Primary immunodeficiency: Difference between revisions
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==Differential Diagnosis== |
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* '''Humoural''' (65%): B cell defects |
* '''Humoural''' (65%): B cell defects |
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** X-linked agammaglobulinemia |
** [[X-linked agammaglobulinemia]] |
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** Common variable immune deficiency (CVID) |
** [[Common variable immune deficiency]] (CVID) |
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** Transient hypogammaglobulinemia of infancy |
** [[Transient hypogammaglobulinemia of infancy]] |
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** Hyper-IgM syndrome |
** [[Hyper-IgM syndrome]] |
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** IgA deficiency |
** [[IgA deficiency]] |
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* '''Cell-mediated''' (5%): T-cell deficiencies |
* '''Cell-mediated''' (5%): T-cell deficiencies |
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** DiGeorge syndrome |
** [[DiGeorge syndrome]] |
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** Chronic mucocutaneous candidiasis |
** [[Chronic mucocutaneous candidiasis]] |
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* '''Combined''' (15%): B- and T-cell deficiencies, often much more severe |
* '''Combined''' (15%): B- and T-cell deficiencies, often much more severe |
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** Severe combined immunodeficiency disease (SCID) |
** [[Severe combined immunodeficiency disease]] (SCID) |
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** Wiskott-Aldrich syndrome |
** [[Wiskott-Aldrich syndrome]] |
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** Ataxia-telangiectasia |
** [[Ataxia-telangiectasia]] |
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** X-linked lymphoproliferative disease |
** [[X-linked lymphoproliferative disease]] |
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** CD40 ligand deficiency |
** [[CD40 ligand deficiency]] |
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* '''Phagocytic''' (10%): neutrophil deficiencies |
* '''Phagocytic''' (10%): neutrophil deficiencies |
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** Phagocyte deficiencies |
** Phagocyte deficiencies |
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** Chronic granulomatous disease (CGD) |
** [[Chronic granulomatous disease]] (CGD) |
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** Leukocyte adhesion deficiency |
** [[Leukocyte adhesion deficiency]] |
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** [[Hyper-IgE syndrome]] |
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* '''Complement''' (5%) |
* '''Complement''' (5%) |
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** C2 |
** [[C2 deficiency]] |
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** [[Properdin deficiency]] |
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==Red Flags for Immunodeficiency== |
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===Children=== |
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* ≥4 new ear infections in 1 year |
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* ≥2 serious sinus infections in 1 year |
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* ≥2 months on antibiotics with little effect |
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* ≥2 pneumonias in 1 year |
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* Failure to gain weight or grow normally |
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* Recurrent deep skin or organ abscesses |
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* Persistent thrush in mouth or fungal skin infection |
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* Need for IV antibiotics to treat infections |
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* ≥2 deep-seated infections including bacteremia |
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* A family history of primary immunodeficiency |
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===Adults=== |
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* ≥2 new ear infections in 1 year |
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* ≥2 new sinus infections in 1 year (in the absence of allergy) |
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* ≥2 new pneumonias in 2 years |
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* Chronic diarrhea with weight loss |
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* Recurrent viral infections, such as colds, herpes, warts, or condylomata |
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* Recurrent need for IV antibiotics to treat infections |
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* Recurrent, deep abscesses of skin or internal organs |
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* Persistent thrush or fungal infections |
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* Infection with non-tuberculous mycobacteria |
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* A family history of primary immunodeficiency |
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Revision as of 13:29, 10 May 2020
Differential Diagnosis
- Humoural (65%): B cell defects
- Cell-mediated (5%): T-cell deficiencies
- Combined (15%): B- and T-cell deficiencies, often much more severe
- Phagocytic (10%): neutrophil deficiencies
- Phagocyte deficiencies
- Chronic granulomatous disease (CGD)
- Leukocyte adhesion deficiency
- Hyper-IgE syndrome
- Complement (5%)
Red Flags for Immunodeficiency
Children
- ≥4 new ear infections in 1 year
- ≥2 serious sinus infections in 1 year
- ≥2 months on antibiotics with little effect
- ≥2 pneumonias in 1 year
- Failure to gain weight or grow normally
- Recurrent deep skin or organ abscesses
- Persistent thrush in mouth or fungal skin infection
- Need for IV antibiotics to treat infections
- ≥2 deep-seated infections including bacteremia
- A family history of primary immunodeficiency
Adults
- ≥2 new ear infections in 1 year
- ≥2 new sinus infections in 1 year (in the absence of allergy)
- ≥2 new pneumonias in 2 years
- Chronic diarrhea with weight loss
- Recurrent viral infections, such as colds, herpes, warts, or condylomata
- Recurrent need for IV antibiotics to treat infections
- Recurrent, deep abscesses of skin or internal organs
- Persistent thrush or fungal infections
- Infection with non-tuberculous mycobacteria
- A family history of primary immunodeficiency
