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In many children, the spondyloarthropathies remains "undifferentiated;" some of these children fulfill criteria for "seronegative enthesopathy and arthropathy". Over time, some with undifferentiated disease develop definite sacroiliitis, leading to a diagnosis of juvenile ankylosing spondylitis.
We present a case of Juvenile ankylosing spondylitis (JAS) in a 28 yr old lady who presented with history of low back ache since 10 years of age and was evaluated for the same. On investigations she had fit into the criteria for JAS and was startred on immunomodulators. She was also subjected to bilateral hip replacement.
Key Words: Juvenile Spondyloarthropathy, low back ache
A 28 year old lady, resident of Aurangabad, Maharashtra, a post graduate by occupation was admitted to this hospital on 09 Oct 09 with complaints of painful swelling & deformity of joints and low back ache of 18 yrs. Patient was apparently asymptomatic till 18 yrs back when she developed painful swelling of joints involving bilateral hip, knee, ankle joints which was insidious onset, gradually progressive and symmetrical. It was severe in intensity for the first 6 months, associated with stiffness of the joints and feeling of warmth around the joints. She also complained of pain in both heels with swelling around the area. There was no improvement with daily activity or as the day progressed. She used to attend college without much difficulty till graduation, but there was restriction of movement of involved joints for last 3yrs. Severe since last 06 months with inability to do activities of daily living. There was no history of involvement of the joints of upper limb or neck pain. She also gave history of low back ache of last 18 yrs duration which was dull aching in nature, more in mornings, associated with night pains+, relieved with exercise and as day progressed, non radiating, no paraesthesias or root pains. Was associated with early morning stiffness lasting 60-90 minutes, alternating pain in the buttocks, severe restriction in bending forward or backward. Patient gives history of intermittent exacerbations during these 18 years associated with episodes of worsening joint pains and low back ache, subsided within 4-5 days along with decrease in the joint pains sometimes associated with feverish feeling.
What are causes of polyarthritis, oligoarthritis, monoarthritis, additive and migratory?
Poly arthritis, usually symmetric- causes include SLE, RA, Systemic sclerosis, Psoriasis, Gonococcal & Viral.
Oligoarthritis, usually asymmetric - causes include Spondyloarthropathies, Reactive, RF, Gonococcal & Viral.
Monoarthritis - causes include OA, Gout, Pseudogout, septic arthritis.
Additive arthritis - causes include RF, Gonococci, RA
Migratory arthritis - causes include Gonococci, Viral
What is enthesis organ? What is enthesitis?
The enthesis is the site of insertion of ligaments, tendons, joint capsule, or fascia to bone; it is composed of dense collagen, fibrocartilage, and adjacent bursae and synovial folds. Enthesitis (or enthesopathy), which refers to inflammation around the enthesis, is relatively specific for Spondyloarthropathy. The most common observable clinical manifestation of enthesitis is swelling at the heels, at the insertion of the Achilles tendon, or the insertion of the plantar fascia ligament into the calcaneus. On palpation, there is tenderness at these sites.
Which region comprises lower back & how frequent is LBA?
The region between lower rib cage and gluteal folds is known as lower back. Lifetime risk of LBA is 80% and it is the commonest musculoskeletal complaint.
What are the risk factors and the natural history of all backaches?
The various risk factors include Heavy lifting, Driving motor vehicle, Jogging, Weak trunk, strength, Obesity, Pregnancy, Psychosocial factors, Cigarette smoking. Most causes of lower backache are self limited and benign. 90% of these patients improve in 4 weeks and < 1% has sciatica.
What are the differentiating features of inflammatory Vs mechanical back ache?
80%, >30 min
Alternating buttock pains
How do you differentiate Sciatica Vs sclerotomal pain?
Sclerotomal pain is a non-neurogenic radiating pain which arises from pathology within the structures like disc, facet joint and lumbar paraspinal muscles/ligaments. Radiation is generally non dermatomal, only upto knees with no parasthesias/ motor weakness/reflex loss. Whereas sciatica is a neurological pain radiating along the distribution of the nerve with sensory, motor and the deep tendon jerk affected as detailed below.
Great toe dorsiflexion
Foot plantar flexion
Describe the common causes of back ache?
The causes of backache could be classified as mechanical and non mechanical as following:
Lumbar canal stenosis
What red flags would you look out for?
