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Table 3 Atypical symptoms in PFAPA with age of onset < 5 vs ≥ 5 years

From: Epidemiology and clinical features of PFAPA: a retrospective cohort study of 336 patients in western Sweden

 

All PFAPA (n = 336)

Onset < 5 years (n = 291)

Onset ≥ 5 years (n = 45)

Any atypical symptom

49/336 (14.6%)

39/291 (13.4%)

10/45 (22.2%)

Skin rash

16/336 (4.8%)

14/291 (4.8%)

2/45 (4.4%)

Cold- or exercise-induced symptoms

12/336 (3.6%)

8/291 (2.7%)

4/45 (8.9%)

Episodes lasting ≥7 days

12/336 (3.6%)

10/291 3.4%)

2/45 (4.4%)

Diarrhea

6/336 (1.8%)

4/291 (1.4%)

2/45 (4.4%)

Chest pain

4/336 (1.2%)

2/291 (0.7%)

2/45 (4.4%)

Conjunctivitis

3/336 (0.9%)

3/291 (1.0%)

0

Arthritis

2/336 (0.6%)

2/291 (0.7%)

0

Periorbital edema

2/336 (0.6%)

2/291 (0.7%)

0

Severe abdominal pain

1/336 (0.3%)

1/291 (0.3%)

0

Sensorineural hearing loss

1/336 (0.3%)

1/291 (0.3%)

0

More than one atypical symptom

8/336 (2.4%)

6/291 (2.1%)

2/49 (4.4%)