Table 1

Patient 1Patient 2Patient 3

Sex/age (y)/race/smokeF/59/Afro-American/—M/60/African/—F/71/Caucasian/—

SymptomsFever, cough, dyspnea, diffuse lung infiltrates, proximal muscle weaknessFever, cough, dyspnea, basilar lung infiltrates, proximal muscle weaknessProximal muscle weakness, severe dysphagia, periungueal erythema, periocular bullous heliotropic lesions
Onset after vaccination 5 days 30 days7 days

CK (nv < 170 U/L) 1-2-32,685-569-986,589-1,700-7521,325-67-20

EMG myogenic alterations+++

AutoimmunityANA 1 : 640 (speckled); ENA- ASMA-; AMA-; ANCA-ANA 1 : 320 (speckled); ENA+ (Jo1); ASMA+ 1 : 160; AMA-; ANCA-ANA 1 : 320 (speckled); ENA+ (Ro/SSA); AMA-; ASMA-; ANCA-

Treatment6-MP 1 mg/Kg/day → slowly tapered to 8 mg/day; Mycophenolate mofetil 2 g/day from February 2010Predn. 1.5 mg/Kg/day → slowly tapered to 25 mg/day Methotrexate 20 mg/week; IVIG 1 mg/kg monthly (February–May 2010); start of Cyclosporine 200 mg/day from September 20106-MP 1 mg/Kg/day → slowly tapered to 16 mg/day; IVIG 1 mg/kg monthly (March–August 2010)

OutcomeRemissionPartially improvedDied in November 2010 (Epstein-Barr virus reactivation)

CK: creatinine kinase (1: at baseline; 2: 1 month after therapy beginning; 3: at the end of followup, in December 2010); EMG: electromyography; ANA: antinuclear antibodies; anti-ENA: antiextractable nuclear antigen antibodies; ASMA: antismooth muscle antibodies; AMA: antimitochondrial antibodies; ANCA: antineutrophil cytoplasmic antibodies; 6-MP: 6-methylprednisolone, Predn: prednisone.