Issues in Rheumatology

  • Cannot clinically separate the two based on joint sxs, fever, elevated WBC, or elevated CRP/ESR.
  • The two can co-exist!
  • Recommendation:  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 risk factors for a septic MTP joint.
  • Failure to aspirate, prepare to litigate.
  • If normal renal function:  NSAIDs (indomethacin, naproxen); colchicine 1.2 mg followed by 0.6 mg 1 hour later (works as well as the old “take until diarrhea” method); corticosteroids (IA, PO – 30mg QD x 5d or 30mg x 1-2 d then taper over 7-10 d) or parenteral (Kenalog 60mg IM x 1, may repeat in 24h if less than 20-50% improvement); ACTH 25-40 IU SQ.
  • In those with renal insufficiency:
    • Corticosteroids:  IA, PO, IM
    • What if they have a systemic infx?  IA or IM
    • What if they have diabetes?  IA or IM + increase insulin
    • What if they’re anticoagulated?  IA up to INR of 4.5 or IM + increase insulin
  • Suggested by constitutionally ill patient with multisystem inflammatory disease.
  • Large vessels = GCA, Takayasu’s – neuro (HA, visual loss, stroke); limb ischemia (claudication, bruit, asymmetric BP/pulse).
  • Medium vessels = polyarteritis nodosa (PAN) – neuro (mononeuritis multiplex, strokes (GACNS)); skin (livedo reticularis, ulcers, nodules, digital gangrene); renal (HTN, microhematuria, abd pain).
  • Small vessels = ANCA vasculitis vs non-ANCA 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 > 24 h
  • Approach to vasculitis:
    • Is this a condition that can mimic a vasculitis?  Infection, cancer, connective tissue disease, drugs/meds.
    • Suspect a vasculitis mimic or secondary cause if:
      • New heart murmur, unusually high fever, or splinter hemorrhages (SBE)
      • Prominent liver dysfunction (hep C with cryos)
      • Drug abuse (HIV, hep B/C, cocaine, amphetamines, etc.)
      • Prior hx of cancer (lymphoma, myelodysplastic dz)
      • Prior hx of CTD (SLE, Sjogren’s, PAPS)
      • Meds (PTU, hydralazine, many others)
    • Workup:
      • Tests establishing systemic inflammation:  CBC – low WBC and/or low plt counts never seen in PRIMARY vasculidities!  Look for thrombocytosis, leukocytosis or abnormal cells.
      • ESR and CRP – if both > 10x ULN with no evidence of infection, acute crystalline arthritis, or cancer…then vasculitis is a likely diagnosis.
      • Tests to rule out etiologies not responsive to immunosuppressive meds:  Blood cultures (SBE), PTT (APLA), LDH (if > 2-3x ULN consider lymphoma, heme malignancy, MDS), infectious serologies (hep B, hep C, HIV, parvo IgM…)
      • Tests to rule out etiologies responsive to immunosuppressive meds:  RF and ANA – should NOT be positive in primary vasculidities; complement levels – if low, r/o SLE, cryos, HUVS.  Tests suggesting ANCA vasculitis:  cANCA (GPA>MPA), pANCA (MPA>GPA).  pANCA not against myeloperoxidase unlikely to be a vasculitis.
      • Histologic or radiologic proof is needed before treatment is initiated in patients with vasculitis
        • Small vessel vasculitis – idiopathic 50%, infection 15-20%, CTD 15-20%, drugs 10-15%, malignancy 5-10%
        • Only ANCA vasculitis can be diagnosed without a biopsy – clinical involvement of ENT, lung, kidney with cANCA against PR3.
      • What to biopsy?
        • Nerve biopsies are positive in 45% of medium vessel vasculitis (PAN); also could biopsy skin nodule, testes or involved organs.
        • Large vessel:  temporal artery biopsy abnormal in 85% of those with cranial/visual sxs, less than 50% if no cranial symptoms.  Takayasu’s – hard to get histology.
      • Angiography?  Mesenteric angiogram only if abnormal exam (abd pain, HTN with hematuria), labs abnormal (Cr, UA), spleen or renal infarcts on CT.
      • Imaging for GCA – PET scan can be helpful.
  • 20% of FUO patients have a rheumatic etiology.
  • Most common:  Adult-onset Still’s disease, large-vessel vasculitis (GCA, Takayasu’s).
  • Rheumatic diseases that can cause sustained fever:
    • Monoarthritis and fever:  gout, pseudogout…and many others.
  • Rheumatic diseases and periodic fevers:
    • Consider infx, heme malignancies, meds first.
    • AOSD:  recurrent fevers, non-pruritic evanescent rash on trunk, polyarthritis/arthralgias, leukocytosis (>10K with 80% PMNs); minor criteria – HSM, ST, LAD
    • Supportive dx criterion:  ferritin > 1000 or high procalcitonin.
  • Lupus and fever:
    • Fever is common at disease onset and with flares.  More likely due to SLE if part of their usual flares, serositis, WBC low, C3/C4 low, elevated ds-DNA.
    • 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:  life-threatening complication in AOSD and SLE patients.  Can be triggered by EBV, CMV or parvovirus.  Sxs:  high fever and HSM, no rash or arthritis.  Labs show pan-cytopenias, elevated LFTs, high triglycerides, high PT/PTT, low fibrinogen, low ESR.  Definitive dx:  bone marrow bx shows hemophagocytosis by macrophages.
  • Do your usual CV risk assessment – but remember, pts with rheum dz have CAD risk ACCELERATED BY 10 YEARS!
  • RA:  consider C-spine XR, assess lung function (CXR, PFTs) if rales or pulm sxs
  • Ank Spond:  C-spine XR if clinically involved
  • SLE – if (+) lupus anticoagulant or high titer ACLA/anti-B2GP1 antibodies – need compression stockings and prophylactic anticoagulation perioperatively and for seven days after surgery EVEN IF NO PRIOR DVT HX.
  • Medication management
    • NSAIDs:  stop 5 half-lives before surgery
    • Prednisone:  stress dose protocol – solucortef 100mg IN on call to OR, 100mg during surgery, 100mg 8 h post-op.  Decrease by half IV or PO equivalent each day until on usual dose.
    • MTX:  controversial.  Stop week of surg and week after.
    • DMARDs:  Stop 1 day preop and start 3 days post-op.
    • Biologics:  Stop 2 half-lives prior to surgery.  [missed when to resume]

From Rocky Mountain Hospital Medicine conference, 2016, West


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