Aims and objectives
The aim of our study was to assess the evolution of hepatosplenic involvement during medium and long-term follow-up in patients with Common Variable Immunodeficiency (CVID) by annual abdominal ultrasound (US).
Methods and materials
We retrospectively analyzed the annual abdominal US of 52 patients (29 male; 23 female),
who referred for CVID to the Immunodeficience Unit of the University of Naples Federico II between 2004 and 2017.
They were followed-up for at least 4 years and up to 13 years.
In particular,
we evaluated the longitudinal diameters of each liver lobe (right,
left and caudate),
the splenic longitudinal diameter,
the portal vein diameter,
the liver and the spleen echotexture.
Results
In all of our patients we observed a significant and progressive increase of the liver and spleen size.
In particular,
we noticed in a subgroup (10 patients; 20%) of our sample that the liver longitudinal diameters were constantly augmented and a progressive echotexture alteration was associated.
Indeed,
at first,
the liver appeared inhomogeneously hyperechoic (bright liver),
then its surface was irregular and finally a coarse liver pattern was noticed.
Furthermore,
these ultrasonographic features preceded the alteration of the laboratory parameters.
In all patients the portal...
Conclusion
In according to the literature (1,
2,
3),
our data confirm the progressive hepato-splenic damage in patients with CVID.
Nowadays,
the pathological mechanisms are poorly defined,
Pulvirenti et al.
(4) proposed the involvement of primary antibody deficient,
frequent inflammations and lymphocyte abnormalities,
typical of these patients.
In this contest,
the abdominal US,
a free-radiation,
widely available and repeatable exam,
plays an important role and to perform it regularly is fundamental (5),
in order to identify precociously the subgroup of patients in which the liver damage...
Personal information
M.
Caruso,
MD,
University of Naples "Federico II",
Department of Advanced Biomedical Sciences,
Via S.
Pansini,
5,
80131,
Naples,
Italy.
Phone: +393478412472; +39 081 7463102;
e-mail:
[email protected]
References
Patuzzo et al,
Autoimmunity and infection in common variable immunodeficiency (CVID); Autoimmunity Reviews 15 (2016).
Malamut et al,
Nodular regenerative hyperplasia: The main liver disease in patients with primary hypogammaglobulinemia and hepatic abnormalities; Journal of Hepatology 48 (2008).
Webster A,
Clinical and Immunological Spectrum of Common Variable Immunodeficiency (CVID); Iranian Journalof allergy,
asthmaand immunology/103 (2004).
Pulvirenti et al,Idiopathic Non Cirrhotic Portal Hypertension and Spleno-Portal Axis Abnormalities in Patients with Severe Primary Antibody Deficiencies;Journal of Immunology Research (2014).
Abbott et al,
Common Variable Immunodeficiency Diagnosis,
management...