​Pathology - Wegener Granulomatosis

​Pathology - Wegener Granulomatosis 
Attributable to an immunologic mechanism, potentially a hypersensitivity reaction.
Primarily affects males between the ages of 40 and 60, however it can manifest at any age.

Study of the nature and causes of diseases. 
Gross Pathology: Vascular granulomatous lesions observed in the upper respiratory tract, lungs, and kidney.
Study of cellular and tissue abnormalities at a microscopic level. The presence of necrotizing granulomas characterized by a central area of necrosis surrounded by lymphocytes. The presence of macrophages and large cells within the vascular walls, together with granulomatous vasculitis characterized by an inflammatory infiltrate surrounded by fibroplastic growth, is observed. Additionally, there is evidence of rapidly increasing glomerulonephritis in the renal biopsy.

Clinical manifestations and indications 
Nasal septum perforation, chronic sinusitis, otitis media, difficulty breathing, coughing up blood, blood in urine, skin rash, muscle pain, heightened risk of deep vein thrombosis.
Complications encompass renal failure, sometimes known as nephritic syndrome, as well as hearing.
Imaging: Chest X-ray reveals prominent nodular opacities.
Laboratory results: The patient has a positive c-ANCA result and the presence of red blood cells and/or red blood cell casts in their urine.

Treatment: The first therapy involves the use of cyclophosphamide and/or prednisone, while methotrexate is used for maintenance therapy.

Microscopic polvangitis is a type of inflammation in the small blood vessels that causes tissue death. It is characterized by the rapid progression of kidney inflammation and involvement of the lungs. The biopsy reveals a condition characterized by the death of tissue and a low immune response in the small blood arteries, without the presence of granulomas. This condition is linked to a positive p-ANCA and is managed using cyclophosphamide and corticosteroids.