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Start free with EleplanRecurrent infections associated with rare immunoglobulin isotypes deficiency
ORPHA:183675Disease
Also called IgG subclass deficiency with IgA subclass deficiency · Isolated IgG subclass deficiency · Kappa-chain deficiency · Selective IgG subclass deficiency
What it is
Deficiencies in immunoglobulin (Ig) isotypes (including: isolated IgG subclass deficiency, IgG sublcass deficiency with IgA deficiency and kappa chain deficiency) are primary immunodeficiencies that are often asymptomatic but can be characterized by recurrent, often pyogenic, sinopulmonary infections.
Key facts
- Age of onset
- Childhood
- Inheritance
- Unknown
- Classified as
- Disease
Signs and symptoms
Common30–79%
8Sometimes5–29%
20- Allergic rhinitis
- Arthralgia
- Arthritis
- Atopic dermatitis
- Bronchiectasis
- Bronchitis
- Chronic gastritis
- Chronic sinusitis
and 12 more in this range
Rare1–4%
19- Celiac disease
- Cerebrospinal fluid rhinorrhoea
- Cholangitis
- Cholecystitis
- Diabetes mellitus
- Epididymitis
- Food allergy
- Liver abscess
and 11 more in this range
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
Genes
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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