Inflammatory Bowel Disease – 7/31/18

Thanks to Connie for presenting a case of a young man with chronic bloody diarrhea, abdominal pain, and fever, found to have a new diagnosis of severe Ulcerative Colitis.


Clinical Pearls

  • Acute diarrhea requires work up in anyone >65, immunocompromised, blood in stools, fever, severe abdominal pain, recent antibiotics, known or suspected IBD, risky jobs like food handler, or recent travel.
  • Fecal calprotectin can help distinguish inflammatory from non-inflammatory diarrhea and is a more sensitive and specific marker than fecal leukocytes.
  • 5-ASA based drugs are generally more effective in the colon so their primary role is in the treatment of Ulcerative Colitis or Crohn’s Colitis.

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Disease severity in IBD:

  • Mild: <4 stools/day, no systemic toxicity
  • Moderate: 4-6 stools/day, no systemic toxicity
  • Severe: >6 stools per day, systemic toxicity
  • Fulminant: >10 BMs per day, continuous bleed, systemic toxicity

Key distinctions between UC and CD:

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Items in red in the table above correlate with disease activity.

Before initiating immunosuppression:

  • Check PPD/quantiferon
  • Hepatitis serologies
  • Administer routine live vaccines
  • Check TPMT level (to assess phenotype for bone marrow suppression secondary to 6MP).  If TPMT level low, do not give 6MP!

Lupus nephritis – 7/26/18

Thanks to Naina for presenting an interesting case of a young woman presenting with fever, nausea/vomiting, and R flank pain found to have pyelonephritis and lupus nephritis!


Clinical Pearls

  • Renal involvement is noted in ~50% of patients with SLE and can present as nephrotic or nephritic syndromes.
  • The most common and severe form is diffuse proliferative lupus nephritis (class IV)
  • It is important to distinguish SLE flare from an infection. When infection is present, it must be treated first before starting immunosuppression.
    • SLE flare is associated with a normal WBC, low CRP (because CRP production by hepatocytes is down-regulated by type 1 IFN release in lupus flare), and absence of fever
  • Lab findings suggestive of flare include elevated anti-dsDNA (correlates with disease activity), low complement levels (predominantly C3 decline), worsening proteinuria, and elevated creatinine.

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* Membranoproliferative GN can present with mixed nephrotic/nephritic picture.

SLE and renal disease:

  • Renal involvement is common and eventually occurs in ~50% of SLE patients
  • 10% progress to ESRD
  • High mortality compared to SLE w/o nephritis
  • More common and severe in African Americans, Hispanics, Asians and can be the only manifestation of lupus on presentation!
  • Classifications of GN:
    • Can evolve from one to another
    • Minimal mesangial lupus nephritis (class I)
      • Earliest and mildest form
      • Rarely diagnosed b/c pts have a normal U/A, no or minimal proteinuria, and normal Cr
    • Mesangial proliferative lupus nephritis (class II)
      • Microscopic hematuria and/or proteinuria
      • Light microscopy would show mesangial hypercellularity or mesangial matrix expansion
    • Focal lupus nephritis (class III)
      • Hematuria, proteinuria, some HTN, decreased GFR
      • Less than 50% glomeruli affected by light microscopy
      • Segmental glomerulonephritis
    • Diffuse lupus nephritis (class IV)
      • Most common and most severe
      • Hematuria, proteinuria, nephrotic syndrome, HTN, reduced GFR
      • Hypocomplementemia (esp C3) and elevated anti-dsDNA during active disease
      • >50% of glomeruli are affected
    • Lupus membranous nephropathy (class V)
      • nephrotic syndrome, Cr normal or slightly elevated
      • Diffuse thickening of the glomerular capillary wall and subepithelial deposits
      • Can present without any other clinical or serologic manifestations of SLE
    • Advanced sclerosing lupus nephritis (class VI)
      • Slow, progressive renal dysfunction with proteinuria and relatively bland urine sediment
      • Global sclerosis >90% of glomeruli
      • Active GN no longer observed
  • Treatment:
    • Best to initiate early but AFTER treatment of active infection:

      • Cyclophosphamide or Mycophenolate PLUS solumedrol 250-1 g/day x 3 days (former takes 10-14 days to have an effect so the latter is much faster) or prednisone 60 mg/day
      • Mycophenolate is the preferred choice to preserve fertility in women of reproductive age
    • Goals of therapy:
      • substantial reduction in urine protein excretion  to <0.33 g/day
      • improvement or stabilization of serum creatinine
      • improvement of urinary sediment

Syphilis – 7/23/18

Carriann presented the case of a young woman with HIV (CD4 250 off ARVs) and prior syphilis s/p treatment five years ago who presented with constitutional symptoms and diffuse rash involving the palms and soles, found to have RPR 1:256 consistent with secondary syphilis!


