Crohn's disease and Takayasu's arteritis: are they associated?

OBJECTIVE: Different types of vasculitis can occur in patients with inflammatory bowel disease [IBD], but large vessels vasculitis seems to be the most prevalent. Indeed, the presence of both Crohn's disease [CD] and Takayasu's arteritis [TAK] has previously been reported, with higher prevalence in young women between the second and the third decade of life. This article aims to provide clinicians with an accurate picture of the most common clinical features and current treatment strategy for patients with both CD and TAK. PATIENTS AND METHODS: We described the coexistence of CD and TAK in three young women and also performed an extensive literature review about the association of these two immune-related disorders. Research on PubMed server was performed typing the terms "Takayasu's arteritis and inflammatory bowel disease", "Takayasu's arteritis and Crohn's disease", and "Takayasu's arteritis and Ulcerative colitis". RESULTS: Although the association of CD with TAK is uncommon, due to the severity of both diseases, concomitance in the same patient may significantly complicate the diagnostic and therapeutic work-up. In addition, since TAK can compromise intestinal vasculature, it may possibly exacerbate the clinical course of patients with IBD. All patients we reported underwent surgery due to IBD complications and two of them started biological therapy with different outcomes. CONCLUSIONS: Early detention of these conditions has a great importance for both gastroenterologists and immunologists, for ensuring a tailored multidisciplinary management, possibly in order to identify a common therapy for these two immune-related disorders.

Nailfold Videocapillaroscopy Findings in Bradykinin-Mediated Angioedema.

BACKGROUND AND OBJECTIVE: Hereditary angioedema with C1-inhibitor deficiency (C1-INH-HAE) and acquired angioedema related to angiotensin-converting enzyme (ACE) inhibitors (ACEI-AAE) are types of bradykinin-mediated angioedema without wheals characterized by recurrent swelling episodes. Recent evidence suggests that a state of "vascular preconditioning" predisposes individuals to attacks, although no data are available on possible structural alterations of the vessels. Objective: This study aims to compare the features of nailfold capillaries to highlight possible structural anomalies between patients affected by C1-INH-HAE and controls and between patients with ACEI-AAE and hypertensive controls. METHODS: We used nailfold videocapillaroscopy (NVC) to assess the following: apical, internal, and external diameter; loop length; intercapillary distance; and capillary density, distribution, and morphology. Plasma levels of vascular endothelial growth factor (VEGF) A, VEGF-C, angiopoietin (Ang) 1, and Ang2 were also measured. RESULTS: Compared with healthy controls (n=28), C1-INH-HAE patients (n = 34) were characterized by significant structural alterations of the capillaries, such as greater intercapillary distance (216 vs 190 µm), increased apical, internal, and external diameter (28 vs 22 µm; 22 vs 20 µm; and 81 vs 65 µm, respectively), decreased density (4 vs 5 capillaries/mm2), more irregular capillary distribution, and more tortuous morphology. Apical diameter was enlarged in patients with ≥12 attacks per year. In ACEI-AAE patients, NVC showed no alterations with respect to hypertensive controls. NVC performed in 2 C1-INH-HAE patients during attacks showed no changes compared with the remission phase. CONCLUSIONS: We detected major structural capillary alterations in C1-INH-HAE patients, thus confirming the involvement of microcirculation in the pathogenesis of angioedema.

Nailfold Videocapillaroscopy Findings in Bradykinin-Mediated Angioedema

Background: Hereditary angioedema with C1-inhibitor deficiency (C1-INH-HAE) and acquired angioedema related to angiotensin-converting enzyme (ACE) inhibitors (ACEI-AAE) are types of bradykinin-mediated angioedema without wheals characterized by recurrent swelling episodes. Recent evidence suggests that a state of “vascular preconditioning” predisposes individuals to attacks, although no data are available on possible structural alterations of the vessels. Objective: This study aims to compare the features of nailfold capillaries to highlight possible structural anomalies between patients affected by C1-INH-HAE and controls and between patients with ACEI-AAE and hypertensive controls.Methods: We used nailfold videocapillaroscopy (NVC) to assess the following: apical, internal, and external diameter; loop length; intercapillary distance; and capillary density, distribution, and morphology. Plasma levels of vascular endothelial growth factor (VEGF) A, VEGF-C, angiopoietin (Ang) 1, and Ang2 were also measured. Results: Compared with healthy controls (n=28), C1-INH-HAE patients (n = 34) were characterized by significant structural alterations of the capillaries, such as greater intercapillary distance (216 vs 190 μm), increased apical, internal, and external diameter (28 vs 22 μm; 22 vs 20 μm; and 81 vs 65 μm, respectively), decreased density (4 vs 5 capillaries/mm2), more irregular capillary distribution, and more tortuous morphology. Apical diameter was enlarged in patients with ≥12 attacks per year. In ACEI-AAE patients, NVC showed no alterations with respect to hypertensive controls. NVC performed in 2 C1-INH-HAE patients during attacks showed no changes compared with the remission phase. Conclusions: We detected major structural capillary alterations in C1-INH-HAE patients, thus confirming the involvement of microcirculation in the pathogenesis of angioedema (AU)

