MANAGING THE PATIENT WITH POSITIVE ANA

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MANAGING THE PATIENT WITH POSITIVE ANA Rafael F. Rivas-Chacon, M.D. Disclosures Grant/Research support for: Pfizer Study JIA A3921104 Tofacitinib not related to this presentation 1

Positive Antinuclear Antibody The presence of an Antinuclear Antibody indicates that the immune system is recognizing part of one self as foreign. It is use in clinical practice as a marker of Auto Immune Diseases Positive Antinuclear Antibody A normal person can have a Positive ANA The ANA has value only when it is done in the correct clinical setting. 2

Positive Antinuclear Antibody 3 to 5 % of the normal population has a + ANA and the frequency increase with age 3

Antinuclear Antibody The association of the staining pattern and disease associated antibodies is weak 4

Antinuclear Antibody It is only a marker it is not pathogenicand when present in a specific disease do not correlate with clinical activity. There is no value in repeated testing of Antinuclear Antibodies Antinuclear Antibody Titer The higher the titer the more significant the clinical implications. Titers of 1:640 are more likely to be related to Collageno Vascular Diseases. 5

Antinuclear Antibody A negative ANA does not rule out the presence of a Collageno Vascular Disease Antibodies Associated with Rheumatic Diseases: Percentage of Patients Affected Antibodies to DS- DNA Sm antigen Histones SS-A SS-B Percentage of patients SLE: 50% -60% SLE: 30% Drug-induced SLE: 85% SLE: <60% Rheumatic arthritis: 20% Sjögren s syndrome: 70% SLE: 30% -40% Scleroderma and mixed connective tissue disease; Frequency and titers low Sjögren s syndrome: 60% Dermatomyositis: 10% 6

Antibodies Associated with Rheumatic Diseases: Percentage of Patients Affected (continued) Antibodies to RNP Scl-70 Nucleolar antigens Centromere antigens Anti CCP PM-1 JO-1 Percentage of patients Mixed connective tissue disease: 95% - 100% SLE: 30% at low titer Scleroderma: low frequency and titer Scleroderma: 10% - 20% Scleroderma: 40% - 50% CREST: 80% -90% Rheumatoid arthritis Polymyositis: 50% Dermatomyositis: 10% ACR Diagnostic Criteria in SLE Serositis-Pleurisy, Pericarditis on examination or Diagnostic ECG or imaging. Oral Ulcers-Oral or nasopharyngeal, usually painless; palate is more specific. Arthritis-Nonerosive, 2 or more peripheral joints with tenderness or swelling. Photosensitivity-Unusual skin reaction to light exposure. Blood Disorders Leukopenia( < 4000 on > 1 ocassion), Lymphopenia( 1500 on > 1 ocassion) Thrombocytopenia ( < 100000 in absence of offending medications), Hemolytic Anemia. 7

Neurological disorder Seizures or psychosis in the absence of other causes. Malar Rash Fixed erythema over the cheeks and nasal bridge, flat or raised. Discoid rash - Erythematous raised- rimmed lesions with keratotic scaling and follicular plugging, often scarring. Work up for a patient with a Positive ANA CBC ESR, CRP. LFT s Creatinine Urianalysis Lupus Panel or Analyzer C3 and C4 8

Positive Antinuclear Antibody It can take many years for a patient with a positive Antinuclear Antibody to develop the sign and symptoms of a CollagenoVascular Disease Case Presentation 13 yo/wf She has been healthy and presented to the office with complaint of generalized myalgiasand arthralgiaspresent for one month associated to fatigue,poorsleep and as per mom description she has been moody. She has an aunt who has Lupus. Physical Exam is negative except for pain with palpation of the supraclavicular muscles. She has laboratory test that show a Positive ANA 1:80 Rest of the laboratory test are WNL You order a Lupus Analyzer that is negative except for a Positive ANA. Management:? 9

Case Presentation 2 yo/hf She present with intermittent limping in the R knee for the last six weeks. Mom has noticed that the patient wake up in the morning and does not want to bear weight on that knee, after 15 min she is almost back to normal. She has not been as active as she used to be. She has not had any other symptoms. Laboratory test show a high ESR and a Positive ANA, Lupus Analyzer is negative except for the Positive ANA Physical Exam show a mild flexure contracture of the R knee, with increase temperature. What is your Diagnosis and what is your concern in relation to the Positive ANA? Case Presentation 16 yo/hf Patient who has been healthy but there is strong family history of Rheumatoid Arthritis. Mom is concern and she ask you to do some test to evaluate the possibility that the patient may develop a Collageno Vascular Disease. Her Physical Exam is normal. You order laboratory test and she is found to have a Positive ANA You order a Lupus Analyzer and she has a DS-DNA 1:40. Does she has Lupus? What will you do next? Does she need any treatment? What recommendations will you give her? 10

Case Presentation 10 yo/hf Patient has been healthy but for the last 6 months has been presenting color changes in the fingers that seem to be trigger by cold exposure, the fingers turn blue but they do not change color to red or white. She doe not have other medical problems and her Physical exam is normal. You order laboratory test including a Lupus Analyzer and she is found to have a Positive ANA, rest of the Lupus Analyzer is negative. What is the Diagnosis and what recommendations will you give her? Thank you! Rafael F. Rivas-Chacon, M.D. 11