Dr Theresa Raju, Dr Suma Balan(Paed rheumatologist), Dr C Jayakumar ,
Amrita Institute of medical science ,KOCHI Kerala ,India
Thirteen ear old girl who was initially symptomatic with complaints of inflammatory polyarthritis involving the right ankle and bilateral knee joints.
She was evaluated in a local hospital for the same and was suspected to have psoriatic arthritis.
Patients also had complaints of abdominal pain since 1 month.
Pain was initially intermittent in nature and later progressed to be persistent in nature after 1 week. In view of the above mentioned complaints, the child was admitted in AIMS for further evaluation and management. No history of rashes, oral or genital ulcers, fever, pain or redness of eyes, diarhhea or blood in stools.
There is no similar past history or family history of the same.
At admission, the child was conscious, alert.
Vitals were stable with normal blood pressure. PICCLE normal
Her peripheral pulses were felt and there was no evidence for aortic regurgitation, bruits, carotidynia (Carotidynia is classified as an idiopathic neck pain syndrome and is associated with point tenderness over the bifurcation of the carotid artery)
Systemic examination
Abdominal examination showed tenderness in the periumblical area on deep palpation. There was no tenderness or swollen joints.
Labs
TC:10.2Ku/ml, N:60.8%, L:26.8%, ESR:74mm/1st hr, CRP:9.20mg/L.
On evaluation, she had elevated inflammatory markers. She underwent colonoscopy which revealed mild inflammation and biopsies were taken which was suggestive of non specific inflammation.
However a CT enterogram was done which revealed aneurysmal outpouching of the abdominal aorta.
An aortogram was done to characterize as well as to evaluate the extent, severity and distribution of disease.
It showed 2 aortic psuedoaneurysms with wall thickening at the origin the celiac axis with no intralesional thrombus.
A provisional diagnosis of large vessel vasculitis was made.
Etiologies considered include Behçet’s disease
IgG4 related aortitis and Takayasu arteritis.
In view of atypical site, lack of stenosing lesions and a past history of arthritis a diagnosis of Behcet’s was considered.
Supporting the diagnosis, HLA B tissue typing was done which was positive for HLA B51.
Ophthalmology did not reveal any uveitis. Pediatric cardiology consultation no evidence of aortic regurgitation or aneurysmal root dilatation.
She was started on pulse methylprednisolone followed oral prednisolone.
As a steroid sparing agent, mycophenolate mofetil was added.
Further she was started on low dose aspirin , in view of intramural thrombus.
She tolerated the medicines well and was discharged on stable vitals. The child is currently on Injection Tocilizumab(interleukin receptor6inhibitor) every month.
Behcet’s disease:
It is classified as a primary variable vessel vasculitis, emphasizing the involvement of any size and type(arterial,venous) of blood vessels.It is a polygenic autoinflammatory disorder. Genetic contribution to BD is evident through the well knows association with HLA-B5101.
The mean age of the first symptom is between 8 and 12 years. The most frequent initial symptom is a painful oral ulcer. Another feature is bilateral eye involvement seen in 30-60% pediatric patients. The main symptoms of anterior uveitis are blurred vision, redness, periorbital or global pain. Pathergy( occurrence of lesions following trauma that closely parallel the pathology of the primary diseaseSimilar to Kobner phenomenon )is another skin feature associated with BD.
GI involvement manifests with abdominal pain, diarrhea and intestinal ulcerations. Oligoarticular arthritis/arthralgia is present in more than 50% patients and can be recurrent.
Treatment: Azathioprine is highly recommended to treat inflammatory eye disease. Anti tumour necrosis factor treatment and interferon alpha should be considered for refractory eye disease.
For oral and genital ulcers, topical treatment is recommended. In pediatric patients with vascular involvement with venous thrombosis , corticosteroids and azathioprine have been used.
Mortality in children with BD is low except for the pulmonary aneurysms. However BD is a chronic disease associated with significant morbidity. Early diagnosis and effective treatment improves the outcome of BD.
Carry home message
Aphthous ulcers are commonly encountered pediatric problem. But if it is associated with abdominal pain and vascular involvement Bechets disease should be strongly considered and managed