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Cases in Rheumatology Learning Objectives • Review the laboratory testing in the work up of systemic Cases in lupus erythematosus and rheumatoid arthritis Rheumatology • Discuss the recent evidence for the management of autoimmune diseases such as systemic lupus Sandeep K. Agarwal, MD, PhD erythematosus and rheumatoid arthritis Chief Section of Immunology Allergy and Rheumatology Director of Biology of Inflammation Center Case 1: 36-year-old woman with chief Case 1 (slide 2) complaint of knee pain • Pain in front of both knees when she runs • Would you order blood tests? and squats, feels swollen • If so, what tests? • No other joint pain, no stiffness in the morning. Acetaminophen helps • More specifically, would you order the “rubber stamp • ROS negative for fevers & chills, rash, oral rheumatology workup” sores, nasal congestion, chest pain, • ESR, CRP, RF, CCP, ANA, anti-dsDNA, anti-Smith, anti-RNP, anti- shortness of breath, abd pain, n/v/d, muscle weakness, paresthesias Ro, anti-La, anti-histone, anti-scleroderma 70, anti-centromere, anti-Jo1, anti-cardiolipin, lupus anticoagulant, anti-beta2 • MSK exam small joints of hands, wrists glycoprotein, anti-smooth muscle antibody, anti-mitochondrial Ab, elbows nl, shoulders good ROM, hips full Lisa, 36, ROM, knees with patellar clicking, no anti-proteinase-3, anti-myeloperoxidase, and for good measure an effusions, vastus medialis atrophy, ankles Knee pain ACE level! and MTPs nl Case 1 (slide 3) Case 2: 36-year-old woman with joint pain • Dx: patellofemoral pain • Started a few months ago, with pain in fingers (PIPs), wrists and knees. • No evidence by history or examination of an inflammatory • Subjective swelling. Pain worse in the arthritis morning, feels stiff for about an hour but will loosen up with moving around • No autoimmune serologies or inflammatory markers are • Ibuprofen helps indicated. • ROS negatives: fevers, chills, weight change, oral sores, nasal sores, shortness • Can consider CBC and CMP if you are going to prescribe of breath, abdominal pain/n/v/d, muscle Flor, 36, NSAIDs weakness, skin rash • ROS positives: hair has been thinning the Joint pain past few weeks, fatigue. Last summer had chest pain that was worse with breathing that resolved with NSAIDs Case 2 (slide 2) Case 2 (slide 3) • MSK Examination: • Would you order blood tests? • DIP nontender, no synovitis • PIP tender and + soft tissue fullness bilaterally • If so, what tests? • MCP nontender, no synovitis • More specifically, would you order – ESR, CRP, RF, CCP, ANA, anti-dsDNA, anti-Smith, anti-RNP, anti-Ro, anti-La, • Wrists trace effusion, no warmth or redness, decreased dorsiflexion with slight tenderness anti-histone, anti-scleroderma 70, anti-centromere, anti-Jo1, anti-cardiolipin, lupus anticoagulant, anti-beta2 glycoprotein, • Elbows, shoulders, hips with good ROM, nontender anti-proteinase-3, anti-myeloperoxidase • Knees with small warm effusions bilaterally no crepitus • Ankles and MTPs negative Case 2 (slide 4) ABIM Choosing Wisely – • Dx: Inflammatory polyarthritis Rheumatology • Differential diagnosis: rheumatoid arthritis, psoriatic arthritis, systemic lupus erythematosus, hypothyroidism, …… Don’t test ANA sub-serologies • Yes, you should order lab work without a positive ANA and clinical • Start with CBC, CMP, urinalysis, TSH, ANA, RF, CCP suspicion of immune-medicated • Defer additional testing for preliminary tests results diseases • Consider x-rays of affected joints • Refer rheumatology Arch Pathol Lab Med. 2000: 124(1): 71-81; Arthritis and Rheum 2002: 47(4): 434-44. Am J Clin Path 2002: 117(2): 316-24 Antinuclear Antibodies (ANA) Antinuclear Antibodies • “False positives” : • Autoantibodies directed against a variety of • 1:40 : 32% of healthy controls nuclear antigens • 1:80 : 13% of healthy controls • Procainamide, • SLE : >95% ( some studies say 99.9%) • >1:320 : 3% of healthy controls hydralazine, minocycline, • ANAs also seen : diltiazem, penicillamine, • Scleroderma ~90% • Hyper/Hypothyroid, autoimmune hepatitis, primary INH, quinidine, • MCTD >95% biliary cirrhosis, idiopathic pulmonary arterial methyldopa, hypertension, hepatitis C, endocarditis, HIV, TB, chlorpromazine • Polymyositis/dermatomyositis ~60-75% mononucleosis • Anti-TNF agents • Medications (see list on the right) • Possible – phenytoin, • Rheumatoid arthritis ~40% rifampin, lithium, IFN, • False negatives: less common others…….. • Sjögren's syndrome ~50% • Anti-Ro antibodies can still be present with a negative ANA test (SLE, Sjogrens) • Discoid lupus ~15% UpToDate.com • Anti-Jo-1 (myositis) Antinuclear Antibodies: Assays Case 2 (slide 5) • Gold standard method for detection is immunofluorescence using • WBC 4.5, Hct 31, Plt 95 HEp2 cells as the substrate • CMP nl • Cons: Visual test, inter-observer