Major Trauma, h/o fall from height
Elderly, minor trauma- suspect osteoporotic fracture
Fever, chills, unexplained wt loss
Recent bacterial infection, IV Drug abuse, Immunosuppressed
Inflammatory back pain
Perianal/perineal sensory loss
Recent bladder/bowel dysfunction
H/O progressive LL weakness
LMN weakness in LL
Why Early morning stiffness? Night pains?
Fluid accumulation that distends the limiting boundary of inflamed tissue (tenosynovium, joint capsule, bursa). With activity- fluid clears and stiffness reduces. Duration & severity of EMS indicates the degree of local inflammation
She gives no history of weight loss, skin rash, photosensitivity, subcutaneous nodules, any nail changes, oral or genital ulcers, raynaud's phenomenon, dyspnoea on exertion, dry cough or palpitations. She also gives no history of recurrent episodes of 'red eye', dry eyes or dry mouth, dysuria, diarrhea, discharge per vaginum or oliguria. In the past there was positive history of being treated with Inj Penidure once in 3 wks for > 10 years by a local Doctor, intermittent treatment with Homeopathy and Ayurvedic medicine. No history of other co-morbid illness in the past. In the family there is history of similar complaints but of lesser severity in elder sister but with no disability. No history of similar complaints in any other family member.
On the personal front she is an unmarried, vegetarian female with no history of high risk sexual behaviou/ addictions. Bowel and bladder habits were normal. She attained menarche at the age of 13 yrs with regular 28 day cycle with each cycle lasting for 4 -5 days each.
To summarize we have a 28 year lady who presented with 18 years history of progressive, symmetrical polyarthralgia, inflammatory low back ache, enthesitis, history of progressive restriction of movements & without any extra articular manifestations. There is also associated family history.
What is the most probable provisional diagnosis at this stage?
She is a case of Juvenile Ankylosing Spondylitis
How is Juvenile AS different from Adult AS?
Juvenile AS spares the spine, predominantly affects large joints of lower limbs, hip involvement is early and rapidly destructive, enthesitis is prominent and radiological sacroiliitis occurs very late.
On general examination, height - 146 cm, Weight - 50 kg, BMI - 23.4 kg/mÂ², P - 84/min, regular, normal volume, BP - 114/84 mm Hg RAS, RR - 16/min, regular, JVP not elevated, Pallor present, No icterus, clubbing, cyanosis, pedal edema, No kyphoscoliosis, No Skin rash, Conjunctivitis, Subcutaneous nodules, Pitting of nails, Ulcerations, Digital gangrene, Vitiligo, Telengiectasia
On Musculo-skeletal Examination, Gait - antalgic gait , Tenderness present in both sacroiliac, hip and knee joints, No shortening of limbs, Upper limb - normal, Spine - lumbosacral spine movements were mildly restricted (Schoeber test 4 cm), Lateral spinal flexion- > 5cm normal, Lower limb Hip - severe restriction in hip flexion, extension, abduction and adduction movements, Knee - B/L knee effusion, mild restriction in knee flexion and hyperextension, Ankle - moderate restriction in dorsiflexion and plantar flexion, eversion, inversion.
What is Schoeber's test & Modified Schoeber's test?
Schober test is to measure the forward flexion of the lumbar spine in a patient with suspected or proven ankylosing spondylitis. With the patient standing erect, make a mark over the spinous process of the 5th lumbar vertebra or on the imaginary line joining the posterior superior iliac spine. Make another mark 10 cm above it in the midline. When the patient bends maximally forward, the distance between the two points normally exceeds 15 cm. This was originally described in 1937 by Schoeber. In 1969 it was modified by McRae and Wright to what we currently use of 10 cm above and 5 cm below and < 5 cm of increase is abnormal and suggestive of ankylosing spondylitis.
On Systemic Examination, Per Abdomen examination- Soft, non tender, No organomegaly, No ascites. CVS , S1, S2 heard, No added sounds, Respiratory System, B/L vesicular breath sounds heard, No adventitious sounds appreciated, Nervous system - NAD
How to examine for sacroiliac tenderness?
The patient can be examined for sacroiliac joint tenderness due to active sacroiliitis by applying direct pressure over each sacroiliac joint.
With the patient lying on the side, pressure is exerted by the examiner to compress the pelvis. This maneuver will elicit sacroiliac pain in patients with active sacroiliitis.
