Block 2 Case 6 Answers

A 47 year old man patient presents to the ER with hematuria and hemoptysis with a history of chronic sinusitis. His UA shows elevated creatinine/BUN in addition to elevated protein. Additionally, he has noted tha his vision is worse and he has very poor circulation in his fingers.

What is the above immunofluorescence pattern associated with? What enzyme is responsible for the reaction?  cANCA - cytoplasmic staining ANCAs that react with proteinase 3 (PR3).

What is the most likley diagnosis? Wegner’s Granulomatosis

Describe the following pathology specimens obtained from the patient:

Note: Necrotizing granulomas that are surrounded by palisading histiocytes, fibroblasts, and occasional giant cells. Note the inflammatory infiltrate in the vasculature (right picture). 

What forms of vasculitis are associated with ANCAs - name 3

• microscopic polyangiitis: pANCA: purpura rash, glomerulonephritis, lung involvement (no granulomas)
• Churg-Strauss syndrome - pANCA: associated with asthma, allergic rhinitis, eosinophilia, granulomas
• Wegeners: c-ANCA: acute necrotizing franulomas: upper/lower respiratory, renal disease

When a biopsy of the kidney is obtained it shows the following - what form of glomerulonephritis is this?

Crescentic Glomerulonephritis secondary to Wegener's Granulomatosis - this is a rapidly progrssing GN. It is also seen in Goodpastures, and SLE. The eosinophilic material is protein (fibrin)  which causes the epithelial cells lining the bowman capsule to proliferate and compress the glomerulus.