variability • Urinalysis: no WBC, no RBC, no protein, no • HEp2000 cells can improve sensitivity with transfection of Ro60 antigen glucose • Solid phase assays replacing IFA (Less specific and less • ANA 1:640 sensitive) • RF, CCP neg Pros: Can handle high demand, less expensive, less technical • Additional antibodies are ordered given expertise, more automated positive ANA: Cons: • Anti Smith positive Flor, 36, • No standardization of antigen selection • Negative: anti dsDNA, RNP, Ro, La, • Limited number of antigens Joint pain cardiolipin • Biochemical nature of the antigen (native antigen, linear antigen) • Fixation & coating, calibrators, etc. • C3 85, C4 12 UpToDate.com ANA: Sub-Serologies Case 2 (slide 6) Antigen Clinical Associations in SLE Prevalence (%) Double stranded DNA Renal disease, marker for disease activity 40-60% • A diagnosis of systemic Smith Antigen (Sm) 20% lupus erythematosus is Subacute cutaneous lupus, photosenitivity, Ro/SSA 40% neonatal lupus, Sjogrens made. Low prevalence of renal disease, Sjogrens La/SSB 10-15% Hydroxychloroquine syndrome Ribonuclear protein (U1- 200 mg twice a day Mixed connective tissue disease 30-40% • Flor is told to get annual RNP) eye exams to screen for Hypercoagulable states, thrombocytopenia, Phospholipids 30% miscarriages, verrucous endocarditis macular toxicity Histones Drug related SLE (not specific) Ribosomal P Psychosis and depression 10-40% Systemic Lupus Erythematosus (SLE) SLE: Clinical manifestations • Constitutional Symptoms • RENAL • Chronic, multisystem autoimmune disease • Fatigue, myalgia, weight loss, fever • GLOMERULONEPHRITIS • Can affect almost every system • Mucocutaneous • Pulmonary • Oral or nasal ulcers (usually painless), • Serositis, pleurisy, pleural effusions, acute • Most common is skin, MSK, mild hematologic, serologic rashes, alopecia (scarring or pneumonitis nonscarring) • Prevalence 20-240 per 100,000 persons • Interstitial lung disease, diffuse alveolar • Musculoskeletal hemorrhage • Incidence 1-10 per 100,000 per-years • Arthralgias, inflammatory arthritis, • Cardiovascular myositis, avascular necrosis • Typically presents ages 15-64 • Pericarditis, myocarditis, verrucous endocarditis, • Raynaud’s phenomenon (15-30%) coronary artery disease!!!!! • Females>males • Hematologic • Neurologic • African American, Hispanic and Asian populations at risk of • Leukopenia, thrombocytopenia, • Neuropsychiatric lupus, cognitive impairment anemia (AOCD, AIH) more severe disease • Ophthalmologic, Gastrointestinal, etc Case 2 (slide 7) SLE: lab monitoring • Blood pressure monitoring • 6 months later, she returns to clinic • CBC screening for cytopenias with 50% improvement in joint pain, • CMP screening for renal impairment and low albumin stiffness and swelling, mild pleurisy, • Urinalysis screening for microscopic hematuria and proteinuria no rash. • Urine protein:creatinine ratio • Complement C3 and C4 • Methotrexate is started but she • A decrease can indicate lupus activity does not tolerate it. She returns • Anti-dsDNA antibodies with the same symptoms two weeks Flor, 36, • An increase titer can indicate lupus activity later. Joint pain • No indication to repeat ANA, anti RNP, anti Ro, anti La – do not change with lupus activity • What labs are needed? Hahn et al. ACR 2012 Lupus Nephritis Guidelines. Arth Care Res. 2012, Vol 64(6): 797-808 SLE: Indications for Renal Biopsy Case 2 (slide 8) • All patients with clinical Increasing serum Confirmed Combinations of the evidence of previously creatinine without proteinuria of ≥1.0 following, assuming Labs untreated active Lupus compelling gm per 24 hours the findings are glomerulonephritis alternative (either 24-h urine confirmed in ≥2 • WBC 4.5, Hct 32, Plt 110 causes specimens or spot tests done within a should get a biopsy (e.g., sepsis, protein/creatinine short period of time • Cr 1.0 (unless STRONGLY hypovolemia, or ratios acceptable) in the absence of contraindicated) medication) LOE: C alternative causes. • Urine : no RBC, no protein LOE: C (A)Proteinuria ≥0.5 • Activity vs. chronicity gm per 24h + • C3 80, C4 12 Flor, 36, hematuria, • Rule out other causes defined as • dsDNA titer negative Joint pain (ATN, hypocalcemia, ≥5RBCs per hpf. hypotension, (B)Proteinuria ≥0.5 thrombotic) gm per 24h + cellular cases • What are the treatment options in a patient with active SLE (arthritis, LOE: C pleurisy, hypocomplementemia) despite hydroxychloroquine? Hahn et al. ACR 2012 Lupus Nephritis Guidelines. Arth Care Res. 2012, Vol 64(6): 797-808 Belimumab (Benlysta) Belimumab in SLE (BLISS-52 trial) • Fully human immunoglobulin (IgG1), monoclonal antibody • Phase 3, RCT, 48 week trial • Primary endpoint- SLE Responder against soluble human B-lymphocyte stimulator (BLyS) • Patients with active SLE, no Index
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