A CASE OF PARANEOPLASIC ANCA VASCULITIS
- Nuno V. Vieira e Brito, Portugal
Abstract
Background and Aims
Vasculitis as a paraneoplasic syndrome is rare and the pathofiosology is yet unclear. Association between ANCA vasculitis and solid tumour is an even rarer condition with very few cases in literature.
Methods
73 year old woman admitted for vision loss with migraine headache, weight loss, asthenia and low fever. She had diabetes, arterial hypertension, prothrombin gene mutation and aortic and mitral valve biological prosthesis. Ophthalmology suggested isquemic optic neuropathy and was referred to internal Medicine.
Results
She was anaemic, high erythrocyte sedimentation rate, positive rheumatoid factor with normal complement and negative HIV and hepatitis. Doppler of the temporal, facial and occipital arteries showed a thickness without halo and biopsy was absent for giant cells. Serologies were positive for C-ANCA antibodies with anti-PR3 of 361. Methylprednisolone was given with partial recuperation of sight. Biopsy of nose turbinate and CT scan of sinus and thorax where absent for granuloma. The patient responded well lowering the erythrocyte sedimentation rate and continued improvement of sight but without lowering of C-ANCA title, 621 at a second evaluation. After discharge was readmitted for myocardial infarction this admission was complicated by endocarditis to streptococcus oralis and hematochezia. Colonoscopy revealed adenocarcinoma and underwent hemicolectomy with adenectomy after undergoing 2 cycles of chemotherapy and suspending all steroids there was no evidence of vasculitis with a lowering of the antibody title to 300.
Conclusions
The authors present a case of a tumor induced vasculitis whose diagnosis precedes that of the tumor and whose symptoms and antibody titer improve only with tumor control and treatment.