Patient | Fever | Elevation of Acute-phase Reactants | Urticarial Rash | Arthralgia/arthritis | Visceromegaly/abdominal Pain | Conjunctivitis | Headache | Hearing Loss | Papilledema | Typical Facies | Bone Overgrowth | Mental Retardation | Treatment | Final Diagnosis |
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
S1 | + | + | + | +/− | −/− | − | − | − | − | − | − | − | Steroids/NSAID | Alternative diagnosis |
S2 | + | + | + | +/− | −/− | − | − | − | − | − | − | − | Steroids/NSAID | Alternative diagnosis |
S3 | + | + | + | +/− | −/− | − | − | − | − | − | − | − | Steroids/NSAID | Alternative diagnosis |
S4 | + | + | + | +/− | −/− | + | + | − | − | − | − | − | Steroids/NSAID/anti-TNF/anti-IL-1 | Alternative diagnosis |
S5 | − | − | + | −/− | −/− | + | + | − | − | − | − | − | Steroids/NSAID | Idiopathic urticaria |
S6 | + | + | + | +/− | −/− | + | + | − | − | − | − | − | NSAID/anti-TNF/anti-IL-1 | Alternative diagnosis |
S7 | + | + | + | +/− | −/− | − | + | − | − | − | − | − | NSAID/anti-TNF | Alternative diagnosis |
S8 | + | + | + | +/− | −/− | + | − | − | − | − | − | − | NSAID/anti-IL-1 | Alternative diagnosis |
S9 | + | + | − | −/− | −/− | + | + | − | − | − | − | − | NSAID/anti-IL-1 | Undifferentiated periodic fevers |
S10 | + | + | + | +/− | −/− | − | + | − | − | − | − | − | Steroids/NSAID/colchicine | Alternative diagnosis |
S11 | + | + | + | +/− | −/− | + | + | − | − | − | − | − | Steroids/NSAID | Alternative diagnosis |
S12 | + | + | + | +/− | −/− | − | + | − | − | − | − | − | Steroids/NSAID | Alternative diagnosis |
S13 | + | + | + | +/− | −/− | − | + | − | − | − | − | − | Steroids/NSAID | Alternative diagnosis |
S14 | − | − | + | +/− | −/− | − | − | − | − | − | − | − | Steroids/NSAID | Idiopathic urticaria |
S15 | − | + | + | +/+ | −/− | − | − | − | − | − | − | − | Steroids/NSAID | Alternative diagnosis |
S16 | − | + | + | −/− | −/− | − | − | − | − | − | − | − | Steroids/NSAID | Alternative diagnosis |
S17 | + | + | − | −/− | +/+ | − | − | − | − | − | − | − | Steroids/colchicine | Undifferentiated periodic fevers |
S18 | − | − | + | −/− | −/− | − | − | − | − | − | − | − | — | Idiopathic urticaria |
S19 | − | − | + | −/− | −/− | − | − | − | − | − | − | − | Steroids/NSAID | Idiopathic urticaria |
S20 | + | + | + | +/+ | −/− | − | − | − | − | − | − | − | Anti-IL-1 | SoJIA |
S21 | + | + | + | +/+ | −/− | − | + | − | − | − | − | − | − | Alternative diagnosis |
S22 | + | + | + | +/+ | −/− | − | − | − | − | − | − | − | Anti-IL-1 | Alternative diagnosis |
S23 | − | − | + | −/− | −/− | − | − | − | − | − | − | − | — | Idiopathic urticaria |
S24 | + | + | + | +/− | −/− | + | + | − | − | + | + | + | Steroids/NSAID | Alternative diagnosis |
S25 | + | + | − | +/− | −/− | − | + | − | − | − | − | − | Steroids/NSAID | Undifferentiated periodic fevers |
S26 | + | + | +* | +/+ | −/− | − | + | + | − | + | + | + | Anti-IL-1 | CINCA |
S27 | + | + | + | +/+ | −/− | + | − | − | − | − | − | − | Steroids/NSAID/CSA/MTX/AZA/MMF/anti-IL-1 | Alternative diagnosis |
S28 | + | + | + | +/+ | −/− | − | − | − | − | − | − | − | Steroids/NSAID/CSA/MTX/Anti-TNF/anti-IL-1 | SoJIA |
S29 | + | + | + | +/+ | −/− | + | − | − | − | − | − | − | Steroids/anti-IL-1 | Alternative diagnosis |
S30 | + | + | + | +/+ | −/− | − | − | − | − | − | − | − | Steroids/NSAID/MTX/anti-TNF/anti-IL-1 | SoJIA |
S31 | − | + | + | +/+ | −/− | − | − | − | − | − | − | − | Steroids/CSA/colchicine/anti-IL-1 | Alternative diagnosis |
S32 | + | + | + | +/+ | −/− | − | − | − | − | − | − | − | Steroids/NSAID/CSA | Alternative diagnosis |
S33 | + | + | − | −/− | +/+ | − | − | − | − | − | − | − | Steroids | Undifferentiated periodic fevers |
S34 | − | − | − | +/− | −/− | − | − | − | − | − | + | − | Steroids/NSAID | Primary hypertrophic osteoarthropathy |
S35 | + | + | + | +/+ | −/− | − | − | − | − | − | − | − | Steroids/NSAID/anti-IL-1 | Alternative diagnosis |
S36 | + | + | + | +/+ | −/− | − | − | − | − | − | − | − | Anti-IL-1 | MWS |
↵* Absence of typical daily urticarial rash, few episodes of diffuse erythematous rash at limbs. NSAID: nonsteroidal antiinflammatory drugs; anti-TNF: antitumor necrosis factor; anti-IL-1: anti-interleukin 1; CSA: cyclosporine A; MTX: methotrexate; AZA: azathioprine; MMF: mycophenolate mofetil; SoJIA: systemic-onset juvenile idiopathic arthritis; CINCA: chronic infantile neurologic, cutaneous, articular syndrome; MWS: Muckle-Wells syndrome.