Le Comorbidit nelle immunodeficienze delladulto: quali e quali - - PowerPoint PPT Presentation
Le Comorbidit nelle immunodeficienze delladulto: quali e quali scelte terapeutiche. Andrea Matucci Immunoallergology Unit AOU Careggi, Florence, Italy andrea.matucci@unifi.it XXXII Congresso della SIAAIC Toscana, Emilia Romagna, S.
Andrea Matucci
Immunoallergology Unit AOU Careggi, Florence, Italy andrea.matucci@unifi.it
XXXII Congresso della SIAAIC Toscana, Emilia Romagna, S. Marino 11-12 Novembre 2016
Hypogamma of Unkwnon origin CVID Secondary Hypogamma Agamma sIgAD CRS deficiencies Combined immunodeficiency CD19; 20, 21, 81 defects ICOS, TACI LRBA; NFkB1 PIK3D GOF
Bonilli et al. JACI Pract 2016 (modified)
Cunningan-Rundles; Warnatz K. In: Stiehms – Immuno deficiency (2014)
Infectious manifestations in CVID
High clinical Controllers Low clinical Controllers High IgG serum levels Low IgG serum levels
“NAIVE” PHENOTYPE OF DISEASE
Multiple systems involvement Single system involvement
“CATABOLIC”PHENOTYPE OF IgG; other
Low High
Matucci A., Vultaggio A., Maggi E 2009
Cunningan-Rundles; Warnatz K. In: Stiehms – Immuno deficiency (2014)
Related to chronic respiratory nfectious No related to chronic respiratory nfectious
Extensive bronchiectasis, Interstitial infiltrates, nodular changes
, et al.
1. > Infezioni respiratorie; 2. TC torace positiva 1. no Infezioni respiratorie; 2. TC torace negativa
< Linfociti B memoria IgM+ = Linfociti B memoria IgM+
May co-exist
Chronic protozoal, bacterial; viral infectious.
Villous flattering Celiac disease («like») Inflammatory bowel-like disease Nodular lymphoid hyperplasia
Nodular lymphoid hyperplasia (jeujunum biopsy) Autoimmune enteropaty (jeujunum biopsy)
CYTOPENIAS Trombocytopenic purpura
Or both (Evans syndrome) Autoimmune neutropenia Vitiligo Pernicious anemia Thyroiditis Epatitis Enteropathy Alopecia
Related clinical manifestations
Expansion of mucosa-associated lymphoid tissue (MALT/BALT) Helicobacter pylori infection POTENTIALLY RISK FACTORS
Resnick ES., et al. Blood 2011;119:1650-7
PRIMARY IMMUNODEFICIENCY
SECONDARY IMMUNODEFICIENCY
AUTOIMMUNE DISEASES (IDTP; AEA; PM-DM; APS)
NEUROLOGICAL DISORDERS (MMNP; CIDP; others)
Tang M. Consensus statement: http/www.aiegg.org
NO chronic lung disease Yes bronchiectasis 400 mg/Kg/mo 600 mg/Kg/mo No serious infectious > Moderate bacterial infections OK, follow up
“ … … … … … . t r
g h I g G a n d d
e r e p l a c e m e n t t h e r a p y t
a n t a i n a m i n i m a l i n f e c t i
s b u r d e n i s u n i q u e t
h e i n d i v i d u a l . … . ”
, et al.
500 mg/dl
Post-Ig 7 gg 21 gg 28 gg S.O.D. Immunoallergologia - Careggi
INFUSIONE INFUSIONE
Berger M. Clin Immunol 2004; 112: 1-7
# 900mg/dl
Ig e.v. Ig s.c.
# 100mg/dl
“RESERVOIR SOTTOCUTANEO”
plasmatico lineare;
Circolo ematico: rapida distribuzione compartimenti extra-vascolari e degradazione
et al.
+ +/-
Haemophilus influenzae Streptococcus pneumoniae
Pseudomonas aeruginosa Mycoplasma pneumoniae Campylobacter Yersinia enteroolitica Giardia lamblia
Chronic airway disease: Bronchiectasis. Chronic interstitial d.: fibrosis Villous flattering Celiac disease («like») Inflammatory bowel-like disease Nodular lymphoid hyperplasia
Higher doses of Ig (600mg/kg/month) (?) Azitromycine Systemic steroids Immunosuppressors (CoA; AZA) Rituximab Diet without gluten
Granulomatous d.: Sarcoidosis-like
Systemic steroids Immunosuppressors (CoA; AZA; Micofen.) idroxyclorochine TNF-α inhibitors 5-aminosalicylic acid Immunosuppressors (AZA; 6-mercaptopur.) Non-absorbed oral CCS TNF-α inhibitors No specific treatment (yes for diarrea, etc)
Immunoallergology (F. Almerigogna) Immunology and Cellular Therapy (E. Maggi)
Alessandra Vultaggio Oliviero Rossi Francesca Nencini Sara Pratesi Francesca Zanieri University of Florence, Italy Careggi Hospital Carolina Orsi Battaglini Giulia Carli Anna Radice