Antecedentes: Tanto el angioedema hereditario con deficiencia de inhibidor del C1 (C1-INH-HAE) como el angioedema adquiridorelacionado con los inhibidores de la ECA (ACEI-AAE), son dos tipos de angioedema mediados por bradicinina que cursan con episodiosde inflamación recurrente sin acompañarse de habones. Existe evidencia de la existencia de un estado de "preacondicionamiento vascular"que predispone a estos pacientes a los ataques, pero no hay datos disponibles sobre las posibles alteraciones estructurales de los vasos.Objetivo: Este estudio tiene como objetivo el evaluar las características de los capilares de la base ungueal para identificar posiblesanomalías estructurales en los pacientes afectados por C1-INH-HAE en comparación con la población sana, y en los pacientes con ACEIAAE en comparación con controles con hipertensión arterial.Métodos: Mediante videocapilaroscopia de la base ungueal (NVC), se evaluaron: los diámetros apical, interno y externo, la longitud delasa, la distancia intercapilar, la densidad capilar, su distribución y su morfología. También se midieron los niveles plasmáticos del factorde crecimiento endotelial vascular (VEGF)-A, VEGF-C, angiopoyetina (Ang)1 y Ang2.Resultados: En los pacientes con C1-INH-HAE (n = 34) se observaron alteraciones estructurales de los capilares significativas, en comparacióncon los controles sanos (n = 28): mayor distancia intercapilar (216 frente a 190 µm), aumento del diámetro apical, interno y externo(28 frente a 22 µm; 22 frente a 20 µm; y 81 frente a 65 µm, respectivamente), disminución de la densidad (4 frente a 5 capilares/mm2),distribución capilar más irregular y una morfología más tortuosa. El diámetro apical fue mayor en aquellos pacientes con ≥12 ataques/año. En los pacientes con ACEI-AAE, las NVC no mostraron alteraciones al ser comparadas con las de los controles hipertensos. Las NVC realizadas en dos pacientes ...(AU)

Component resolved diagnosis by recombinant allergens in patients with allergies to inhalants.

Molecular characterization of IgE reactivity of specific individual components of allergenic extracts is now possible due to the technology of recombinant allergens derived from studies of molecular biology of allergic pathology. The identification of the immunoreactivity to single allergenic components in allergic subjects allows to specifically define her/his allergic profile and obtain the so-termed Component Resolved Diagnosis (CRD). Molecular allergens can be classified into those that induce the respiratory allergic reactivity and those that identify the food-related allergic pathology. It is also essential to identify those molecular allergens whose immunoreactivity is able to connect the two clinical conditions: respiratory symptoms and food allergy symptoms. The present study was conducted on 50 patients with a clinical history of hypersensitivity to pollen and/or allergy and positivity to Skin Prick Test. The sera were analyzed in our laboratories and the panel of recombinant allergens was applied in the case of positivity of the specific IgE. Of the 50 patients enrolled, 31 were selected as positive to 4 main pan-allergen Bet v1, Par j2, Art v1 and Phl p1; among these, 14 subjects showed one allergen-specific IgE towards natural extracts of tested foods even in absence of clinical history. CRD allows for an increased accuracy in allergy diagnosis and prognosis and plays an important role in: a) resolving genuine vs cross-reactive sensitization in poly-sensitized patients, b) assessing, in selected cases, the risk of severe, systemic vs mild, local reactions in food allergy, and c) identifying patients and triggering allergens for specific immunotherapy (ITS). In light of our results, we believe that the transition from a diagnostic based on the use of allergenic extracts to another one based on the use of single allergenic molecules that is able to define the specific allergenic profile of each patient, seems to be able to revolutionize the allergy diagnosis.