Hala Fekry EL-HadaryRheumatology & Rehabilitation MDCairo University2012

Case presentation

Case history

A 32 years old female presented to our clinic in May 2012 complaining of generalized bony pain and inability to walk for the last 2 years (wheel chair bound ).

12 years ago she had thrombocytopenia which improved with steroid treatment & splenectomy.

She has been receiving steroids for the last 12 years in the form of prednisone 60mg/d for 10 years which was decreased gradually to 10mg/d over the last 2 years.

She hasn’t been receiving any anti-osteoporotic treatment.

No other significant symptoms on history taking.

Case examination

Loss of height Severe kyphosisProtuberant chestTender fibromyalgia pointsCushinoid features

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Labs Revealed: Labs Revealed: ESR : 32 mm/hr CRP : negative HB : 8.7 g/dl WBC: 14.2/cumm PLT : 100,000/cmm Creat: 0.8 mg/dl AST: 56 IU/L ALT: 74 IU/L S.alb: 3.4g/dl Alk phos : 382 IU/L TP : 8.8 g/dl Thyroid profile: normal

ANA & DNA : negative Anti-platelet Ab: positive Hepatitis C: positive Total Ca: 8.6 mg/dL S.phosp: 2.1 mg/dl PTH : 129 pg/ml 25 (OH) vit D: 15 ng/ml =

37.5nmol/L

Serum 25-Hydroxyvitamin D [25(OH)D] Concentrations

nmol/L** ng/mL* Health status

<30 <12

Associated with vitamin D deficiency, leading to rickets in infants and children and osteomalacia in adults

30–50 12–20

Generally considered inadequate for bone and overall health in healthy individuals

≥50 ≥20

Generally considered adequate for bone and overall health in healthy individuals

>125 >50

Emerging evidence links potential adverse effects to such high levels, particularly >150 nmol/L (>60 ng/mL)

1 nmol/L = 0.4 ng/mL

Radiological findings

Abd U/S : chronic parenchymetous liver disease + splenectomy + no cirrhosis

Chest x-ray :Kyphosis + lung collapse + mild pleural effusion

X-ray Dorsolumber spine : kyphoscoliotic deformity of the dosal spine Multiple wedge and compression fractures of dorsal and lumbar spine1st degree spondylolithesis of L5 over S1 Spondylotic changes are foundSurgical clips are seen at left hypochondrial region ( splenectomy)

X-ray of pelvis and both hips:Bilateral coxa vena deformityBones appear poroticSub-chondral sclerotic areas are seen at the left femoral head ( ? Avascular necrosis)

DEXA

Site T-Score

Spine ‒ 6.3

Femur ‒ 5.5

Radius ‒ 6.5

Tec Bone scan :Revealed features of metabolic bone disease causing a metabolic superascan pattern that may be attributed to either BONE MARROW DISORDER (myelo-fibrosis or myelo-sclerosis) or OSTEOMALACIA .

Tec Bone scan :Presence of old healed fractures at the proximal third of the left tibial shaft ,in addition old mal-united fractures causing evident angulations of both humeral shafts are seen

Bone marrow aspiration

Bone marrow aspiration was done in 3/2001

Site : sternalRevealed normal bone marrow apart

from hypercellularity

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Key questions to this case:Key questions to this case:

Are any further investigations necessary?

Which is the correct therapeutic approach to this case ?

Is it appropriate to initiate physiotherapy?

Is there is a role for surgical interference?

Follow up : when & what to do ?

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Management :Management :

Azathiaprine : 100mg/d

Prednisone : 7.5mg/d

Paracetamol

Parathormone inj/d Calcium 500mg /twice Vit D : 0.25 mcg / d Nasal calcitonin 200 IU/d

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Patient’s question was :Patient’s question was :

Would I be able to walk again &

when?

Thanks