With the patient lying supine, he or she is instructed to flex one of the knees and then abduct as well as externally rotate the corresponding hip. Pressure on the flexed knee causes pain at the corresponding sacroiliac joint.
To summarize at the end of examination - A 28 year lady presented with 18 years history of progressive, symmetrical polyarthralgia involving the axial skeleton with history of restriction of movements & without any extra articular manifestations. There is also associated family history. On general physical examination, she has pallor. Musculoskeletal exam revealed antalgic gait, tenderness of b/l sacroilliac joints, bilateral hip involvement, knees, ankles with moderate to severe restriction of joints.
What are the common features of Seronegative Spondyloarthropathy and which conditions comprise this entitity?
Features include RF negativity, Scaroiliitis, Axial involvement, Peripheral arthritis, Enthesopathy, Eye involvement, Familial clustering and HLA B27 association. The various conditions which are included as part of Spondyloarthropathy are
On Investigations, Hb - 8.7 gm/dl, TLC - 10800 / cmm, DLC - P87L10M3E0, Platelets - 630 x 10Â³/ Î¼L, ESR (WG) - 120 mm fall in 1st hr, Retic count - 1 %, PBS- Microcytic hypochromic, MCV - 75 fl, Serum Iron- 30 mcg/dl , TIBC- 350 mcg/dl, RFT - WNL, LFT - WNL, CRP - 10.2 ng/ml (<3.0), Rheumatoid factor - neg , HLA - B 27 - positive.
X ray revealing sacroillitis and Hip involvement.
How common is HLA B27 in AS?
HLA B27 and AS
Frequency in normal individuals 8-14%
Frequency in AS > 90%
Prevalence of AS 05-1.4%
Prevalence of AS in B27 positive 2-6%
What are the causes of sacroiliitis?
Almost always Spondyloarthropathy, but also other diseases such as infections: Brucella, TB, Pyogenic and whipple disease. Rarely hyperparathyroidism, gout and sarcoidosis can also cause sacroillitis.
On Synovial fluid analysis Cytology - WBC's - 3000/cmm, RBC's - 450/cmm, predominant cells - neutrophils, Biochemistry - appearance- yellow, proteins- 4.0 g/dl, albumin - 2.0 g/dl, sugar - 76mg/dl, Gram stain & ZN stain - no organism seen, Culture & senstivity - no growth
Pt was managed with Inj Depomedrol 80 mg/wk IM, Tab Methotrexate 12.5 mg / wk, Tab Folic acid 5 mg/wk, Tab FeSO4 1-0-1, NSAIDS, Cap Omeprazole, Inj Triamcinolone 20mg each intraarticular b/l knee. She was stabilised medically, she underwent bilateral total hip replacement on 18 Nov 09 & 11 Dec 09.
X ray Pelvis - after B/L BHR.
Current status - she is able to walk with support, had a gradually improving course with Hb of 11 gm%. Presently she is on methotrexate, folic acid and Iron. Currently she is at home and will review with us after 1 month.
The spondyloarthropathies are a diverse group of arthritides, which typically begin in adolescence and are linked by a predilection for involvement of the back and the large joints of the lower extremities. Back involvement is often unsuspected and must be actively sought. It has been estimated that juvenile ankylosing spondylitis accounts for approximately 20 percent of cases of spondyloarthropathy, with most such children initially having the undifferentiated form. Juvenile ankylosing spondylitis (JAS) is one of the spondyloarthropathies that characteristically show inflammation around the enthesis (the site of ligament and tendon attachment to bone) and an association with the human leukocyte antigen HLA-B27. HLA-B27 is present in 90 percent of children with JAS, but only about 60 percent of children with spondyloarthropathy. Although HLA-B27 occurs with an increased frequency in these children, the presence or absence of the allele cannot be relied upon to establish or refute the diagnosis. Other spondyloarthropathies include the reactive arthropathies, psoriatic arthritis, and the axial arthropathy associated with inflammatory bowel disease. The clinical issues associated with childhood spondyloarthropathy are discussed here. The aim of this presentation is to discuss approach and management of a case of spondyloarthropathy, Highlight early hip joint involvement in Juvenile AS. Juvenile Ankylosing Spondylitis is defined as onset before 16 years of age (mean age at 10 yr). It is characterized by bilateral sacroiliitis and predominantly involves the hip.