Asunto(s)
Anticuerpos Antinucleares/metabolismo , ADN-Topoisomerasas de Tipo I/inmunología , Dermatosis de la Mano/etiología , Esclerodermia Sistémica/complicaciones , Úlcera Cutánea/etiología , Femenino , Dedos , Dermatosis de la Mano/inmunología , Humanos , Masculino , Persona de Mediana Edad , Factores de Riesgo , Esclerodermia Sistémica/inmunología , Úlcera Cutánea/inmunología , Factores de TiempoRESUMEN
Capillaroscopy has high diagnostic and prognostic value in autoimmune connective tissue diseases, in particular systemic sclerosis (SSc). Our working group has developed a consensus on nomenclature, technical equipment, procedure, and diagnostic interpretation of results. The following are required: binocular microscopes with at least 20-/50- and 160-/200-fold magnification and digital archiving. Documentation of defined findings is mandatory. The simultaneous occurrence of, e.g. caliber variations, ectasia, ramifications, elongation (length > 350 microm), torsion (at least two crossing segments per capillary loop), sludge, hemorrhage, and edema is of pathological significance. The isolated occurrence of bushy capillaries (multiple ramifications), thrombosis, giant capillary (capillary lumen > 50 microm), and avascular areas also indicates disease. The latter two findings are highly specific for SSc. Other findings are consistent with connective tissue diseases. These standardized definitions increase quality and comparability of nailfold capillaroscopy in Germany.
Asunto(s)
Enfermedades del Tejido Conjuntivo/diagnóstico , Angioscopía Microscópica/normas , Enfermedad de Raynaud/diagnóstico , Esclerodermia Sistémica/diagnóstico , Terminología como Asunto , Adolescente , Factores de Edad , Síndrome Antifosfolípido/clasificación , Síndrome Antifosfolípido/diagnóstico , Capilares/patología , Niño , Enfermedades del Tejido Conjuntivo/clasificación , Dermatomiositis/clasificación , Dermatomiositis/diagnóstico , Progresión de la Enfermedad , Documentación/métodos , Documentación/normas , Humanos , Lupus Eritematoso Sistémico/diagnóstico , Lupus Eritematoso Sistémico/patología , Angioscopía Microscópica/instrumentación , Angioscopía Microscópica/métodos , Pronóstico , Enfermedad de Raynaud/clasificación , Estándares de Referencia , Valores de Referencia , Esclerodermia Sistémica/clasificaciónRESUMEN
BACKGROUND: Digital ulcers (DU) are a major complication in the course of systemic sclerosis (SSc). In recent years, efficacious, but expensive therapies (e.g. iloprost, sildenafil, bosentan) have been shown to improve healing or to reduce the recurrence of DU. For optimal management it would be useful to identify the risk factors for DU. Such statistical analyses have been rare because they require a high number of patients. OBJECTIVES: To identify potential risk factors for DU in patients with SSc. METHODS: We used the registry of the German Network for Systemic Scleroderma and evaluated the data of 1881 patients included by August 2007. We assessed potential risk factors for DU by comparing patients with (24.1%) and without active DU at time of entry (75.9%). RESULTS: Multivariate analysis revealed that male sex, presence of pulmonary arterial hypertension (PAH), involvement of the oesophagus, diffuse skin sclerosis (only when PAH was present), anti-Scl70 antibodies, young age at onset of Raynaud's phenomenon (RP), and elevated erythrocyte sedimentation rate (ESR) significantly impacted on the appearance of DU. Certain combinations increased the patients' probability of presenting with DU, with the highest probability (88%) for male patients with early onset of RP, ESR>30 mm h(-1), anti-Scl70 antibodies and PAH. Patients with DU developed RP, skin sclerosis and organ involvement approximately 2-3 years earlier than patients without DU. CONCLUSIONS: The results reveal possible risk factors for the occurrence of DU in SSc. As DU are prone to local complications, prophylactic vasoactive treatment for patients presenting with these factors may be justified.