Answering the most common rheumatology questions

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1 Answering the most common rheumatology questions Sterling West, MD, MACP, MACR Professor of Medicine University of Colorado SOM Disclosures I have no relevant financial relationships to disclose. Learning Objectives Describe the treatment and pitfalls in treating acute gout in a patient with chronic renal disease. Outline an approach to the evaluation and management of a patient with suspected vasculitis. Formulate and implement an approach to evaluate fever in a patient with a rheumatic disease. Discuss the perioperative management of a rheumatic disease patient who requires surgery.

2 Do I have to aspirate all patients with a known history of gout (? crystal- proven) who develop an acute inflammatory monoarticular arthritis? Margaretten ME. JAMA 297: , 2007 Carpenter CR. Acad Emerg Med 18: , 2011 Lim SY. Rheumatology 54: , 2015 You gotta ask yourself one question: Dirty Harry, 1971 Gout vs septic arthritis Cannot clinically separate gout from septic joint based on joint signs/sxs, fever, elevated WBC count, or elevated ESR/CRP. Gout likes to involve distal lower extremity joints (1 st MTP, midfoot, ankle, knee). Gout should not affect the back, shoulder, or hip. Septic arthritis likes large joints (knee, hip, shoulder). Pts with gout have 2-3x increased risk of developing septic arthritis. Gout and septic arthritis can coexist in the same joint. Patients with septic arthritis usually have a risk factor: increased age, other site of infection, HIV, RA, DM, joint surgery, immunosuppression.

3 Recommendations Aspirate an acute monoarticular arthritis in all patients with known crystal- proven gout except for those patients who have only the first MTP involved and no other significant risk factors to suspect a septic MTP joint. When in doubt, aspirate, because: Failure to aspirate, prepare to litigate. What is the best therapy for an acute crystalline arthritis in a patient with significant renal insufficiency? Khanna D. Arth Care Res 64: , 2012 Richette P. Ann Rheum Dis 2016, DOI: Possible treatments for acute crystalline arthritis in patients with normal renal function NSAIDs Indomethacin 50mg TID x 2 days then 25mg TID x 3-5 days Naproxen 500mg BID x 5-7 days Colchicine: 1.2mg followed by 0.6mg one hour later Corticosteroids Intraarticular Oral: 30mg qd x 5 days and D/C or 30mg qd x 2-5 days with taper over 7-10 days Parenteral Triamcinolone acetonide (kenalog) 60mg IM x 1 with option of second shot in 24 hrs if not improved > 20-50%. Can follow with oral prednisone 30mg qd with taper by 5mg qd until off. Methylprednisolone 30mg IV qd until resolved then D/C or taper. ACTH: IU subq

4 Treatment for acute crystalline arthritis in patients with renal insufficiency Corticosteroids: oral, IA, IM What if they also have a systemic infection? Corticosteroids: IA or IM What if they also have diabetes? Corticosteroids: IA Corticosteroids: IM and cover with insulin What if they are on anticoagulation, have diabetes, and are infected? Corticosteroids: IA up to an INR of 4.5 Corticosteroids: IM and cover with insulin Does this patient have some kind of vasculitis (SKV consult)? Sharma AM. Tech Vasc Interventional Rad 17: 226, 2014 Jennette JC. Arthritis Rheum 65: 1-11, 2013 Suresh E. Postgrad Med J 82: 483, 2006 Common vasculitis presentations Consider vasculitis in any patient who is constitutionally ill with multisystem inflammatory disease. Large vessel involvement (GCA, Takayasu s) Neuro: Headaches, visual loss, strokes Limb ischemia: claudication, bruit, asymmetric BP/pulse Medium vessel involvement (PAN) Neuro: mononeuritis multiplex, strokes (GACNS) Skin: livedo reticularis, ulcers, nodules, digital gangrene Renal/GI: HBP, microhematuria, abd pain Small vessel involvement (ANCA vasculitis, cryos, HSP, HUVS) Pulmonary- renal syndromes (ANCA Dz): DAH, RPGN EENT- pulmonary syndromes (ANCA Dz): scleritis, sinusitis, lung nodules, mass lesions (pseudotumor orbiti, subglottic stenosis) Skin: palpable purpura, urticaria lasting > 24hrs