Clinical Pearls 

  • Lues maligna or malignant syphilis is a rare manifestation of secondary syphilis in immunocompromised individuals and presents as an ulceronodular rash.
  • Syphilitic hepatitis is seen in ~10% of patients with secondary syphilis and presents as predominantly elevated alkaline phosphatase with normal or mildly elevated transaminases.
  • Neurosyphilis can occur at any point after infection with syphilis!
  • Treatment success is defined as a four-fold drop in nontreponemal titers (ie RPR).

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Diagnosis:

Keep in mind the following principles:

  • Treponemal tests remain positive long-term
  • Non-treponemal tests can become negative after treatment.  They are useful for treatment monitoring because they can be quantitative
  • Both tests can be falsely negative early in disease course so repeat tests if clinical suspicion remains
  • Screening algorithm

Treatment monitoring:

  • Jarisch-Herxheimer reaction is a self-limited condition that can occur in ~10-35% of patients within 24 hours of treatment with antibiotics.
  • A four-fold decline in titers (2 dilutions) is considered treatment success.
  • Monitor titers q 6-12 months post treatment.  Increasing titer is concerning for treatment failure, neurosyphilis, or reinfection!

New A fib, severe MR, and papillary muscle rupture… 7/17/18

Thank you Naina for presenting the case of an elderly man with 20 packyear smoking history presenting with acute onset of dyspnea and scant hemoptysis, found to have new onset A fib and L heart failure secondary to severe mitral regurgitation resulting from papillary muscle rupture!


Clinical Pearls

  • In patients with severe mitral regurgitation (MR) and a normal L atrium size, think about acute causes of MR.  TEE is often indicated to better visualize the valve structure and determine need for operative intervention.
  • MR can be caused by papillary muscle rupture, especially 3-7 days post MI.  Other etiologies of rupture include endocarditis and myxomatous valve degeneration.
  • Patients with rupture present with acute onset hypotension, pulmonary edema, and a hyperactive precordium. A systolic murmur is not always present!
  • Treatment:
    • Aggressive afterload reduction AND
    • Surgery (high mortality rate 20-25%)

Atrial Fribrillation

Categories:

  • Paroxysmal (terminates within 7 days)
  • Persistent (>7 days)
  • Long-standing persistent (>1 year)

Differential for new onset A fib: (PIRATES!)

  • Pulmonary (OSA, PE, COPD, PNA)
  • Ischemia/infarction/CAD*
  • Rheumatic heart disease/mitral regurgitation
  • Alcohol/anemia (high output failure
  • Thyrotoxicosis/toxins (stimulants)
  • Electrolytes/endocarditis
  • Sepsis/sick sinus syndrome
  • Other: HTN*, congenital heart disease, previous cardiac surgery, viral infections

* Most common causes in the US.

Treatment:

  • Rate control (preferred method based on AFFIRM and RACE trials)
    • Beta blockers
    • Calcium channel blockers ⇒ contraindicated in decompensated heart failure
    • Digoxin ⇒ avoid use in renal failure, hypokalemia, hypomagnesemia, or hypercalcemia
    • Amiodarone
  • Rhythm control
    • Methods:
      • Chemical (~30% success rate)
        • Class III (amiodarone, sotalol, ibutilide)
      • Electrical (synchronized to QRS, ~80% success rate)
    • Preferred modality in
      • Hemodynamically unstable
      • Young patient (age <65) or good functional status
      • Early in natural history of disease
      • Failure of rate control agents
      • Heart failure

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Complications post MI:

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Figure from article by Reed et al. Lancet. 2017.

Papillary muscle rupture:

  • Posteromedial muscle is 6-12x more likely because blood supply is through PDA only. Anterolateral muscle receives dual supply from LAD and LCx.
  • Clinical presentation
    • Acute onset hypotension, pulmonary edema
    • Hyperactive precordium
    • Mid, late, or holosystolic murmur with widespread radiation (though many have no murmur!)
    • Diagnosis requires TTE/TEE
    • Treatment:
      • Aggressive afterload reduction
      • Urgent/emergent surgical intervention (20-25% mortality)