Table 2.

Symptoms and signs that can point towards the presence of PID

Medical history

Recurrent (proven) bacterial infections

Two ore more severe infections (pneumonia, sepsis, meningitis, osteomyelitis)

Atypical presentation of infection

Unusually severe course of infection or impaired response to treatment

Infections caused by an unexpected or opportunistic pathogen

Recurrent infections with the same type of pathogen

Abscesses of internal organs or recurrent subcutaneous abscesses

Failure to thrive with prolonged or recurrent diarrhea

Generalized long-lasting warts or mollusca contagiosa

Extensive prolonged candidiasis (oral/skin)

Delayed (>4 weeks) separation of the umbilical cord

Delayed shedding of primary teeth

Family history of immunodeficiency, unexplained infant deaths, or consanguinity of the parents

Difficult-to-treat obstructive lung disease, unexplained bronchiectasis

Atypical autoimmune disease and/or lymphoproliferation

Physical examination

Dysmorphic features, especially facial abnormalities and microcephaly

Partial albinism, abnormal hair, severe eczema, dermatitis

Telangiectasia, ataxia

Gingivitis, oral ulcers/aphthae

Abnormal wound healing

Absence of immunological tissue (lymph nodes, tonsils)

Lymphadenopathy

Organomegaly

Digital clubbing

Vasculitis

Clin Exp Immunol 2006 and the Jeffrey Modell Foundation warning signs for PID