An uncommon cause Dr.Nihal Gunatilake - Consultant Rheumatologist - - - PowerPoint PPT Presentation

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An uncommon cause Dr.Nihal Gunatilake - Consultant Rheumatologist - - - PowerPoint PPT Presentation

Jul 02, 2023 292 likes •769 views

Knee pain An uncommon cause Dr.Nihal Gunatilake - Consultant Rheumatologist - CSTH Dr.Dinesha Sudusinghe - Registrar Medicine Case history Mrs.J, 57 years P/C B/L knee pain for 2 years H/P/C Apparently healthy.

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“Knee pain” An uncommon cause

Dr.Nihal Gunatilake - Consultant Rheumatologist - CSTH Dr.Dinesha Sudusinghe - Registrar Medicine

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Case history

Mrs.J, 57 years

P/C

  • B/L knee pain for 2 years

H/P/C

  • Apparently healthy.
  • Progressive bilateral knee pain for the last 2 years.
  • Pain increases with activity and towards the end of the day.
  • Pain worst at night.
  • No associated swelling.
  • Neck pain and lower backache for the last 6 months.
  • No small joint pain or stiffness.
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  • No heel pain, red eyes or scaly skin rash.
  • Did not have alteration of bowel habits or lower urinary tract symptoms.
  • LOA and LOW without evening pyrexia and drenching night sweats.
  • No pleuritic chest pain, chronic cough or contact history of TB.
  • Denied palpitation, easy fatiguability suggestive of anaemia.
  • Menopause 12 years back.
  • No family history of arthritis or malignancy.

PMHx - Not significant. Social Hx - Mother of three children. Activities of daily living — maintained (slowed).

Case history …

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SLIDE 4
  • Middle aged female
  • Non inflammatory KJ pain
  • LOA and LOW

?? Diagnosis

  • 1. KJ osteoarthritis
  • Primary
  • Secondary
  • 2. ?? Occult malignancy
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Examination

  • Not pale.
  • No LN , clubbing
  • No goiter/breast lumps
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Cardiovascular Respiratory

  • BP-130/90mmHg - No added sounds
  • PR- 88 bpm
  • Apex - normal position
  • No murmur

Examination…

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Examination…

  • Abdomen
  • No organomegaly
  • Neurology
  • Cranial nerves - normal
  • No motor weakness
  • No sensory impairment
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Musculoskeletal examination

B/L KJ

  • No deformity
  • No swelling
  • Full range of movements
  • Crepitus +

Spine

  • No deformities or muscle spasms
  • Full range of movement without pain

Early

  • steoarthritis
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What is the diagnosis?

  • 1. KJ osteoarthritis - Primary
  • Secondary
  • 2. ?? Occult malignancy
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X-ray knee joint - sclerotic lesions

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X-ray cervical spine - lateral view

Sclerosis of cervical vertebrae

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Sclerotic bone lesions

Focal or multifocal sclerotic bone lesions

  • Vascular
  • Hemangioma
  • Infarct
  • Infection
  • Chronic osteomyelitis
  • Neoplasm
  • Primary

▪ Osteosarcoma

  • Metastatic
  • Trauma
  • fracture (stress)
  • Endocrine/Metabolic
  • Paget's disease

Diffuse Sclerotic Bone Lesions

  • Vascular
  • Infarct (e.g. sickle cell)
  • Neoplasm
  • Metastatic

▪ Prostate ▪ Breast

  • Drugs
  • Vitamin D
  • Fluoride
  • Endocrine/Metabolic
  • Hyperparathyroidism
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D/D

  • Metastatic bone disease
  • Paget’s disease
  • Osteoblastic lymphoma
  • KJ osteoarthritis - Primary
  • Secondary
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SLIDE 14
  • FBC

WBC 5.8 x Hb 12.8 g/dl PLT 230 x 103

  • ESR 15 mm 1st hr
  • S.Ionized Ca2+ 1.13 mmol/l
  • S.Phosphate 4 mg/dl

Investigations

Normal

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  • LFT

ALT 28 U/L AST 38 U/L ALB 58 mg/dl Total protein 70 mg/dl ALP 1726 U/L GGT 38 U/L TBIL 14 µmol/l

  • Scr 60 µmol/l
  • USS abdomen
  • No organomegaly
  • No intra abdominal lymphadenopathy

Investigations

Isolated elevation of ALP

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Skull x-ray - lateral

“Cotton wool” skull

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Cortical thickening

X-ray pelvis - lytic and sclerotic lesions

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Diagnosis

  • Metastatic bone disease
  • Paget’s disease
  • Osteoblastic lymphoma
  • KJ osteoarthritis - Primary
  • Secondary
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Paget’s disease

  • Sir James Paget first described chronic

inflammation of bone as osteitis deformans in 1877.

  • Today it is known as, Paget’s disease
  • f bone.
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Paget's disease

  • Second most common bone disorder (after osteoporosis)

in elderly.

  • Common among male.
  • Cause unknown.
  • Chronic, progressive disorder.
  • Localized area of excessive bone resorption and

formation.

  • Frequently multifocal.
  • New lesions rarely develop in previously un affected

areas after the diagnosis.

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Paget's disease

  • Predilection for the

axial skeleton. (pelvis, femur, lumbar spine, and skull) (descending order of frequency)

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Pathophysiology

Normal Paget’s

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Three phases

  • 1. Lytic phase
  • 2. Mixed phase
  • 3. Sclerotic phase

At any one time, multiple stages of the disease may be demonstrated in different skeletal regions at different rates of progression.