5 Approach to vasculitis Is this a condition that can mimic a primary vasculitis or could there be a secondary cause of vasculitis? R/O infection, cancer, CTD, drugs/meds Suspect vasculitis mimic or a secondary cause in patients with: New heart murmur, unusually high fever, and/or splinter hemorrhages (SBE). Prominent liver dysfunction (hepatitis C with cryos) Drug abuse (HIV, hep B/C, cocaine, amphetamines, etc) Prior hx of cancer (esp lymphoma, myelodysplastic dz) Prior hx of CTD (SLE, Sjogren s, PAPS) Known offending medication (PTU, hydralazine, minocycline, etc) Smoking (Buerger s/thromboangiitis obliterans) Necrosis of LE digits (cholesterol emboli) Vasculitis mimics Large artery Common: fibromuscular dysplasia (FMD), IgG4 dz Uncommon: coarctationof aorta, neurofibromatosis type I, Marfan s, Loeys- Dietz, syphilic aortitis, infection (VZV), sarcoidosis, radiation fibrosis Medium artery Common: cholesterol emboli syndrome, atrial myxoma, FMD Uncommon: segmental arterial mediolysis, lymphomatoidgranulomatosis, angioblastic T- cell lymphoma, ergotism, thromboembolic dz, PXE, type IV EDS, Buerger s/to Cerebral arteries Common: reversible cerebral vasoconstriction syndrome (RCVS),posterior reversible encephalopathy syndrome (PRES), infections (VZV, others) Uncommon: cerebral amyloid angiopathy, CADASIL syndrome, Susac syndrome, progressive multifocal leukoencephalopathy, Moyamoya, intravascular lymphoma Small artery Common: SBE, cholesterol microemboli syndrome, mycotic aneurysm with emboli, antiphospholipid antibody syndrome, sepsis (Neisseria), SLE, Sjogren s, cryos (HepC), meds (PTU, hydralazine, minocycline, others), drugs (cocaine, amphetamine) Uncommon: Rickettsia, other systemic viral infection (parvo, herpes, EBV, CMV), CVID, calciphylaxis, livedoid vasculopathy (atrophie blanche), Degos disease, TTP, sickle cell Approach to vasculitis: workup Is there a secondary cause of the vasculitis and what is the extent of organ involvement? R/O infection, cancer, CTD, and drugs/medications Tests establishing systemic inflammation CBC: ACD, thrombocytosis, WBC count and differential to look for neutrophilia, eosinophilia, or abnormal cells Low WBC and/or low platelet count never seen in primary vasculitis (R/O SLE, antiphospholipid antibody syndrome, heme malignancy, TTP, medications) ESR and CRP: If both > than 10x ULN with no evidence of infection, acute crystalline arthritis, or cancer, then vasculitis is a likely diagnosis. Tests establishing extent of organ involvement Creatinine and urinalysis Liver- associated enzymes: if elevated consider hep B/C Creatine kinase Stool for occult blood CXR Others depending on Hx and P/E: EMG/NCV, sinus CT, echo, abd CT scan, brain MRI

