Jason Kidd Morning Report 11/18/2009 Wegener: Controversy “Unlike doctors who joined the Nazi Party to be allowed to practice, Wegener joined the movement.
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Wegener’s Granulomatosis
Jason KiddMorning Report
11/18/2009
Wegener: Controversy
“Unlike doctors who joined the Nazi Party to be allowed to practice, Wegener joined the movement in 1932, before Hitler took power. He rose to a relatively high military rank and spent some of the war in a medical office three blocks from the Jewish ghetto in Lodz, Poland. Sketchy records suggest that he might have participated in experiments on concentration camp inmates.”
Feder, NY Times 1/22/2008
Definition“A distinct clinicopathologic entity
characterized by granulomatous vasculitis of the upper and lower respiratory tracts together with glomerulonephritis.”
Harrison’s
Incidence and PrevalenceEstimated prevalence: 3 per 100,0001:1 male to female ratioMean onset: 40
Harrison’s
Diagnostic Criteria: American College of Rheumatology proposed
clinical criteria for diagnosis of WG:Nasal or oral inflammationAbnormal CXR Abnormal urinary sediment: hematuria with or
without RBC castsGranulomatous inflammation on biopsy
Adapted from UpToDate and Harrison’s
DiagnosisAccording to the Chapel Hill Consensus
Conference:Granulomatous inflammation of respiratory
tractVasculitis of small to medium size vessels
Jennette JC et al, Arthritis Rheum 1994
Upper Respiratory TractUpper airway disease occurs in 90% of
patientsNasal involvement may present with
epistaxis, rhinorrhea, purulent dischargeOtitis media and eustachian tube blockage
can occurNasal septal perforation may occur, leading
to saddle nose deformitySubglottic tracheal stenosis can cause airway
obstruction
Pulmonary InvolvementCough, hemoptysis, dyspneaInfiltrates and cavitary lesions can be seen on
radiographyLung involvement typically appears as
bilateral, nodular cavitary infiltratesBiopsy: necrotizing granulomatous vasculitis
Renal InvolvementGenerally manifested with acute renal failureUrine sediment: red cells, red cell casts,
proteinuriaAccounts for most of the mortality of this
diseaseRenal biopsy: segmental necrotizing
glomerulonephritis
Urine Sediment
Renal Biopsy
Laboratory testsNonspecificLeukocytosis, thrombocytosis, elevated CRP,
elevated ESR can all be seen90-95% of patients with active Wegener’s are
ANCA positiveDiagnosis is confirmed by biopsy at site of
active disease
Other ManifestationsJoints: myalgias, arthralgiasEyes: episcleritis, uveitisSkin: palpable purpura, ulcerative lesionsCNS: cranial nerve abnormalitiesCardiac: pericarditis, coronary vasculitisHigh incidence of DVT
TreatmentDiagnosisRemission InductionRemission maintenanceRelapsing DiseaseRefractory Disease
Remission InductionGlucocorticoids
Dosing has not been examined in randomized trialsGenerally start 1 mg/kg/day10-20 mg daily at 12
weeksCyclophosphamide
Pulsed, IV is thought to be safer then oral Risk of myelosuppresion, infection, malignancy
Plasma ExchangeShown in small studies to improve chances of renal
recovery Recommended in lung hemorrhage
Jayne, Nephrology 2009
Remission MaintenanceAzathioprine or Methotrexate can be
substituted for Cyclophosphamide after 3-6 months
No standard length of treatment or regimenRelapses occur mostly in pt’s with continued
ANCA positivityRelapse in WG has been assoc with nasal
carriage of S. AureusUtility of TMP/SMX prophylaxis?
Jayne, Nephrology 2009
What happened?She received IV Cytoxan and Plex while
hospitalizedShe has received a total of 3 doses of IV
CytoxanBAL grew Penicillium, currently being
treated w/VoriShe developed LE petechiae and RLE DVTShe continues to be HD dependent
Sources Carruthers D et al, Evidence based management of ANCA vasculitis,
Best Practice & Research Clinical Rheumatology 2009, 23:367-378 Feder B, A Nazi Past Casts a Pall on Name of Disease, New York
Times, Jan 22 2008 Jayne D, Progress of treatment in ANCA-associated vasculitis,
Nephrology 2009, 14: 42-48 Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL,
Hagen EC, Hoffman GS, Hunder GG, Kallenberg CG, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum 1994;37:187-92
Jennette JC, Falk RJ, Small Vessel Vasculitis, New England Journal of Medicine, 1997, 21: 1512-1523
Kasper et al, Harrison’s Principles of Internal Medicine, 2005 UpToDate 2009
Questions?
Anything is possible.
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