RESUMEN
Pulmonary arterial hypertension (PAH) is an uncommon manifestation of systemic lupus erythematosus (SLE), affecting about 0.5% to 23.3% of the population worldwide. The causes of PAH associated with SLE are multifactorial. While it is generally associated with a full-blown picture of SLE, it may rarely be the presenting manifestation of the disease. We describe the case of a middle-aged woman who presented with features of severe PAH due to SLE. She was treated with vasodilators and immunosuppression (steroids and mycophenolate mofetil), with a partial response to treatment at six months follow-up.
RESUMEN
OBJECTIVES: Apolipoprotein A-1 (ApoA-1) is a protein fraction of the high-density lipoproteins with anti-inflammatory and antioxidant properties that play a major role in reverse cholesterol transport. The presence of anti-ApoA-1 IgG has been reported in SLE to be variably associated with disease activity or cardiovascular events (CVEs). We assessed the clinical performance of anti-ApoA-1 IgG and of antibodies directed against its immunodominant F3L1 peptide (F3L1 IgG) in a well-characterized Swiss SLE cohort study. METHODS: A total of 354 biological samples and interviews from 176 individuals were studied. SLEDAI, clinical characteristics, anamnestic CVEs and therapy details were recorded. Sera were tested for the presence of anti-ApoA-1 IgG, anti-F3L1 IgG, anti-dsDNA IgG and aPL. RESULTS: Anti-ApoA-1 and anti-F3L1 IgG positivity was associated with higher SLEDAI, mostly due to concomitant positivity of dsDNA IgG and low complement. Variations in time of anti-ApoA-1 IgG correlated positively with variations of anti-dsDNA IgG and inversely to variations of C3 levels. No cross-reactivity was found between anti-ApoA-1 and anti-dsDNA IgG. Positivity for anti-Apo-A1 IgG was more frequent in individuals receiving 10 mg/day or more of prednisone. We did not find any significant association between anti-ApoA-1 IgG positivity and CVEs. CONCLUSION: Anti-ApoA-1 and anti-F3L1 IgG in SLE correlate strongly with laboratory markers of activity, particularly with the presence and titre of dsDNA IgG. These results confirm and extend previous findings and support the use of anti-ApoA1 IgG in the clinical setting. Their role in CVEs deserves further investigation.
Asunto(s)
Apolipoproteínas A/inmunología , Autoanticuerpos/sangre , Lupus Eritematoso Sistémico/diagnóstico , Adolescente , Adulto , Anciano , Niño , Femenino , Humanos , Lupus Eritematoso Sistémico/sangre , Lupus Eritematoso Sistémico/inmunología , Masculino , Persona de Mediana Edad , Índice de Severidad de la Enfermedad , Adulto JovenRESUMEN
Phenotypic resistance to APC is a complex mechanism associated with increased risk of venous thrombosis in women with recurrent spontaneous abortions. The primary aim of this prospective case control study was to find out the frequencies of different congenital and acquired thrombophilic factors predisposing to APC resistance and to evaluate the strength of their association with recurrent pregnancy losses. FV Leiden accounted for around 40% of all APCR positive patients and the difference in the group frequencies compared with controls, was found to be statistically significant (p=0.001). 18.33% (11/60) FV Leiden-negative APC-resistant patients had FVIII: c values exceeding 95th percentile of the control population (145IU/dL), as compared to 3% in the control group (p=0.001). Mean FVIII level in control subjects was 118±14.0IUdL(-), compared with 127.7±31.2IUdL(-) in the patient group (p=0.009). Apart from FVIII, only the anti-phospholipid antibodies showed a statistically significant association with APCR phenotype (p=0.028), unlike other thrombophilic factors such as Protein C, Protein S, FV levels, HR2 haplotype or other rarer FV variants. The strong positive association of FVL mutation, anti-phospholipid antibodies and elevated FVIII levels with APCR phenotype calls for incorporating them as first line investigations in patients with recurrent spontaneous miscarriages with APCR positivity.