly lower in patients with CD than in control subjects (0.07 [0.03-0.14] vs. 0.09 [0.06-0.15], p < 0.001; 0.0036 ± 0.0012 vs. 0.0051 ± 0.0014, p < 0.001, respectively). However, the aortic stiffness index was significantly higher in patients with CD than in controls (1.14 [0.57-2.69] vs. 0.91 [0.59-1.92], p = 0.002). Coeliac disease was the only independent parameter that was correlated with aortic strain, aortic stiffness index, and aortic distensibility (b = -0.427, p < 0.001; b = 0.375, p = 0.003; b = -0.434, p < 0.001, respectively). CONCLUSIONS: In this study, we showed deteriorated aortic functions by echocardiography in CD patients, which predicted subclinical atherosclerosis. Because deteriorated aortic functions is a strong predictor of future cardiovascular events, close cooperation with cardiologists and gastroenterologists is needed in the management of CD patients, and increased awareness of ischaemic heart disease risk factors in these patients and healthcare providers is warranted.