Shared epitope and radiologic progression are less prominent in elderly onset RA than young onset RA.

The aim of this study was to determine the influence of HLA-DRB1 and HLA-DQB1 genes on the disease susceptibility and the disease severity in elderly onset rheumatoid arthritis (EORA) compared with young onset rheumatoid arthritis (YORA) in Korean patients. Genetic analysis of HLA-DRB1 and HLA-DQB1 alleles was performed in three groups. Group 1 included 63 patients who were diagnosed with (rheumatoid arthritis) RA after the age of 60 (EORA). Group 2 consisted of 109 patients who were diagnosed with RA before the age of 60 (YORA). Group 3 involved 133 normal controls. The shared-epitope-coding alleles included the members of the HLA-DRB1*04 allele group (*0401, *0404, *0405, *0408, *0410), HLA-DRB1*01 allele group (*0101,*0102), HLA-DRB1*1001, and HLA-DRB1*1402. The disease severity was assessed by the modified total sharp score (mTSS). The shared-epitope-coding alleles were more frequently observed in the RA patients than in the normal controls. The shared-epitope-coding alleles were less frequently found in EORA group than YORA group (31/63 (49.2%) in group 1, 72/109 (66.1%) in group 2, 45/133 (33.8%) group 3, p = 0.02). Although the mTSS of the group 1 was higher than group 2 at symptom onset, the overall mean mTSS of the group 1 was lower than that of group 2 (26.8 vs. 57.5, p < 0.05). HLA-DQ*04 showed the higher frequency in the patients group than in normal controls (p < 0.001). And HLA-DQ*04 was less commonly found in the patients with EORA than YORA (p < 0.05). The influence of shared epitope and HLA-DQ*04 alleles may be less significant on disease susceptibility in EORA. The presence of shared-epitope-coding alleles did not appear to influence on disease severity in EORA patients as well as in YORA patients. Radiologic deterioration in EORA group was less severe than in YORA group. The presence of shared epitope and radiologic progression are less prominent in EORA patients than YORA patients.

[A case of limited Wegener's granulomatosis with gastroenteritis and mesenteric lymphadenopathy].

Wegener's granulomatosis (WG) is a disease characterized by a granulomatous necrotizing vasculitis of small vessels. Although any organ systems can be involved, gastrointestinal involvement in WG is notably uncommon. We present a case of 67-year-old man who was admitted with abdominal pain and diarrhea lasting for 3 weeks. Colonoscopy and abdominal CT scan revealed vasculitis and multiple mesenteric lymphadenopathy. Jejunum and mesenteric lymph nodes biopsies confirmed limited form of WG. The present case indicates that WG might involve only gastrointestinal tract and the histological confirmation is important for diagnosis.

A20 suppresses inflammatory responses and bone destruction in human fibroblast-like synoviocytes and in mice with collagen-induced arthritis.

OBJECTIVE: Nuclear factor-kappaB (NF-kappaB) has been implicated as a therapeutic target for the treatment of rheumatoid arthritis (RA). The purpose of this study was to determine whether A20, a universal inhibitor of NF-kappaB, might have antiarthritic effects. METHODS: An adenovirus containing A20 complementary DNA (AdA20) was used to deliver A20 to human rheumatoid fibroblast-like synoviocytes (FLS) in vitro as well as to mice with collagen-induced arthritis (CIA) in vivo via intraarticular injection into the ankle joints bilaterally. RESULTS: In vitro experiments demonstrated that AdA20 suppressed NF-kappaB activation, chemokine production, and matrix metalloproteinase secretion induced by tumor necrosis factor alpha in FLS. Mice with CIA that were treated with AdA20 had a lower cumulative disease incidence and severity of arthritis, based on hind paw thickness, radiologic and histopathologic findings, and inflammatory cytokine levels, than did control virus-injected mice. The protective effects of AdA20 were mediated by the inhibition of the NF-kappaB signaling pathway. The severity of arthritis was also significantly decreased in the untreated front paws, indicating a beneficial systemic effect of local suppression of NF-kappaB. Surprisingly, mice treated with AdA20 after the onset of CIA had significantly decreased arthritis severity from the onset of clinical signs to the end of the study. CONCLUSION: These results suggest that using A20 to block the NF-kappaB pathway in rheumatoid joints reduces both the inflammatory response and the tissue destruction. The development of an immunoregulatory strategy based on A20 may therefore have therapeutic potential in the treatment of RA.

A case of Hughes-Stovin syndrome associated with hyperhomocysteinemia.

We report a case of Hughes-Stovin syndrome (HSS) associated with hyperhomocysteinemia. A 24-year-old man who has no clinical features suggestive of Behcet's disease was admitted for hemoptysis and dyspnea. Radiological and laboratory evaluation revealed multifocal pulmonary artery aneurysms involving bilateral segmental pulmonary artery, thrombi in right atrium and ventricle, and hyperhomocysteinemia. Accordingly, HSS associated with hyperhomocysteinemia was diagnosed, and the clinical and radiological improvement was achieved after treatment with prednisolone, warfarin, and folic acid.

The diagnostic accuracy of power Doppler ultrasonography for differentiating secondary from primary Raynaud's phenomenon in undifferentiated connective tissue disease.

This study was conducted to evaluate the diagnostic accuracy of power Doppler ultrasonography (PDU) for differentiating secondary from primary Raynaud's phenomenon (RP), and also compared PDU with nailfold capillaroscopy (NFC) for the assessment of microvascularity in undifferentiated connective tissue disease (UCTD) patients with RP. Microvascularity in the nailfold and finger tip was evaluated using PDU with cold challenge, and the findings of PDU were classified according to the qualitative grading system before and after cold challenge. NFC was performed at the same day in all persons. The results of PDU were compared with the clinical, laboratory data, and the findings of NFC. The 14 UCTD patients with RP were included in our study. Seven patients were suspected of secondary RP in NFC examination, thus NFC yielded a correct classification into secondary RP in 50% of the UCTD patients. The PDU finding of pattern II, which is regarded as the finding of secondary RP, was observed in 12 UCTD patients with RP. Thus, PDU yielded a correct classification into secondary RP in 86% of UCTD patients. In conclusion, we confirmed that PDU has a higher correct classification rate than NFC for the diagnosing of secondary RP in UCTD patients. Our results suggest that PDU has better accuracy than NFC in differentiating secondary from primary RP, and PDU is more useful in assessing microvascular abnormalities in UCTD patients with RP.