46 yr old woman with asthma and rightsided paresthesias

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46 yr old woman with asthma and right-sided
paresthesias

• Anh Innes

2
History

• 66 yo Asian woman with long-standing history of
  asthma presents with right-sided weakness and
  paresthesias.
• 5 d PTA pt noted sx in RLE, then in RUE
• weakness, decreased sensation, and pain
• 1 previous episode of b/l LE paresthesias 1 year
  ago
• ROS mild wheezes, SOB (no change from
  baseline) weight loss (10 lbs/3-4 mos)

3
History, cont.

• PMH
• Asthma
• Allergic rhinitis
• DM type 2
• Hx Graves disease
• Breast mass s/p FNA
• PPD (Ø tx)
• ? cholesterol

• Meds
• Flovent 220 mcg
• Serevent
• Singulair 10 mg qd
• Prednisone 10 mg qd
• Nasocort
• Albuterol MDI prn
• Glyburide
• Pravastatin
• alendronate

4
History, cont.

• Social
• China to SF 1979
• Lives alone
• Denies tob/Etoh/IDU
• Ø pets/birds/mold/
• mildew

5
Exam

• 37.6 130/65 91 18 96 RA
• Elderly Asian woman, supine in bed, NAD
• No thyromegaly
• Diffuse, mild expiratory wheezes without rhonchi
  or crackles
• Neuro exam
• Motor 3/5 RUE and RLE distal 4/5 RUE and RLE
  proximal R foot drop
• Sensory decreased sensation RLE distal lateral
  distribution and dorsum of foot

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Data, cont.

• 13.1
• 20.1 193
• 38.6
• 33 segs/9 lymphs/2 monos/55 eos
• Chem panel WNL
• ESR 62
• UA WNL

• EMG mononeuritis multiplex
• Median n., peroneal n., bilateral tibial n.

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Churg-Strauss Syndrome

• Allergic granulomatosis and angiitis
• Allergic rhinitis
• Asthma
• Eosinophilia
• Incidence 3 cases/million general population
• 10 pts with major vasculitis CSS
• Frequency 2nd to Wegeners
• Etiology unknown likely autoimmune

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Churg-Strauss Syndrome

• Clinical Features
• 3 phases
• Prodrome atopy, asthma, allergic rhinitis
• Eosinophilic phase
• Vasculitis
• Asthma gt95
• Skin involvement 66
• Neurologic disease 75
• Cardiovascular 50 of deaths

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Churg-Strauss Syndrome

• Diagnosis
• ACR criteria 4 of 6 85 sensitive, 99.7
  specific
• Asthma
• Eosinophilia gt10
• Mononeuropathy or polyneuropathy
• Migratory/transient pulmonary opacities
• Paranasal sinus abnormality
• Biopsy with vesseleos in extravascular areas

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Churg-Strauss Syndrome

• Treatment
• Corticosteroid therapy x 6-12 weeks
• Follow eosinophils and ESR
• pANCA positivity persistence unhelpful
• Cyclophosphamide, imuran, IVIG
• Prognosis unclear
• Most deaths in vasculitic phase
• Cardiac and GI involvement poor prognosis

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Leukotriene Receptor Antagonists and CSS
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Churg-Strauss Syndrome

• Multi-system disorder characterized by asthma,
  allergic rhinitis, eosinophilia
• Diagnosis difficult ACR criteria to be used in
  conjunction with clinical judgment
• Etiology unknown
• Role of LTA not defined presently
• Allow oral corticosteroid taper
• Elicit or promote development of CSS
• Treatment with corticosteroids
  immunosuppressive agents

15
references

• Green RL, Vayonis AG. CSS after zafirlukast in
  two patients not receiving systemic steroids.
  Lancet 1999353725-726.
• Jamaleddine et al. LTA and the Churg-Strauss
  Syndrome. Semin Arthritis Rheum 31218-227.
• Le Gall C., et al. Inhaled Corticosteroids and
  Churg Strauss Syndrome a report of five cases.
  Eur Respir J 200015978-981.
• Pedersen K.E., Bochner BS, Undem BJ. Journal of
  Pharmacology and Experimental Therapeutics.
  1997281655-662.

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references

• Rothenberg M.D. Eosinophilia. NEJM
  19983381592-1600.
• Tuggey JM, Hosker HSR. Churg Strauss Syndrome
  associated with montelukast therapy. Thorax
  200055805-806.
• Weller PF, Plaut M, Taggart V et al. The
  relationship of asthma therapy and Churg Strauss
  syndrome NIH workshop summary report. J
  Allergy clin Immuno 2001108175-83.
• Weschler ME, garpestad E, Flier SR et al.
  Pulmonary infiltrates, eosinophilia, and
  cardiomyopathy following corticosteroid
  withdrawal in patients with asthma receiving
  zafirlukast. JAMA 1998279455-457.