Discussed by Prof Asim Das
Addl. Professor
Department of Histopathology, PGI, Chandigarh.
A partial autopsy was performed. The prosector noted flattening of the nasal bridge with serosanginous material exuding from the nasal cavities in this emaciated small – built female. Multiple reddish macular lesions were seen over limbs.
Serous cavities- No significant effusion noted.
Larynx & Lungs:- The larynx showed mucosal ulceration with granulation tissue formation. There was granulomatous inflammation with many giant cells and small vessel involvement. The lungs weighed 1200gms. Fibrinous pleuritis with many adhesions and thickened pleura at base were seen. The trachea and bronchi showed focal ulcerations. The lungs were solid to feel and reddish brown in colour. On slicing there was variegated appearance. There was involvement of both the lungs and all the lobes to varying extent, by areas of haemorrhagic consolidation punctuated by large yellowish areas of necrosis. These measured from half a centimeter to as large as 6cms. The larger lesions showed cavitation. Only a small portion of parenchyma was spared. The bronchi contained purulent material.
Microscpically:- A variety of changes were noted. Large areas of geographic necrosis with nuclear debris surrounded by organization, fibrosis, granulomatous inflammation with many giant cells were seen. Many alveoli contained proteinaceous exudates. Extensive vasculitis involving both arteries and veins with transmural inflammation, thrombosis with recanalisation seen. Mostly small & medium sized vessels were involved; the outlines of which were better recognized by EVG stain. Fibrinoid necrosis was not particularly present. Capillarities was noted but more prominently appreciated in areas of haemorrhages. Superadded bronchopneumonia containing many Gram positive bacterial colonies present. Stains for fungus and Mycobacteria were negative. Many pulmonary veins showed thrombo-emboli. The hilar lymphnodes were enlarged & showed reactive hyperplasia.
Kidneys:- Wt.300gms together. They showed “flea-bitten appearance’. C/s revealed tiny haemorrhages with slight medullary congestion. Renal arteries and veins were patient.
Micro:-Approx 20% glomeruli were involved with segmental necrotizing glomerulonephritis. Fibrinoid change was restricted to one or two lobules but occasionally involved the whole tuft. An occasional adhesion was seen. Tubules showed RBC casts. Mild interstitial inflammation was noted. No granulomas or definite extra-glomerular vasculitis noted.
Liver:- Wt.1100gms. The outer surface and cut surfaces did not show any infarcts. The IVC and hepatic vein radicles – No thrombi.Micro:- No significant pathology.
Spleen:- Wt. 110gms. Perisplenitis with adhesions to diaphragm was noted. Micro:- Multiple granulomas were seen. There was granulomatous change with giant cell formation involving small arteries and venules with microscopic infarcts.
GIT:- The oesophagus, stomach & large gut showed patchy areas of congestion. However, small gut revealed focal mucosal ulcerations which were limited to mucosa submucosa and showed granulation tissue at the base with vasculitis in some areas. The pancreas was normal but revealed thrombi in small vessels without inflammation of the wall.
Heart:- Wt. 285gms. Small mural thrombi in right atrium were seen. The valves & chambers WNL. No necrosis or inflammation. Coronary arteries – normal.
Other Organs:- The adrenals, uterus, ovary, thyroid, Urinary bladder,mesenteric lymphnodes and muscle were WNL.
Final Autopsy Diagnosis:-
- Wegener's Granulomatosis
- Bronchopneumonia.
