Internist, Rheumatologist Iraj Salehi-Abari The “Bohan and Peter Criteria” is not a good instrument for the diagnosis of Polymyositis/Dermatomyositis Iraj Salehi-Abari, MD. Internist, Rheumatologist Tehran, Iran salehiabari@sina.tums.ac.ir

In the name of God the merciful and compassionate

At first; the presentation of some cases with Polymyositis (PM) or Dermatomyositis (DM)

Case 1: A 48 year old man with: y Symmetric proximal muscle weakness Iraj Salehi-Abari Case 1: A 48 year old man with: Symmetric proximal muscle weakness Elevated AST, ALT and CPK in serum Positive Anti-Jo-1 Abnormal EMG with myogenic pattern No another positive finding y

Case 2: A 41 year old woman with: y Heliotrope rash Iraj Salehi-Abari Case 2: A 41 year old woman with: Heliotrope rash Symmetric proximal muscle weakness Inflammation signaling in muscle MRI No another positive finding y

Case 3: A 51 year old man with: y Symmetric proximal muscle weakness Iraj Salehi-Abari Case 3: A 51 year old man with: Symmetric proximal muscle weakness Positive Anti-SRP Lymphocyte infiltration in muscle pathology No another positive finding y

Case 4: A 45 year old man with: y Shawl sign along with V sign Iraj Salehi-Abari Case 4: A 45 year old man with: Shawl sign along with V sign Elevated AST, ALT and Aldolase in serum Positive Anti-Mi-2 No another positive finding y

Case 5: A 17 year old man with: y Symmetric proximal muscle weakness Iraj Salehi-Abari Case 5: A 17 year old man with: Symmetric proximal muscle weakness Polyarthritis, fever, ILD and Raynaud’s P. Positive Anti-Jo-1 Nail fold telangiectasia & Mechanic’s hands, No another positive finding y

Case 6: A 39 year old woman with: y Symmetric proximal muscle weakness Iraj Salehi-Abari Case 6: A 39 year old woman with: Symmetric proximal muscle weakness Positive Anti-SRP Inflammation signaling in muscle MRI Abnormal EMG with myogenic pattern No another positive finding y

Case 7: A 32 year old woman with: y Gottron’s sign Iraj Salehi-Abari Case 7: A 32 year old woman with: Gottron’s sign Elevated AST, ALT and CPK in serum Abnormal EMG with myogenic pattern No another positive finding y

Case 8: A 55 year old man with: y Symmetric proximal muscle weakness Iraj Salehi-Abari Case 8: A 55 year old man with: Symmetric proximal muscle weakness Elevated AST, Aldolase & CPK in serum Inflammation signaling in muscle MRI Abnormal EMG with myogenic pattern No another positive finding y

Case 9: A 36 year old woman with: y Gottron’s papules & Holster sign Iraj Salehi-Abari Case 9: A 36 year old woman with: Gottron’s papules & Holster sign Symmetric proximal muscle weakness Lymphocyte infiltration along with vasculitis in muscle pathology No another positive finding y

Case 10: A 44 year old woman with: y Gottron’s papules Heliotrope rash Iraj Salehi-Abari Case 10: A 44 year old woman with: Gottron’s papules Heliotrope rash Shawl sign & V sign Symmetric proximal muscle weakness No another positive finding y

Gold standard for the diagnosis of PM/DM: Iraj Salehi-Abari Gold standard for the diagnosis of PM/DM: Is the Clinical/Paraclinical judgement of an expert Rheumatologist, the same as other systemic Rheumatic disease y

Gold standard for the diagnosis of PM/DM: Iraj Salehi-Abari Gold standard for the diagnosis of PM/DM: All of above cases upon the Clinical/ Paraclinical judgement of an expert Rheumatologist are Polymyositis (PM) or Dermatomyositis (DM) y

Bohan and Peter Classification Criteria of PM/DM: Iraj Salehi-Abari Bohan and Peter Classification Criteria of PM/DM: Symmetric proximal muscle weakness Elevated muscle enzymes in serum Abnormal EMG with myogenic pattern Inflammation in muscle pathology Gottron’s papules &/or Heliotrope rash y

Bohan and Peter Classification Criteria of PM/DM: Iraj Salehi-Abari Bohan and Peter Classification Criteria of PM/DM: For definite diagnosis of Polymyositis, we need all first four criteria whereas the combination of characteristic skin rash in the last criteria with at least 3 out of 4 first criteria means definite Dermatomyositis y

Bohan and Peter Classification Criteria of PM/DM: Iraj Salehi-Abari Bohan and Peter Classification Criteria of PM/DM: Anyone of above cases can not be classified as Polymyositis (PM) or Dermatomyositis (DM) according to the Bohan and Peter criteria y

2015 Persian Gulf Criteria (PGC) for Early Diagnosis of Polymyositis: Iraj Salehi-Abari 2015 Persian Gulf Criteria (PGC) for Early Diagnosis of Polymyositis: Clinical Criteria: Symmetric proximal muscle weakness 1 point First-degree family history of muscle weakness -1 point Laboratory Criteria: Elevated muscle enzymes in serum 1 point Myositis Specific Autoantibodies (MSA) 2 points EMG: myogenic (1point) &/or neurogenic pattern (-1point) Muscle inflammation: 1 point Inflammation signaling in muscle MRI or Lymphocyte infiltration &/or Vasculitis in muscle pathology y

2015 Persian Gulf Criteria (PGC) for Early Diagnosis of Polymyositis: Iraj Salehi-Abari 2015 Persian Gulf Criteria (PGC) for Early Diagnosis of Polymyositis: MSA: Anti-Jo-1, Anti-SRP, Anti-Mi-2 For definite diagnosis of Polymyositis we need at least 4 points including 1 point from clinical criteria y

2015 Persian Gulf Criteria for Early Diagnosis of Dermatomyositis: Iraj Salehi-Abari 2015 Persian Gulf Criteria for Early Diagnosis of Dermatomyositis: Cutaneous Criteria: Gottron’s papules or sign 2 points Heliotrope rash 2 points Holster sign 1 point Shawl sign 1 point V sign 1 point Mechanic’s hands 1 point Periungual telangiectasia 1 point Muscular Criteria: The same as Polymyositis y

2015 Persian Gulf Criteria for Early Diagnosis of Dermatomyositis: Iraj Salehi-Abari 2015 Persian Gulf Criteria for Early Diagnosis of Dermatomyositis: For definite diagnosis of Dermatomyositis we need at least 4 points including at least 2 points from cutaneous criteria and at least one point from muscular criteria y

2015 Persian Gulf Criteria for Early Diagnosis of PM/DM: Iraj Salehi-Abari 2015 Persian Gulf Criteria for Early Diagnosis of PM/DM: All of above cases are definite Polymyositis or Dermatomyositis according to the 2015 Persian Gulf Criteria for Early Diagnosis of PM/DM y