Objective
To verify whether the prototypical long pentraxin PTX3 represents an indicator of the activity of small‐vessel vasculitis.
Methods
Concentrations of PTX3, a pentraxin induced in endothelium ...by cytokines, were measured by enzyme‐linked immunosorbent assay in the sera of 43 patients with Churg‐Strauss syndrome, Wegener's granulomatosis, and microscopic polyangiitis. PTX3 was also measured in the sera of 28 patients with systemic lupus erythematosus (SLE), 22 with rheumatoid arthritis, and 16 with CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasias). Serum concentrations of C‐reactive protein (CRP) were measured by immunoturbidimetry. The cells involved in PTX3 production in vivo were identified in skin biopsy samples.
Results
Patients with active vasculitis had significantly higher concentrations of PTX3 than did those with quiescent disease (P < 0.001). PTX3 levels in the latter group were similar to those in healthy controls. PTX3 levels were higher in patients with untreated vasculitis and lower in patients who underwent immunosuppressive treatments (P < 0.005). In contrast, patients with active SLE had negligible levels of the pentraxin. PTX3 levels did not correlate with CRP levels in vasculitis patients. Endothelial cells produced PTX3 in active skin lesions.
Conclusion
PTX3 represents a novel acute‐phase reactant produced at sites of active vasculitis.
Objective
To assess fetal and maternal outcomes in women with systemic sclerosis (SSc).
Methods
Prospectively collected data on 99 women with SSc from 25 Italian centers were analyzed ...retrospectively. Women with SSc were observed during 109 pregnancies (from 2000 to 2011), and outcomes were compared to those in the general obstetric population (total of 3,939 deliveries). The maternal age at conception was a mean ± SD 31.8 ± 5.3 years, and the median disease duration at conception was 60 months (range 2–193 months).
Results
SSc patients, compared to the general obstetric population, had a significantly increased frequency of preterm deliveries (25% versus 12%) and severe preterm deliveries (<34 weeks of gestation) (10% versus 5%), intrauterine growth restriction (6% versus 1%), and babies with very‐low birth weight (5% versus 1%). Results of multivariable analysis showed that corticosteroid use was associated with preterm deliveries (odds ratio OR 3.63, 95% confidence interval 95% CI 1.12–11.78), whereas the use of folic acid (OR 0.30, 95% CI 0.10–0.91) and presence of anti–Scl‐70 antibodies (OR 0.26, 95% CI 0.08–0.85) were protective. The disease remained stable in most SSc patients, but there were 4 cases of progression of disease within 1 year from delivery, all in anti–Scl‐70 antibody–positive women, 3 of whom had a disease duration of <3 years.
Conclusion
Women with SSc can have successful pregnancies, but they have a higher‐than‐normal risk of preterm delivery, intrauterine growth restriction, and babies with very‐low birth weight. Progression of the disease during or after pregnancy is rare, but possible. High‐risk multidisciplinary management should be standard for these patients, and pregnancy should be avoided in women with severe organ damage and postponed in women with SSc of recent onset, particularly if the patient is positive for anti–Scl‐70 antibodies.
Pentraxin 3 (PTX3) and complement component C1q are humoral factors of innate immunity, produced at sites of inflammation, and are essential in immune defense against several microbes such as ...Aspergillus, which is commonly implicated in nasal polyposis.
PTX3 and C1q were measured in nasal polyp tissue, normal nasal mucosa, and serum of patients and healthy subjects. Immunohistochemistry for the two proteins was done on normal nasal mucosa and nasal polyps. In addition, PTX3 and C1q production from mononuclear cells from patients and healthy subjects was assessed.
Normal nasal mucosa was found to have 100-fold higher levels of PTX3 compared with serum. No measurable local increase of PTX3 was observed in polyps compared with normal mucosa. Immunohistochemistry revealed PTX3 expression in the lining of blood vessels both within normal mucosa and nasal polyps. PTX3 also was present in mononuclear cells infiltrating nasal polyps. C1q levels were higher in polyps than in normal nasal mucosa.
High levels of PTX3 are present in normal nasal mucosa, suggesting a role in the maintenance of tissue homeostasis. Elevated C1q levels in nasal polyps might be indicative of an ongoing inflammatory response in the nasal mucosa in these patients.
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