Atypical sarcoidosis

PURPOSE: Sarcoidosis is a granulomatous disease of unknown etiology. Lung and lymphatic system are the principal localisations. Clinical presentations are various depending on involved organs. Some presentations, which are easily diagnosed, are typical and frequent. Atypical forms have unusual presentations and/or are rare. Beside, in a multisystemic sarcoidosis, the affection of only one organ can be unusual. Rigorous diagnosis procedure could avoid errors. CURRENT KNOWLEDGE AND KEY POINTS: Twenty percent of sarcoidosis have atypical presentation. However, each of them are infrequent. Atypical features are wide and can concern pulmonary or extrapulmonary manifestations, general manifestations, blood testing or pathological pattern. FUTURE PROSPECTS AND PROJECTS: Describing atypical forms are necessary for their diagnosis. The diagnosis of atypical sarcoidosis is found on the knowledge of atypical forms previously described, presence of granulomas on specimen biopsy and excluding other granulomatous disease.     

Pediatric Guillain-Barré syndrome: Indicators for a severe course

Objectives:

This study aims to retrospectively evaluate pediatric Guillain-Barré syndrome cases in a tertiary center in Istanbul, Turkey.

Materials and Methods:

The data of 40 patients with Guillain-Barré syndrome who had been admitted to the Department of Pediatrics at the Istanbul University Medical Faculty between 2005 and 2011 were collected. Mann-Whitney U, Kruskal-Wallis, chi-square, and Fisher''s exact tests were used for statistical analysis.

Results:

Mean patient age was 5.4 ± 3.0 years; 20 out of 40 patients (50%) were female and 20 (50%) were male. Preceding infection was detected in 32 cases (80%). Six patients had speech impairment. Out of eight patients with respiratory distress (20%), five required respiratory support (12.5%) of which three of them had speech impairment as well. According to nerve conduction studies, 21 patients (52.5%) had acute inflammatory demyelinating polyradiculoneuropathy, 14 (35%) had acute motor axonal neuropathy, and five (12.5%) had acute motor-sensory axonal neuropathy. Thirty-three patients (82.5%) received intravenous immunglobulin, 3 (7.5%) underwent plasmapheresis and 4 (10%) received both. Time until recovery (P = 0.022) and time until aided (P = 0.036) and unaided (P = 0.027) walking were longer in patients with acute gastrointestinal infection than in those with upper respiratory tract infection (P < 0.05). Time until response to treatment (P = 0.001), time until aided (P = 0.001) and unaided (P = 0.002) walking, and time until complete recovery (P = 0.002) were longer in acute motor axonal neuropathy cases as compared to acute inflammatory demyelinating polyradiculoneuropathy cases.

Conclusion:

Recovery was longer with acute gastrointestinal infection and acute motor axonal neuropathy. Speech impairment could be a clinical clue for the need of mechanical ventilation.     

Flow patterns regulate hyperglycemia-induced subendothelial matrix remodeling during early atherogenesis

Objective

Altered subendothelial matrix composition regulates endothelial dysfunction and early atherosclerotic plaque formation. Hyperglycemia promotes endothelial matrix remodeling associated with multiple microvascular complications of diabetes, but a role for altered matrix composition in diabetic atherogenesis has not been described. Therefore, we sought to characterize the alterations in matrix composition during diabetic atherogenesis using both in vitro and in vivo model systems.

Methods and results

Streptozotocin-induced diabetes in atherosclerosis-prone ApoE knockout mice promoted transitional matrix expression (fibronectin, thrombospondin-1) and deposition in intima of the aortic arch as determined by qRT-PCR array and immunohistochemistry. Early plaque formation occurs at discrete vascular sites exposed to disturbed blood flow patterns, whereas regions exposed to laminar flow are protected. Consistent with this pattern, hyperglycemia-induced transitional matrix deposition was restricted to regions of disturbed blood flow. Laminar flow significantly blunted high glucose-induced fibronectin expression (mRNA and protein) and fibronectin fibrillogenesis in endothelial cell culture models, whereas high glucose-induced fibronectin deposition was similar between disturbed flow and static conditions.

