primary immune deficiency,immunoglobulin deficiency,common variable immune deficiency

1. Fawzeia Abo Ali
Prof. of Internal Medicine &
Immunology

3. The 4 Arms of Immune System & Their
functions:
ADAPTIVE IMMUNITY INNATE IMMUNITY
Humoral (B cell)
1-Encapsulated organisms
(S.pneumo; H. flu)
2-Giardia
Phagocytic
1-Fungi (aspergillus)
2-Bacteria (low low-
virulence organisms
Cellular (T cell)
1-Viruses
2Fungi(candida,aspergillus)
3-Protozoa (Pneumocystis
carinii, toxoplasma)
4-Bacteria (mycobacterium
Complement
1-Bacteria (encapsulated
organisms

4. Primary antibody deficiency Secondary antibody
deficiency
Common variable ID
X liked a gammaglobuinemia
IgA deficiency
IgM deficiency
IgG subclass deficiency
Immunodeficiency with
increased Igm
Transient
hypogammaglobulinaemia of
infancy
protein-losing states
lymphoproliferative
disorders
infections
immunosuppressive
therapies.
anti-CD20 therapies,
such as rituximab

5. Anti-epileptics Immunosuppres
sants
Monoclonal
antibodies
Others
Carbamazepine Azathioprine Belimumab Antimalarial agents
Lamotrigine
Cyclophosphamid
e
Ofatumumab Captopril
Levetiracetam Cyclosporine Rituximab Fenclofenac
Oxcarbazepine Gold salts Imatinib methotrexate
Phenytoin Mycophenolate
Valproate Penicillamine
Steroids
Sulphasalazine

6. x-linked
aγglobulinemia
xLA DiGeorge
Syndrome d
Common Variable Hypoγglobulinemia
/ x-linked hyperIgM syndrome/Selective Ig
deficiency
Primary Immunodeficiencies
Stem Cell
Myeloid
Progenitor
Lymphoid
Progenitor
Neutrophil Monocyte Pre-B Pre-T
Mature B
Plasma
Cell Memory B
Thymus
Reticular Dysgenesis
Severe combined
Immunodeficiency
SCID
Congenital
Agranulocytosis
Chronic
Granulomatous
Disease (x or r)
Bare Lymphocyte
Syndrome
Mature
T

8. B CELL DEFICIENCY
Common variable immundeficiency
X liked a gammaglobuinemia
IgA deficiency
IgM deficiency
IgG subclass deficiency
hyperIgm syndrome
Transient hypogammaglobulinaemia
of infancy

9. Ten warning signs of PID

10. CVID
CVID is a relatively frequent form of primary
immunodeficiency, found in about 1 in 25,000
persons; this is the reason it is called
“common.”
The degree and type of deficiency of serum
immunoglobulins, and the clinical course, varies
from patient to patient, hence, the word
“variable.”
In still others there are defects of the T-cells.

11. CVID patients are similar to zebras is that if you
look at one hundred zebras none of their stripes
will match exactly

13. Signs and symptoms

15. Dermatologic manifestations:Alopecia
Necrotizing &Nonnecrotizing granulomas
Cutaneous vasculitis including polyarteritis nodosa
A syndrome similar to sarcoidosis can affect
patients with CVID.
Maculopapular rash ,Nodules with ulcerations

16. The diagnostic criteria for CVID include:
Decreased serum IgG level, AND decreased
serum IgA or IgM
Decreased ability to make specific antibodies
in response to immunizations
Exclusion of primary immunodeficiencies
leading to decreased IgG
Exclusion of secondary causes of decreased
serum IgG
Greater than 2 years of age

17. Differential diagnosis
• Cystic fibrosis.
• Allergy.
• Other primary immune deficiencies including:
– Selective IgG subclass deficiency.
– IgA deficiency.
– Bruton's agammaglobulinaemia.
– Severe combined immunodeficiency.
• Protein-losing enteropathy.
• Thymoma.
• Transient hypogammaglobulinaemia secondary to
infection.

21. • Management
• Antibiotics for bacterial infection,
• Immunoglobulin replacement therapy:
– This can be given intravenously or subcutaneously.
– Infusions may delay progression to bronchiectasis by
reducing the number of infections.
– They may improve life expectancy and quality of life.
– Unfortunately, infusions may have no effect on the
autoimmune or granulomatous disease which
accompanies CVID.

22. • Corticosteroids
• TNF-alpha antagonists have also been used
with some degree of success.
• Immunisations
• Inactivated vaccines can be given to patients
with CVID but these may not be effective
because of the underlying antibody deficiency.
• The influenza vaccine is commonly
recommended annually.

23. Prognosis
This depends on:
the extent of lung damage as a
consequence of infection,
severity of autoimmune disease and
the development of a malignancy.
Age of diagnosis