Study of idiopathic inflammatory myopathies with special reference to borderland between idiopathic inflammatory myopathies and muscular dystrophies.
BACKGROUND
Idiopathic inflammatory myopathies (IIMs) form important treatable myopathies, hence it is important to recognize and categorize them. In some cases, the differential diagnosis between IIM and muscular dystrophies can be difficult.


OBJECTIVE
To study the clinical and laboratory features of patients with IIMs and compare and contrast this group with limb girdle muscular dystrophies (LGMDs).


METHODS
A prospective study for the period of five years [1999-2004] was undertaken at a tertiary neuromuscular center.


METHODS
Bohan and Peter criteria were used for the diagnosis of IIM and Bushby criteria were used for the diagnosis of LGMD. Patients underwent history, clinical examination, hematological tests, electrophysiological studies and muscle biopsy. The biopsies were studied for histology and immunocytochemistry. A clinical scoring system was evolved to differentiate IIM from LGMD and was validated in a blinded manner. Receiver operator curves were used as the statistical method to analyze the sensitivity and specificity.


CONCLUSIONS
In the IIM group, dermatomyositis was most common, followed by polymyositis, occurring in young females. Overlap group was less common. In patients with polymyositis, onset in upper girdle was associated with adverse outcome. The scoring system helped to differentiate IIM from LGMD, mainly using clinical pointers. This was particularly valuable in chronic cases.
Khadilkar
Satish V
SV
Department of Neurology, Grant Medical College and Sir JJ Group of Hospitals, Mumbai, India. Khadilkar@vsnl.com
Patil
Shekhar G
SG
Amin
Sanjeev N
SN
D000293:Adolescent; D000328:Adult; D001706:Biopsy / Q000379:methods; D019703:Calcineurin / Q000378:metabolism; D002648:Child; D003402:Creatine Kinase / Q000097:blood; D003625:Data Collection; D003937:Diagnosis, Differential; D004311:Double-Blind Method; D000073939:Dysferlin; D005260:Female; D006801:Humans; D008297:Male; D008565:Membrane Proteins / Q000378:metabolism; D008875:Middle Aged; D009124:Muscle Proteins / Q000378:metabolism; D009136:Muscular Dystrophies / Q000175:diagnosis* / Q000378:metabolism; D009220:Myositis / Q000175:diagnosis* / Q000378:metabolism; D011446:Prospective Studies; D012372:ROC Curve; D015203:Reproducibility of Results; D049031:Sarcoglycans / Q000378:metabolism; D012680:Sensitivity and Specificity; D012720:Severity of Illness Index; D055815:Young Adult
C114839:DYSF protein, human; D000073939:Dysferlin; D008565:Membrane Proteins; D009124:Muscle Proteins; D049031:Sarcoglycans; D003402:Creatine Kinase; D019703:Calcineurin