Juvenile Ankylosing Spondylitis

Background

Ankylosing Spondylitis is a rare chronic inflammatory autoimmune disorder affecting primarily sacroiliac joint and axial skeletal. Affecting males more than females with a ratio of 2:1. It is one of the diseases among Spondyloarthropathies, others include

* Psoriatic Arthritis

* Reactive Arthritis

* Enteropathic Arthritis

* Undifferentiated Spondyloarthropathies.

There is another form of Ankylosing Spondylitis that is juvenile Ankylosing Spondylitis affecting children under 16 years of age.

Clinical Presentation

Morning Stiffness lasting more than 30 minutes.

Insidious onset pain.

onset in early age, before 40.

Peripheral  Enthesitis and Arthritis.

organ-specific extra-articular manifestation such as uveitis.

Bamboo spine typical presentation of AS

Pathophysiology

It is a chronic inflammatory response mediated by CD 4 and CD 8 cells. Tumor necrosis factors and growth factors along with macrophages play a key role in inducing fibrosis and joint limitation.

Etiology

Both Environmental and Genetic factors such as HLAB27 Allele.

Diagnosis

clinical history and radiograph showing inflammation and sclerosis of SI and other joints. Labs investigation includes raised ESR levels and positive HLAB27 allele.

 

Case Report  #  Juvenile Ankylosing Spondylitis

A 21-year-old girl presented to the orthopedic OPD of THQ Wazirabd with lurching Gait and a chief complaint of joint pain mainly of hip, spine, and knee associated with morning stiffness lasting more than 2 hours. The pain was non-radiating, moderate to severe in intensity, relief with activity, and little response to NSAIDs.

History of the present illness reveals that at the age of 5 she suddenly got both knee pain and swelling for that she visited a medical center and was advised for a home exercise plan. At the age of 12, she visited a medical center to seek help for her back and hip pain, there she came to know that her hip joints are becoming less mobile. Some days later she fell due to limited hip movement and got a fracture of the Right shaft of the femur that was treated by a quack and resulted in a short right leg.

Her objective examination reveals that her right leg was internally rotated and with a valgus deformity at the knee joint. Bilateral hip joints show limited SLR and a scoliotic deformity at lumber level with convexity towards the Left side. Her neurological exam was intact.

Investigations

Several x rays and labs were investigated to rule out the cause

pelvis Ap view radiograph show marked reduction of joint space in hip joints

Left SI joint was completely fused

The right SI joint show marked sclerosis on both sides of the joint

A lower limb CT scanogram show 1.5cm shortening of the right limb

 

 

 

 

 

 

 

 

An ultrasound abdomen show no gross pathology

ANA +ve

HLA-DRB 1 +ve

Management

pharmacological management includes DMARD with NSAIDs

physiotherapy

patient education

dietary and lifestyle modification

surgical management includes corrective osteotomy to correct femur internal rotation and genu valgum.

Follow up

the patient showed a marked reduction in pain and was much satisfied.