Conclusions

Taken together, these data demonstrate that flow patterns and hyperglycemia coordinately regulate subendothelial fibronectin deposition during early atherogenesis.     

18F-fluorodeoxyglucose positron emission tomography/computer tomography as an objective tool for assessing disease activity in Sjögren's syndrome

Objective

This study aims to determine the value of FDG-PET/CT to assess disease activity in patients with Sjögren's syndrome (SS).

Methods

Thirty-two patients with SS who underwent PET/CT were retrospectively analyzed. PET/CT activity score was measured using a 6-point scale including the 6 following items (0/1: absence or presence of an item): lymphadenopathy on CT, high-resolution CT (HRCT) evidence of interstitial lung disease (ILD), parotid glands SUVmax > 3, submandibular glands SUVmax > 3, lymph node uptake, ILD uptake. Combined PET/CT score was correlated to ESSDAI (EULAR Sjögren's Syndrome Disease Activity Index) score and other parameters of SS activity.

Results

Pathological FDG uptake was observed in 75% of patients (24/32): lymph-nodes (n = 19, 60%), salivary glands (n = 17, 53%), lungs (n = 9, 28%), and thyroid (n = 2). Median ESSDAI and PET/CT activity scores were 9.5 [5–12] and 2 [0–3], respectively. PET/CT activity score correlated with ESSDAI (r = 0.49, p = 0.005), unlike SUVmax. Patients with a high ESSDAI score had a higher PET/CT activity score than patients with a low ESSDAI score (3 vs 1, p = 0.004). PET was also correlated with gammaglobulin levels (r = 0.43, p = 0.02), but not with the presence of cryoglobulinemia, activated complement or beta-2 microglobulin levels. The FDG uptake in patients with lymphoma (n = 4) was higher than in patients without lymphoma (SUVmax = 5.4 vs. 3.2, p = 0.05).

Conclusion

We described a new PET/CT activity score, which correlates to ESSDAI and could help to assess disease activity in SS patients. PET can also help in the diagnosis of lymphoma, even if inflammatory lymph nodes can be frequently observed in SS patients.     

Short-term antidepressant treatment of comorbid dental anxiety in patients with panic disorder

OBJECTIVES: The aims of this study were to determine the frequency of dental anxiety (DA) and dental phobia (DP) in panic disorder, and to follow the changes in DA levels during antidepressant treatment of panic disorder. METHODS: Fifty-three controls and 102 panic disorder patients were assessed using the Structured Clinical Interview (SCID), the Panic-Agoraphobia Scale (PAS), and the Corah Dental Anxiety Scale (DAS). Oral health status was defined by the number of decayed, missing, and filled teeth (DMFT) index. The patients were classified into three groups: (1) those without dental anxiety (WDA), (2) those with dental anxiety (DA), and (3) those with dental phobia (DP). All patients were treated with antidepressants for 3 months and the response rates were assessed. RESULTS: At baseline, DAS was significantly higher in both the DA and the DP groups than in the control group. Ten (9.8%) of the panic disorder patients fulfilled the diagnostic criteria for DP; 31 (30.4%) had severe DA. In the control group, none of the patients was diagnosed as DP, whereas 7 (13.5%) had severe DA. Panic disorder and DA both responded to the antidepressant treatment, but DAS scores remained significantly higher in the DP group than in the DA group and the control group at the end of the third month. CONCLUSIONS: Our data suggest that both DA and DP are more frequent in panic disorder than in healthy controls. Antidepressant treatment may have been helpful in decreasing DA levels in the DA group but not in the DP group.     

Chronic urticaria and monoclonal IgM gammopathy (Schnitzler syndrome): report of 11 cases treated with pefloxacin

BACKGROUND: Schnitzler syndrome is characterized by chronic urticarial rash and monoclonal IgM gammopathy and is sometimes associated with periodic fever, arthralgias, and bone pain. Current treatment is unsatisfactory. OBSERVATIONS: Eleven patients with Schnitzler syndrome were treated with oral pefloxacin mesylate (800 mg/d). In 10 patients, we observed a dramatic and sustained improvement of urticarial and systemic manifestations. Corticosteroid therapy could be stopped or reduced in 6 patients. In 9 patients, pefloxacin was administered for more than 6 months (相似文献