Wegener’s (GPA–Granulomatosis with Polyangitis)

WEGENER’S  GRANULOMATOSIS
DESCRIBED IN 1936 BY FREIDRICH WEGENER
RENAMED WHEN IT WAS REALIZED HE WAS A NAZI
AN ASEPTIC (AUTOIMMUNE) IDIOPATHIC NECROTIZING GRANULOMATOUS VASCULITIS(BOTH A VASCULITIS AND A GRANULOMATOUS Dz)
FEMALE 3:2 MALE ?
CHILDREN CAN GET IT
USUALLY AGE 30-40
SYMPTOM TRIAD OF:
1. UPPER RESP TRACT NECROTIZING GRANULOMAS
NOSE: FIRST SYMPTOM = SINUSITIS, GINGIVAL HYPERPLASIA “STRAWBERRY GUMS”, SIALENITIS, AND SUBGLOTTIC STENOSIS
RHINITIS, EPISTAXIS, ULCERATIONS, POST. BONEY (VOMER) SEPTAL PERF–SADDLE NOSE—DO NOT OPERATED ON THEM!
LARYNX–SUBGLOTTIC STENOSIS (STRIDOR)—HIGHER INCIDENC IN PATIENTS < 20—?FREE FLAP LTP
EAR– #1 ETD–OME (CHL), #2 MASTOIDITIS (CAN EVEN GIVE FACIAL PARESIS)—MOST OFTEN MISTAKEN FOR AURAL TB (HAVE CAVITARY PULM LESIONS WITH SIMILAR INFLAMMATORY FINDINGS), #3 SSNHL
2. DISSEMINATED VASCULITIS
OFTEN AFFECTS THE LUNG–CAVITARY LUNG LESIONS—HEMOPTYSIS
3. CRESCENTRIC FOCAL GLOMERULAR NEPHRITIS–CLASSIC RBC CASTS-HEMATURIA, PROTEINURIA(HIGH UA SED RATE)
RENAL STATUS = THE MOST IMPORTANT PROGNOSTIC INDICATOR
ALSO CONSTITUTIONAL Sx: FEVER, MALAISE
Dx: Hx, UA, HIGH ESR, ANEMIA, THROMBOCYTOPENIA, CXR-CAVITARY PULM NODULES, HIGH Cr, C-REACTIVE PROTEIN
+ C-ANCA (IgG AUTO Ab—ANTI NEUTROPHILIC CYTOPLASMIC ANTIBODIES)–92-93%, + ANTI-PROTEASE – 3
P-ANCA MAY ALSO BE IMPORTANT
BIOPSY: LUNG = BEST, THEN KIDNEY—NASAL Bx = GET 10 SPECIMENS
SILVER METHAMINE STAIN–PLASMA CELLS MULTINUCLEATED LANGHANS GIANT CELLS, HISTIOCYTES, ISCHEMIC GEOGRAPHIC NECROSIS–BASOPHILIC “SMUDGY” NECROSIS
Rx: COMBO TREATMENT IS BEST
STEROIDS 1MG/KG/DAY,PULSED CYTOXAN IF RENAL INVOLVEMENT (CYCLOPHOSPHAMIDE)–(HEMORRAGIC CYSTITIS, BLADDER CA, MYELODYSPLASIA), IMMURAN (AZATHIOPRINE), TMP/SMX—-VERY SUSPECT, MTX, NS IRRIGATIONS (SUPERINFECTION OFTEN IS S.a)
REMISSION RATES 70-85%
AVOID RT

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