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Q.170. What are the clinical manifestations & diagnosis of W.G. and M.P.?


Revise please the abbreviation list on:


Vascular diseases and Vasculitis  

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Q.170. What are the clinical manifestations & diagnosis of W.G. and M.P.?  

A. Pulm. dis.:  All ptns é W.G present é upper airway/pulm. involv. & majority hv both. One 1/3rd é pulm. involv.: aSmtic. Pulm. involv. also occ. é MPA. The most common manif.:F[hemoptysis, pulm. hge & pleuritis and/or pl. eff.].

Renal dis.: in WG & MPA manf. By [ARF + hematuria, RBCs casts + Prot.]. R. biopsy: segmental NGN. é few or no immune deposits (pauci-immune) on I.F. &  E/M. Crescents: commnly occ. & almost all pauci-immune CGN. ANCA-+ve. (96 %). ANCA- -ve, pauci-immune CGN= Part of this spectrum & hv similar R. biopsy & prognosis, but there’s diff. in C.P., G. involving usually accompied by mononuclear T.I. infiltrates. However, some ptns present é T.I. +/- granuloma in abs. of typical G. les., but later on hs classic pauci-immune NGN.

R.-limited vasculitis: Alth. pauci-immune NGN. typically occ. é affection  of other organs in WG/MPA, some ptns present é R-limited, ANCA-+ve(80 % MPO-ANCA) vasculitis. R.-limited vasc. is considered a part of WG/MPA spectrum. bec.  histp. finding in  kidney are indistinguishble fr. those in WG &MPA, some ptns usally develop extrarenal manif. of WG or MPA . R.-limited vasc. tend to hv more G. sclerosis on R. biopsy thn those é WG,  bec. ptns é R.-limited dis. present later due to abs. of extrarenal manifestation. Limited form é clinical finding isolated to upper resp. or lungs, occ. in 1/4th  of cases. Alth. many (80%) dev. G.N., there’re incompletly understood phenolltypic diff. in WG. esp, ptns é limited dis. are younger at dis. onset, and more likely to be women. Despite younger at onset, ptns é limited dis. hv  longer dis. dur. thn ptns é sev. dis. & hv greater likelihood of hv. ch.recurr. dis.. It’s also hv  higher prevelence of destructive upper resp. dis. (e.g, saddle-nose), but less likely to be ANCA +ve or hv autoimmune thyroid dis.. Even if G.N. doesn’t occ. in é limited dis., other organs involv. e.g. pulm., cardiac, G.I. & CNS., may be serious & life-threatening

Q.171.What are the most common Sm. of W.G. & M.P.?  

A. The most comm. Sm.:[persistent rhinorrhea, purulent/bldy nasal disch., oral and/or nasal ulcers, polyarthralgias, myalgias, or sinus pain]. Less cmm.: upper airway Sm.:[hoarseness, stridor, earache, conductive & sensorineural hear-ing loss, otorrhea]. Lower resp. Sm. incl. [Cough, dyspnea, hemoptysis (alv. Cpll.  necrotic, or endobronchial dis.) & pleuritic pain]. Sm. cn be ass. é Sn.  of pulm. cons-olidation and/or pl. effusion. Pulm. Sm. é abs. of upper resp. Sm./Sn are unusual.

Chest x-ray” find., incl. one or more of: