MSK Re-Up #2 Flashcards
Eosinophilic Granulomatosis with Polyangiitis (EGPA - Churg-Strauss) is systemic small and medium sized granulomatous necrotizing vasculitis.
What is this a rare side effect of?
Montelukast and Zafirlukast
What triad of symptoms is associated with EGPA?
What labs (there is a specific lab)
Asthma + Eosinophilia + Chronic Rhinosinusitis
Eosinophilia + P-ANCA HALLMARK
What diagnostic is most accurate for EGPA?
Treatment?
Biopsy
Glucocorticoids (may add Cyclophosphamide if severe)
On the other hand, granulomatosis with polyangiitis is inflammation and necrosis of what three things?
What are symptoms?
-Nose, lungs, kidneys
-Triad: upper respiratory tract involvement + lower respiratory tract involvement + glomerulonephritis
–Nasal congestion, saddle nose deformity, otitis media, stridor, refractory sinusitis
–cough, dyspnea, wheezing, hemoptysis, pulmonary infiltrates
–rapidly progressive glomerulonephritis (crescent on biopsy), hematuria, RBC casts, proteinuria
Best initial test for GPA - Wegener’s
C-ANCA
Treatment for GPA - Wegener’s
Glucocorticoids PLUS Cyclophosphamide
Microscopic Polyangiits (MPA) is small and medium vessel vasculitis that is NOT associated with _________.
It’s symptoms include…
Nasopharyngeal symptoms, necrosis, or granulomatous inflammation (like in GPA)
Palpable purpura, cough, dyspnea, hemoptysis, acute rapidly progressive glomerulonephritis (crescentic)
Lab and treatment for MPA
-P-ANCA
Glucocorticoids PLUS Cyclophosphamide
Immunoglobulin A Vasculitis (Henoch-Schoenlein-Purpura) MC occurs in ________.
It often occurs after __________
What are the four cardinal symptoms (HSPA)
-MC occurs in children (3-15 years old)
After infection (URI, GABHS, Parvovirus B19)
Hematuria
Synovial (arthritis or arthralgia)
Palpable Purpura (on LE)
Abdominal Pain (may present with GI bleeding)
Usually a clinical diagnosis, Henoch-Schonlein Purpura has what lab studies?
What is seen on kidney biopsy?
-Normal PT, PTT, and platelets
-Mesangial IgA deposits
What is the treatment for HSPA?
-Supportive: NSAIDs, hydration, bed rest
Anti-GBM Antibody (Goodpasture’s) Disease is a Type II hypersensitivity reaction where IgG antibodies are against…..
Symptoms are limited to which systems? What are the symptoms?
Against Type IV collagen of alveoli and glomerular basement membrane of the kidney
Limited to lungs and kidneys
-Hemoptysis, dyspnea, dry cough, hematuria, oliguria
What is the best initial test for Goodpasture’s?
Next diagnostic (specific antibody)
What is seen on biopsy of the kidney?
UA: Increased BUN/Cr, hematuria, proteinuria, dysmorphic RBC’s, RBC casts
-Anti-glomerular basement membrane antibodies
-Linear IgG deposits. Crescentic glomerulonephritis
Treatment for Goodpasture’s
-Glucocorticoids + Cyclophosphamide + Plasmapheresis
Psoriatic Arthritis is inflammatory arthritis in patients with psoriasis. What are risk factors associated with this, along with other seronegative spondyloarthropathies?
Young males, < 40 years old, HLA-B27 positivity