Discussed by Prof. Seza Ozen*, Discussed by Prof Asim Das**
Department of Pediatrics, Unit for Nephrology and Rheumatology
Hacettepe University Faculty of Medicine
Sihhiye, Ankara, Turkey.*, Addl. Professor
Department of Histopathology, PGI, Chandigarh. **
|17 yrs old girl admitted with complaints of ear discharge for 1 year and 7th |
Cranial nerve palsy for 1 month. On examination the patient was sick, febrile, tachypneic. Tachycardia, enlarged right axillary lymph node, depressed nasal bridge, membrane over uvula & palate, purpuric rash, hearing loss, facial nerve palsy and crepts on auscultation over chest were noted. There was cough and mucoprulent expectoration. Granulation tissue was seen on myringotomy. On investigation high ESR, high platelet count, low Hb, abnormal renal function tests, multiple veinous thrombosis and normal coagulogram were recorded. Chest X ray revealed cavitating lesion and blood culture grew Staph. Aureus at admission.
With this history following possibilities can be considered:
- Vasculitis secondary to infection: Staph? Secondary fungal? Microbiology and biopsy
- Septic emboli and infection
- HSP: Purpura not typical?Too severe. Biopsy IgA?
- Associated Malignancies: Need biopsy
- Behçet's Disease: Does not meet criteria
- Secondary vasculitis of SLE: No autoAb, but needs biopsy (IF staining)
- Lymphomatoid granulomatosis: Need biopsy
- Good Pasture Syndrome: (-) anti-GBM
- Churg Strauss Syndrome: No asthma, no eosinophilia, no eosinophilic granuloma, no MPO-ANCA
- Microscopic polyangiitis
Wegener's Granulomatosis (points in favour)
- Multisystem involvement
- Symptoms not adequately explained with infection
- High APR, high thrombocytes
- Skin rash
- Depressed nasal bridge
- Triad: URT+LRT+Kidney
Final Diagnosis: Wegener's Granulomatosis
partial autopsy was performed. The prosector noted flattening of the nasal bridge with serosanguinous 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 1200 gm. 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.
Microscopically: 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 lymph nodes were enlarged & showed reactive hyperplasia.
Kidneys: Wt. 300gm 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. 1100gm. 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. 110gm. 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. 285gm. 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 lymph nodes and muscle were WNL.
Final Autopsy Diagnosis:
- Wegener's Granulomatosis
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|Ozen S P b D, Das A P b D.. Available From : http://www.pediatriconcall.com/fordoctor/ Conference_abstracts/report.aspx?reportid=100|