Pathophysiology

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Histology

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Clinical features

  • Majority are asymptomatic.
  • Patient may present with non-specific symptoms or symptoms suggestive of

another disease, Bone pain Osteoarthritis Deformity Fracture Deafness

  • Diagnosis is often based on incidental findings

Elevated total or bone specific ALP Radiological findings

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Bowing of long bones Facial disfiguration

Examination

Skull enlargement

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Diagnosis

  • Serological investigations
  • Total alkaline phosphatase (ALP)
  • Bone specific ALP
  • Radiograph - characteristic appearance
  • Bone scan - to assess the extent of the disease
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Radiological investigations - Lytic phase

‘V’ shaped “blade

  • f grass” lesion

Osteoporosis circumscripta

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Sclerotic phase

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Advanced paget’s disease sclerotic and lytic lesions

“Cotton wool” skull

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Paget’s disease of vertebra - picture frame vertebral body

Cortical thickening

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Bone scan - polyostotic disease

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Impaired bone micro architecture Bowing deformity of weight bearing bones Micro-fractures Hypervascularity Nerve impingement syndrome Bone enlargement Bony overgrowth around nerves

Acceletated bone remodeling

Gait change and mechanical stress Back pain and joint pain

Osteosarcoma

Complications

Fractures High output cardiac failure Secondary

  • steoarthritis
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Treatment

Indications

Metabolically active disease

  • Bone pain
  • Fracture
  • Bony deformities and weight-bearing bone involvement.
  • Compression of spinal cord or nerve roots
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Preparation for orthopedic surgery. (If joint replacement anticipated at involved site within 6 months) Hypercalcaemia or hypercalciuria - recurrent renal calculi. Serum ALP levels greater than twice the upper limit of the reference range.

Treatment

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Treatment

Non - pharmacological

  • Gait abnormality - canes and walkers

Pharmacological

  • Bisphosphonate
  • NSAID/Opioid - pain management

Surgery

  • Bone deformities, fractures or secondary osteoarthritis
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Bisphosphonates

  • Antiresorptive agent - osteoclast apoptosis
  • Inhibit bone turnover
  • Improve bone pain
  • C/I if GFR < 35 ml/min - can substitute with

calcitonin

Bisphosphonates

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Compare the effects of two management strategies on fracture, quality of life, bodily pain, and other common complications of PDB, including the requirement for orthopedic surgery and hearing loss.

Symptomatic Intensive Any fracture 7.4% 7.0% Pagetic bone pain 30.8% 26.4% Any bone pain 73.7% 69.7% Quality of life

  • 1.2%
  • 1.3%
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Bisphosphonates…

Drug Dose Fall in ALP Reference Alendronate 40mg/day, orally, for 6 months 73-79% in 6 months Siris 1996 Risedronate 30mg/day, orally, for 2 months 69% in 6 months Reid 1996 Pamidronate 60mg/day, intravenously, for 3days 53% in 6 months Miller 2004 Zolendronic 5mg, intravenously, single dose 80% in 6 months Reid 2005

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Follow up

  • Serum total or bone specific ALP - fall within 7-10 days of starting

treatment and nadir after 3-4 months.

  • ALP every 1-2 years in zolendronic acid treated group
  • Periodic x-rays of osteolytic lesions.

Retreatment indicated if patient has not responded after 6 months of treatment or clinical or biochemical relapse.

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Surgical treatment

Hip replacement Corrective osteotomy for deformity

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Our patient..

  • Alandronate 70 mg EOD
  • Awaiting Zolendronic acid
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Future….

ZiPP (Zoledronate in Prevention of Paget’s disease)

Randomized trial of genetic testing and targeted zolendronic acid therapy to prevent SQSTM1 mediated Paget’s disease.

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Take home message

  • Morbidity from Paget’s disease can be extensive.
  • Most of the patients are asymptomatic at presentation.
  • Important to suspect and initiate treatment early to prevent

complications.

  • Treatment does not cure the disease, but it can control.
  • Prognosis is good, if treatment administered before major

changes have occurred.

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References

  • Singer FR, Bone HG, Hosking DJ, Lyles KW, Murad MH, Reid IR, Siris ES, Endocrine Society. Paget's disease of bone: an

endocrine society clinical practice guideline. J Clin Endocrinol Metab. 2014 Dec;99(12):4408-22.

  • Guideline Stresses Bisphosphonate Infusion for Paget ...www.medscape.org/viewarticle/837040
  • Ralston SH, Layfield R. Pathogenesis of Paget disease of bone. Calcif Tissue Int 2012;91:97-113.
  • Stuart H. Ralston, M.D. Paget's disease of bone. N Engl J Med 2013; 368:644-650.
  • Siris ES, Roodman GD. Paget's disease of bone. In: Rosen C, ed. Primer on the metabolic bone diseases and disorders of

mineral metabolism. Hoboken, NJ: Wiley, 2012:335-43.

  • Reid IR, Lyles K, Su G, et al. A single infusion of zoledronic acid produces sustained remissions in Paget disease --

data to 6.5 years. J Bone Miner Res 2011;26:2261-2270

  • Langston AL, Campbell MK, Fraser WD, MacLennan GS, Selby PL, Ralston SH. Randomized trial of intensive

bisphosphonate treatment versus symptomatic management in Paget's disease of bone. J Bone Miner Res 2010;25:20-31

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  • Dr. Nihal Gunatilake - Consultant Rheumatologist
  • Dr. Apsara Epa - Consultant Radiologist
  • Patient

Acknowledgement

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Thank you…..