Severe granulomatosis with polyangiitis
Gisela Lage1, Antónia Furtado1, Ana Marta Gomes2, Cátia Cunha2, Daniela Lopes2, Ana Ventura2, Clara Almeida2, João Carlos Fernandes2
1 Anatomic Pathology Service; Centro Hospitalar de Vila Nova de Gaia/Espinho
2 Nephrology Service; Centro Hospitalar de Vila Nova de Gaia/Espinho
An 81-year-old female presented at the emergency room with asthenia, anaemia and oliguria. In the previous 6 months, she had noticed recurrent epistaxis, nasal crusting and saddle nose. Two months prior to the presentation her haemoglobin was 7.2g/dl and serum creatinine was 1.25mg/dl. One month before, the patient had cystitis caused by Escherichia coli and was treated with ciprofloxacin.
At presentation, she was alert and oriented, the blood pressure 165/57 mmHg, pulse 83 per minute, and the oxygen saturation 99%.
Laboratory test results showed acute renal failure (serum creatinine 11.5 mg/dl, urea 244 mg/dl), hyperkalemia (7.2 mmol/l), anaemia (haemoglobin 6.5 g/dl), leucocytosis (11300/µl), elevation of C-reactive protein (183 mg/l) and hypoalbuminemia (2.5g/l).
Renal ultrasound showed normal kidneys size and absence of urinary tract obstruction. X-ray showed pulmonary reticular opacity localized on right inferior lobe. Endoscopic examination showed no signs of oesophageal, gastric, duodenal or colonic bleeding.
Her past medical history was significant for hypertension, and sigmoid cancer eight years before treated with chemotherapy and surgery. Her chronic medication was: 80mg telmisartan, 12,5 mg hydrochlorothiazide, 10 mg amlodipine, 1mg (lorazepam)m, 50mg quetiapine, 10mg escitalopram, 40mg sinvastatin and 20 mg (pantoprazole).
A central venous catheter was placed and started on haemodialysis; she received blood transfusion and large spectrum antibiotics.
A kidney biopsy was performed.n 81-year-old female presented.
Histology (figuras 1 a 8)
Methenamine silver, 20x. Silver staining highlighting the glomerular structure, as well as the necrotic areas. There is an intense cellularity of the interstitium, with inconspicuous small tubules, and absence of vessel staining.
H&E, 200x. Glomerulus with cellular crescent, partial destruction of the glomerular wall, and focal fibrinoid necrosis.
H&E, 100x. Collapsed vessel, with surrounding inflammation and necrotizing granuloma in the interstitium.
H&E, 100x. Necrotizing granulomatous reaction (right lower corner) in a highly cellular intertistium, with infiltration of lymphocytes and hystiocytes.
H&E, 400x. Detail of a giant multinucleated cell. It is possible to recognize besides the lymphocitic infiltration some eosinophils and polymorphonuclear cells.
H&E, 200x. Renal tubular regeneration characterized by cytoplasmic basophilia, kariomegaly, hyperchromatic nuclei. Dense infiltration of the interstitium by lymphocytes, eosinhophils and neutrophils.
CD-34 immunohistochemistry, 100x. Positivity in collapsed vessels and glomerulus.
CD-68 immunohistochemistry, 20x. Positivity in histiocytes organized in palisade around a necrotic area.
Cytoplasmic anti-neutrophil cytoplasmic antibodies (c-ANCA) 1/80, Proteinase 3 anti-neutrophil cytoplasmic antibody (PR3 ANCA) was >200 RU/ml, myeloperoxidase cytoplasmic antibody (MPO ANCA) 1.3 RU/ml, anti-glomerular basement membrane antibodies (anti MBG)
Evolution and Treatment
Treatment for ANCA positive vasculitis was initiated with steroids and cyclophosphamide. Plasmapheresis was avoided due to pulmonary infection. The patient remained haemodialysis dependent.
See the discussion in the last Portuguese Journal of Nephrology and Hypertension