A case of reversible cerebral vasoconstriction syndrome associated with anti-phospholipid antibody syndrome and systemic lupus erythematosus

The pathomechanisms and treatment strategy for rare presentations of reversible cerebral vasoconstriction syndrome (RCVS) with anti-phospholipid syndrome (APS) remain to be determined. We report a 67-year-old woman with APS who presented with ischemic stroke due to RCVS. She was treated with low-dose cilostazol and lomerizine hydrochloride, which resulted in functional improvement and recovery of vasoconstriction within 12 weeks. Her plasma endothelin-1 level was decreased after relief of vasoconstriction, compared with the pre-treatment condition. Increased plasma endothelin-1 may be related to the underlying pathomechanism of RCVS with APS, against which cilostazol and lomerizine hydrochloride could be effective

A case of pulmonary arterial hypertension complicated by anti-neutrophil cytoplasmic antibody-associated vasculitis and systemic sclerosis

Pulmonary arterial hypertension (PAH) is a rare complication of ANCA-associated vasculitis (AAV). We report a 37-year-old man with PAH complicated by both AAV and SSc who presented with dyspnea, cardiac enlargement, positive myeloperoxidase (MPO)-ANCA, anti-centromere antibodies, proteinuria, and urinary casts. Elevated pulmonary arterial pressure (58/22/34 mmHg) and low PAWP (2 mmHg) were confirmed by right heart catheterization. Treatment with glucocorticoids (GC) decreased urinary protein and serum MPO-ANCA; however, PAH did not respond to GC. Therefore, a combination of beraprost, bosentan, and tadalafil was needed. The differences in responses to GC suggest that the pathophysiology of nephropathy is different from that of PAH. We considered that nephropathy was associated with AAV but that PAH was associated with SSc in the present case. We discuss the pathophysiology and treatment response of PAH complicated by AAV, referring to nine past cases

Lactams. XI. Construction of Lactam Carbonyl Function in 1,3-Disubstituted Piperidines by Mercuric Acetate-EDTA Oxidation : Effects of Carbonyl and Related Groups at the 3-Position

1-(3,4-Dimethyoxyphenyl)-2-(3-substituted piperidino) ethanols (3a-d), which carry the carbamoyl, methoxycarbonyl, acetyl, and 1,1-ethylenedioxyethyl group as the 3-substituent in the piperidine ring, have been prepared from 3-substituted pyridines (type 1) through 1-(3,4-dimethoxyphenacyl) pyridinium bromides (type 2). In the mercuric acetate-EDTA oxidation of 3a-d, these 3-substituents have been found to orient the lactam carbonyl formation to the 6-position almost exclusively. It is suggested that the 3-substituents exert both steric and electronic effects

Lactams. XII. Improvements in the Synthesis of Ethyl trans-5-Ethyl-2-oxo-4-piperidineacetate

A highly stereoselective, efficient synthetic route to ethyl trans-5-ethyl-2-oxo-4-piperidineacetate (11a) from 1-benzyl-2,4-dioxo-5-ethylpiperidine (2) is described. The steps involved are conversion of 2 into 1-benzyl-5-ethyl-2-oxo-1,2,5,6-tetrahydropyridine (6) through the lactam alcohol (5), the Michael condensation of 6 with diethyl malonate followed by alkaline hydrolysis, decarboxylation of the resulting trans-lactam dicarboxylic acid (8a) to the trans-lactam acid (9a), and debenzylation of 9a followed by esterification

Preparation of Some Ring-Oxygenated Phenacyl Bromides

3,4-Dimethoxy, 3-benzyloxy-4-methoxy, 3,4,5-trimethoxy, 2-hydroxy-3,4-dimethoxy, and 2-benzyloxy-3,4-dimethoxy derivatives (3a-e) of phenacyl bromide have been prepared in 44-84% yields from the corresponding acetophenones by bromination with bromine in a mixture of ether and chloroform

Serum BAFF and APRIL levels in patients with IgG4-related disease and their clinical significance.

[Introduction]B cell-activating factor of the tumor necrosis factor family (BAFF) and a proliferation-inducing ligand (APRIL) play a crucial role in B cell development, survival, and antibody production. Here we analyzed the serum levels of BAFF and APRIL and their respective clinical associations in patients with an immunoglobulin (Ig) G4-related disease (IgG4-RD). [Methods]We measured serum levels of BAFF and APRIL in patients with IgG4-RD, primary Sjögren's syndrome (pSS), and healthy individuals. Serum BAFF and APRIL levels in IgG4-RD were assessed for correlations with serological parameters, including Ig, particularly IgG4, and the number of affected organs. Serum BAFF and APRIL levels in IgG4-RD were monitored during glucocorticoid (GC) therapy. [Results]Serum BAFF and APRIL levels in patients with IgG4-RD were significantly higher (P < 0.01) than in healthy individuals. The BAFF levels of patients with IgG4-RD were comparable to those of patients with pSS. Although clinical parameters, such as serum IgG4 and the number of affected organs, were not correlated with the levels of BAFF, serum APRIL levels were inversely correlated with serum IgG4 levels (r = -0.626, P < 0.05). While serum BAFF levels decreased following GC therapy, serum APRIL levels increased during follow-up. [Conclusion]These results indicate that BAFF and APRIL might be useful markers for predicting disease activity in IgG4-RD. Further studies are needed to elucidate the role of BAFF and APRIL in the pathogenesis of IgG4-RD