Abstract
Introduction
The diagnosis and management guidelines for giant cell arteritis (GCA) are relatively straightforward. However the diagnosis can be challenging when it is hidden under other inflammatory conditions and management is difficult when affected patient has other co- morbidities especially in the background of chronic suppurative infection. This article demonstrates the challenges in diagnosing and managing such case.
Case description
Our patient is an 83-year-old lady who was referred to rheumatology. She has complex medical history of chronic suppurative lung disease with pseudomonas infection. In addition she had previous ESBL urinary tract infection (UTI), deep vein thrombosis and pulmonary embolism. Management of infection is severely limited by her multiple antibiotic intolerances (gentamycin, tobramycin, colomycin, fluoroquinolones, doxycycline, cefaclor, amoxicillin, co-amoxiclav, meropenem, tazobactam/piperallin and ceftazidime). She is on prophylactic cefradine for UTI and prophylactic amoxicillin for lung infection.
On presentation, she had inflammatory polyarthritis which was subsequently diagnosed as seronegative rheumatoid arthritis (RA). She had limited therapeutic options owing to chronic suppurative lung disease. She was advised against methotrexate and leflunomide by chest physician. She could not tolerate sulfasalazine twice during the course of her illness due to gastrointestinal side effects and had to stop hydroxychloroquine because of headache and nausea. Therefore, she had to be managed with tapering doses of prednisolone and intraarticular knee injection during flairs.
Nearly two years after her first presentation, she developed recurrent headache. However, there were no other symptoms of giant cell arteritis. On clinical examination her temporal arteries were prominent, thickened and tender to touch but pulsatile. CRP was 88. Ultrasound of temporal arteries showed evidence of inflammation. Biopsy of temporal artery was not performed.
She was treated with prednisolone 20 mg daily. She continued treatment for six weeks and showed clinical improvement with normalization of CRP. Her prednisolone dose continued to be tapered successfully. However, she developed complications from treatment, in form of worsening cataract and recurrent urinary tract infections. At the time of article submission, her GCA and RA remain in remission while her prednisolone had been tapered to 9 mg daily.
Discussion
Based on NICE guidelines, the treatment of GCA is 60mg of prednisolone for patients with visual symptoms and 40mg of prednisolone for patients without visual symptoms. The risk of infections in high-dose glucocorticoids is well-established. A study using administrative data found that GCA patients, when compared with a matched historical cohort, had increased rates of lower respiratory tract infections, UTI, and other serious infections, which are defined as pneumonias, upper urinary tract infections, and sepsis. The rate of infection is the highest in the first six months of treatment during the greatest glucocorticoid exposure.
Due to her pre-existing chronic suppurative lung disease, it was deemed to be of too high risk to commence on the standard dose of prednisolone and to adhere to the recommended length of tapering. We have decided to opt for a lower dose (20mg) of prednisolone for 6 weeks and taper after that. The dose was selected based on the risk and benefit assessment of her underlying chronic infection. There may be difficulties in future management because usual steroid-sparring agents (DMARDs and biologics) would be contra-indicated.
According to NICE guidelines, the diagnosis of GCA should be confirmed on temporal artery biopsy (TAB). We have decided that the clinical and imaging evidences were sufficient to diagnose her GCA. We felt that temporal artery biopsy would not add or change her therapeutic management.
Key learning points
This case highlights the difficulty in managing multiple inflammatory conditions in the presence of complex chronic infections. In case in which all recommended treatments are contraindicated, we have to be prepared to deviate from the recommended guidelines and make decision based on patient tailored risk benefit assessment.
This case also serves as a reminder of the importance of shared decision-making and multi-disciplinary approach in managing complex clinical case. She is at risk of developing complications from prolonged glucocorticoid use, and therefore we have involved the local respiratory team and discussed the case in the rheumatology multi-disciplinary (MDT) meeting.
Conflicts of interest
The authors have declared no conflicts of interest.
Clinical course of children with chronic suppurative lung disease or bronchiectasis infected with Pseudomonas aeruginosa