6 Approach to vasculitis: workup Tests to rule out etiologies not responsive to immunosuppressive meds Blood cultures: R/O SBE PTT: if elevated consider antiphospholipid antibody syndrome LDH (if > 2-3x ULN): consider lymphoma, heme malignancy, myelodysplastic syndrome, TTP Infectious serologies: HepB (PAN), hepc (cryos), HIV, parvo IgM (PAN), CMV/EBV/herpes IgM Cryoglobulins: if positive rule out hepc and lymphoma SPEP: R/O myeloma Urine toxicology screen: cocaine, amphetamines Others depending on presentation: echo (SBE, atrial myxoma), CSF (VZV, herpes) Approach to vasculitis: workup Tests to rule out etiologies responsive to immunosuppressive medications. Tests suggesting immune complex deposition RF and ANA: should not be positive in primary systemic vasculitis. RF positive: R/O SBE, cryos, CTD (RA, Sjogren s) ANA positive: R/O SLE, Sjogren s Complement (C3/C4) levels: if low R/O SLE, cryos, HUVS Tests suggesting ANCA vasculitis canca: if against serine proteinase 3, usually GPA> MPA panca: if against myeloperoxidase, usually MPA> GPA panca not against myeloperoxidase is unlikely to be due to vasculitis Meds (PTU, hydralazine, minocycline) and cocaine can cause atypical ANCA, panca, or less commonly canca. Approach to vasculitis: workup Histologic or radiographic proof must be obtained in most patients with suspected vasculitis. Small vessel vasculitis(lcv, small artery) Idiopathic (50%) Infection (15-20%) Bacterial (strept, Neisseria, Rickettsia), HIV Hep C (cryos) CTD (15-20%) Primary vasculitis HSP (IgA vasculitis): Bx and immunofluorescence HUVS Cryoglobulinemia (essential) ANCA- vasculitis: usually MPA Secondary: SLE, Sjogren s, RA, Behcets, IBD Drugs (10-15%) Antibacterials, NSAIDs, cocaine, sulfasalazine PTU, hydralazine, GM- CSF, anti- TNF, allopurinol Thiazides, phenytoin, pantoprazole Malignancy (5%): heme, solid (lung, GI)

7 Approach to vasculitis: workup ANCA vasculitis Granulomatous polyangiitis (Wegener s) Clinical: ENT, lung, kidney canca against PR3 May be able to diagnose clinically without a biopsy Microscopic polyangiitis (MPA): consider pulmonary- renal syndromes Clinical: kidney, lung > skin Kidney bx: pauci- immune GN +/- crescents panca against MPO R/O drug induced: PTU, hydralazine Eosinophilic granulomatosis with polyangiitis (EGPA) (Churg Strauss) Clinical: asthma, lung, eosinophilia, rhinitis, neuropathy, skin, heart panca> MPO in only 50% Approach to vasculitis: workup If histology difficult to obtain in a patient with large or medium vessel vasculitis then need angiographic documentation. Medium vessel vasculitis (PAN) Histology Nerve bx positive in 45% (+ EMG/NCV) Skin nodule, testes, organ involvement Angiography (aneurysms) Mesenteric angiogram only if Abnormal exam: abd pain, HBP with hematuria Labs: LAEs, creatinine, U/A (blood) Abd CT scan: spleen or renal infarcts Approach to vasculitis: workup Large vessel vasculitis (GCA, Takayasu s) Histology GCA: temporal artery bx abnormal 85% with cranial/visual sxs, less than 50% if no cranial sxs. Takayasu s: hard to get histology Angiography (routine, CTA, MRA) Vessel wall thickening with enhancement Long tapering stenosis PET scan Contrast vessel wall enhancement and hyperemia

8 Is this patient s FUO caused by a rheumatic disease? This patient is known to have a rheumatic disease. Is the fever due to their rheumatic disease? Bleeker- Rovers CP. Medicine 86: 26, 2007 Toth E. Clin Rheumatol 31:1649, 2012 Fever and Rheumatic Diseases Rheumatic disease is found as the cause in 20% of patients with an FUO. Most common rheumatic diseases presenting with an FUO: Adult- onset Still s disease (AOSD) Large vessel vasculitis (GCA, Takayasu s) Rheumatic diseases that can cause sustained daily fever Monoarthritis and fever: acute gout (9%), acute pseudogout (up to 50% can have low grade fever), infectious arthritis (50-90% have fever), osteomyelitis Polyarthritis and fever: RA (< 5% with acute onset), viral arthritis (parvo B19, others), SAPHO, Whipple s disease, rheumatic fever Systemic inflammatory disease and fever (occurs at disease onset or with flares):sle (80-90%), systemic vasculitis [ GCA (33%), Takayasu s (20% early), PAN (60%), EGPA (Churg- Strauss) (60%), GPA (Wegener s) (25%)], inflammatory myositis (antisynthetase antibody syndrome, e.g anti- Jo- 1) Fever and Rheumatic Diseases Rheumatic diseases causing recurrent periodic fevers AOSD Behcet s disease Relapsing polychondritis Sarcoidosis Crystal- induced diseases Autoinflammatory syndromes: familial periodic fever syndromes

