Lysozyme determination in parotid saliva from patients with Sjögren's syndrome

Lysozyme and total protein concentrations in parotid saliva were measured in 17 patients with primary Sjögren's syndrome, in six patients with Sjögren's syndrome secondary to hyperlipoproteinemia and in 14 age- and sex-matched healthy control subjects. Increased lysozyme concentrations were found only in patients with primary Sjögren's syndrome and correlated well with the presence of parotid gland enlargement. The total protein concentration in the saliva of patients with Sjogren's syndrome was not different from that of the control subjects. Parotid saliva lysozyme determination may be useful as an early adjunctive diagnostic test of primary Sjögren's syndrome.     

Pulmonary capillary endothelial dysfunction in early systemic sclerosis

OBJECTIVE: Pulmonary capillary endothelium-bound angiotensin-converting enzyme (PCEB-ACE) activity is a sensitive and quantifiable index of endothelial function in vivo. Systemic sclerosis (SSc) is characterized by endothelial damage and excess collagen formation, causing mainly pulmonary hypertension (PH) in the limited cutaneous SSc (lcSSc) subset and interstitial lung disease with pulmonary interstitial fibrosis (PIF) in the diffuse cutaneous SSc (dcSSc) subset. This study was undertaken to investigate the hypothesis that PCEB-ACE activity is reduced early in SSc, in the absence of PH or PIF. METHODS: Applying indicator-dilution techniques, we measured single-pass transpulmonary hydrolysis and percent metabolism (%M) of a synthetic ACE substrate and calculated functional capillary surface area (FCSA) in 25 SSc patients and 11 controls. Substrate hydrolysis and %M reflect ACE activity per capillary; FCSA reflects ACE activity per vascular bed. RESULTS: PCEB-ACE activity was decreased in both SSc subsets. Among patients without PH, substrate hydrolysis and %M were decreased in patients with lcSSc and more profoundly in those with dcSSc; loss of FCSA normalized to body surface area (FCSA/BSA) was observed in dcSSc, but not in lcSSc. High-resolution computed tomography of the lung, performed in all SSc patients, revealed no correlation between substrate %M, hydrolysis, or FCSA/BSA and the degree of PIF; 5 dcSSc and 5 lcSSc patients with no detectable PIF exhibited decreases in hydrolysis and %M, while FCSA/BSA was decreased only in dcSSc. CONCLUSION: Depression of PCEB-ACE activity, indicating pulmonary endothelial dysfunction, occurs early in SSc, in the absence of PH or PIF, and is more pronounced, at this early pulmonary disease stage, in dcSSc than in lcSSc.     

HLA class II sequence polymorphisms and susceptibility to rheumatoid arthritis in Greeks. The HLA-DR beta shared-epitope hypothesis accounts for the disease in only a minority of Greek patients.

OBJECTIVE. In Northern Europeans, rheumatoid arthritis (RA) is strongly associated with a relatively conserved pentapeptide sequence of HLA-DR beta found notably in the HLA-DR4 subtypes Dw4 and Dw14 and in DR1. A previous serologic study of HLA class II polymorphism in a Greek population with RA failed to show significant associations with any antigen. METHODS. We characterized HLA-DRB polymorphisms in Greek patients with RA and in control subjects by restriction fragment length polymorphism analysis. Allelic DRB subtypes were examined by polymerase chain reaction amplification and oligonucleotide hybridization. RESULTS. DNA analysis in the RA patients showed that although individual HLA-DR allelic associations were weak, a relatively conserved HLA-DR beta motif was significantly associated with RA in this population of Greek patients. The third hypervariable region amino acid sequences QRRAA, QKRAA, or RRRAA were found in the HLA-DR beta 1 of 43.5% of the RA patients versus 15.5% of the controls (uncorrected P = 0.00004). CONCLUSION. Sequences shown to influence susceptibility to RA in patients in the UK also play a role in patients in Greece. However, 57% of Greek patients lack the putative HLA-DR beta motif, which suggests that considerable immunogenetic heterogeneity underlies disease susceptibility in this population.     

Asymptomatic avascular necrosis in patients with primary antiphospholipid syndrome in the absence of corticosteroid use: a prospective study by magnetic resonance imaging

OBJECTIVE: To evaluate the prevalence of avascular necrosis (AVN), using magnetic resonance imaging (MRI), in patients with primary antiphospholipid syndrome (APS) and in patients with systemic lupus erythematosus (SLE), with or without anticardiolipin antibodies (aCL), who are asymptomatic for AVN and have not taken corticosteroids. METHODS: Seventy-nine subjects who were asymptomatic for AVN were evaluated by MRI of the femoral heads: 30 patients with primary APS who had never received corticosteroids, 19 SLE patients who had never received steroids (divided into 2 groups, aCL positive and aCL negative, in order to examine any association between AVN and aCL), and 30 healthy subjects who were age- and sex-matched with patients with primary APS. Established MRI criteria were used for a diagnosis of AVN. RESULTS: Asymptomatic AVN was evident in 6 (20%) of 30 patients with primary APS: 3 of them (1 man, 2 women) had intermediate bilateral AVN, and 3 (all women) had early AVN (bilateral in 1 patient). Results of hip and pelvis radiography and dynamic scintigraphy were negative. Followup MRI 6 months later revealed no changes. At the time of the initial MRI examination, the mean (+/-SD) age of patients in whom AVN was identified was 31.2 +/- 7.3 years, and that of patients without AVN was 42.4 +/- 11.9 years (P = 0.036). Livedo reticularis occurred significantly more commonly in the group with AVN (P = 0.041). None of the healthy subjects and none of the patients with SLE demonstrated AVN on MRI. CONCLUSION: AVN can be detected by MRI in 20% of patients with primary APS. Younger patients tend to develop AVN more frequently than do older patients, and the presence of livedo reticularis may identify individuals at risk for AVN. Clinicians should be aware of this possible clinical manifestation of primary APS, because early diagnosis can lead to early intervention.     

Tuberculous spondylitis: a cause for paraplegia in lupus

Summary A case of systemic lupus erythematosus (SLE) with paraplegia due to tuberculous spondylitis is presented.