Table 2.

Disease Characteristics at Diagnosis

Pt #	Age (y)	Symptoms	Duration (weeks)	MMT				Rash	Muscle Enzymes					MRI	Other (muscle biopsy, EMG, NCS)
				UE	LE	Neck	Abd		CPK	ALD	AST	ALT	LDH
1	4.3	Rash, myalgias	20	4+	4+	4	4	Gottron's papules, nailfold abnormalities	↑	↑	N	N	↑	N	ND
2	5.2	Fatigue, fever, rash, arthralgias, falling, weakness, “waddling” gait	8	4+	4	4	4	Gottron's papules, heliotrope nailfold abnormalities	↑	↑	N	N	ND	+	EMG +
3	6.2	Fatigue, rash, leg pain, trouble walking up stairs	8	5	4	5	4	Gottron's papules, malar rash, nailfold abnormalities	N	↑	N	N	ND	+	ND
4	7.3	Rash	4	4+	4+	4+	4+	Gottron’s papules, mild heliotrope, malar rash, bilateral ear lesions	N	↑	↑	N	ND	N	ND
5	8.8	Leg pain	52	4+	4+	5	4+	Gottron's papules, nailfold abnormalities	N	N	N	N	ND	+	ND
6	11.0	Fatigue, rash, arthralgias, myalgias, weakness, rash	16	4+	5	4+	5	Gottron’s papules, nailfold abnormalities	N	↑	N	N	↑	N	ND
7	11.1	Fatigue, rash, abdominal pain, difficulty walking and climbing stairs	4	4+	4+	4	5	Gottron's papules, mild heliotrope, nailfold abnormalities,	↑	↑	↑	↑	↑	+	ND
8	12.3	Fatigue, arthralgias, difficulty with ADLs	2	4	4	4	4	Gottron’s papules, mild heliotrope, malar rash, shawl sign	↑	↑	↑	↑	↑	+	ND

MMT = manual muscle testing, UE = upper extremities, LE = lower extremities, Abd = abdominal musculature, CPK = creatine phosphokinase, ALD = aldolase, AST = aspartate aminotransferase, ALT = alanine aminotransferase, LDH = lactate dehydrogenase, MRI = magnetic resonance imaging of proximal thighs, EMG = electromyogram, NCS = nerve conduction study, N = normal, ND = not done, ADLs = activities of daily living