9 Clinical pearls on fever and rheumatic diseases Always rule out infection, heme malignancies, and medications first as the cause of the fever. LDH > 2-3x ULN: consider heme malignancy or heme Dz Procalcitonin elevated (>1ng/ml): consider infection (75% spec and sensitivity) (Shaikh M. Rheumatology 54:231, 2015) AOSD Major criteria: recurrent fevers (>39 C) > 7 days, nonpruritic evanescent rash on trunk, polyarthritis/arthralgias, leukocytosis (> 10,000/uL with 80% neutrophils). Plot out fever curve: quotidian or double- quotidian Minor crtieria: hepatosplenomegaly, sore throat, lymphadenopathy, abnormal LAEs Supportive: Ferritin > 1000 ng/ml (70%) Note that procalcitonin can be > 1ng/ml Clinical pearls on fever and rheumatic diseases SLE Fever is common at disease onset and with flares. Ask patient if fever is usually a part of their flare. Fever more likely due to SLE if part of their usual flare, serositis, WBC low, C3/C4 low, elevated anti- dsdna Fever more likely due to infection if atypical for their flares, WBC higher than normal, serologically inactive or unchanged, CRP> 6x ULN, elevated procalcitonin. Macrophage activation syndrome (MAS) Life- threatening (mortality 20%) hemphagocytic syndrome seen in AOSD and SLE patients. Can be triggered by EBV, CMV, or parvovirus infection. High fever and hepatosplenomegaly. No rash or arthritis. Labs: cytopenias ( 2 of 3 cell lines), elevated LAEs, triglyceride level (> 180 mg/dl), PT/PTT, low fibrinogen, low ESR Supportive: ferritin > 10,000, elevated soluble IL- 2 receptor (CD25) Dx: bone marrow or liver bx showing hemophagocytosis by macrophages Your patient with a rheumatic disease is going to surgery. What additional preoperative evaluation or precautions need to be taken? Bissar L. Rheumatology J 7: 42, 2013 Goodman SM. Rheum Dis Clin North Am 38: 747, 2012

10 Perioperative management of patient with rheumatic disease Hx, PE, labs, EKG, CXR per established guidelines. Rheumatic disease pts have their risk of atherosclerosis accelerated by 10 years. Cardiac risk stratification Assessment of specific clinical problems Rheumatoid arthritis: cervical spine xray, assess lung function if rales or sxs (CXR, PFTs) Ankylosing spondylitis: cervical spine xray if clinically involved SLE: pts with lupus anticoagulant and/or high titer anticardiolipin/antib2gpi antibodies need compression stockings and prophylactic anticoagulation perioperatively and continued for seven days after surgery even if no prior hx of clot. Perioperative management of patient with rheumatic disease Adjust medications NSAIDs: stop five half lives before surgery Prednisone: stress dose protocol (solucortef 100mg IV on call to operating room, 100mg during surgery, 100mg 8hrs postop). Decrease by half (IV or oral equivalent) each day until on usual dose. Methotrexate: controversial. Stop week of and week after major surgery. Mycophenolate mofetil: stop 3 days prior to surgery and start 3 days after surgery Other DMARDs (hydroxychloroquine, sulfasalazine, azathioprine): stop 1 day preop and start 3 days postop Biologics: stop two half lives prior to surgery and resume once wound healing is satisfactory. Anti- rheumatic disease drugs can mask infections: prednisone, tocilizumab, and anti- TNF agents are biggest offenders. Summary Failure to aspirate, prepare to litigate. Corticosteroids are best for gout flare if have renal insufficiency. Strategies for how to evaluate a patient with suspected vasculitis. Need biopsy or angiographic confirmation of vasculitis. FUO: AOSD, GCA, Takayasu s most common Rule out infection, heme malignancy, drugs, and MAS as cause for fever before ascribing it to the underlying rheumatic disease. When a rheumatic disease is the cause of a fever there should be other clinical and laboratory evidence that the rheumatic disease is active. Perioperative management considerations for rheumatic disease patients. CAD risk stratification. Clot more common.

11 Thank You University of Colorado School of Medicine Anschutz Medical Center Aurora, Colorado