Basic Calcium Phosphate Disease / Milwaukee Shoulder Syndrome

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Overview

Basic Calcium Phosphate (BCP) disease is a crystal-associated arthropathy characterized by deposition of BCP crystals in periarticular and intra-articular tissues. A severe clinical presentation of BCP disease is Milwaukee Shoulder Syndrome, which typically affects elderly women, leading to rapid joint destruction, massive rotator cuff tears, and non-inflammatory joint effusion. It often involves the shoulder, but other joints like the hip and knee may be affected.

Definition

BCP Crystals: Includes hydroxyapatite, octacalcium phosphate, and tricalcium phosphate; not birefringent and very small.
Milwaukee Shoulder Syndrome (MSS): Rapidly destructive shoulder arthropathy due to BCP crystals, classically in elderly women.
Rotator Cuff Tear Arthropathy: Shoulder joint destruction secondary to long-standing rotator cuff tear, often seen in MSS.
Hydroxyapatite Deposition Disease (HADD): Disease resulting from abnormal deposition of HA crystals in tendons or bursae, leading to pain and inflammation
Calcific tendinitis: Clinical term describing symptomatic HADD, often in the shoulder.

Anatomy and Physiology

  • Shoulder Joint (Glenohumeral): Ball-and-socket joint stabilized by the rotator cuff tendons and labrum
  • Rotator Cuff (SITS): Supraspinatus, Infraspinatus, Teres minor, Subscapularis — commonly disrupted in MSS.
  • BCP crystals deposit in periarticular soft tissues, tendons, and joint cartilage, triggering matrix degradation and joint destruction.

Aetiology and Risk Factors

Aetiology

  • Abnormal crystal formation and deposition due to joint aging, cartilage degeneration, or microtrauma
  • Enzymatic activity (e.g. collagenase, MMPs) leads to structural breakdown

Risk Factors

  • Age > 70
  • Female sex
  • Long-standing rotator cuff tear
  • Osteoarthritis
  • History of joint trauma or surgery
  • Crystal arthropathies (e.g. CPPD, gout)

Remember

Milwaukee Shoulder Syndrome = Elderly woman + shoulder swelling + rotator cuff tear + non-inflammatory fluid.

Pathophysiology

  • BCP crystals form in joint or periarticular tissues due to altered phosphate metabolism or cartilage damage.
  • Crystals stimulate macrophages and fibroblasts to release degradative enzymes (e.g. collagenase, elastase).
  • Results in:
    • Rotator cuff rupture
    • Joint instability
    • Joint space narrowing and erosions
    • Massive joint effusion with low cellularity
  • Crystals are too small for polarised microscopy, making diagnosis challenging.

Unlike gout/pseudogout, BCP crystals do not cause overt inflammation, but cause chronic degeneration.

Clinical Manifestations

Milwaukee Shoulder Syndrome (classic presentation)

  • Elderly woman with painless or mildly painful swelling of shoulder
  • Large non-inflammatory effusion
  • Marked shoulder instability and crepitus
  • Loss of active motion (due to rotator cuff tear), preserved passive motion
  • Pain and loss of function as destruction occurs of joint

Other features

  • Recurrent joint effusions
  • Affects shoulder > hip > knee
  • May resemble neuropathic (Charcot) joint

Triad Milwaukee Shoulder Syndrome: Elderly lady + rotator cuff tear + painless massive effusion

Diagnosis

No formal criteria; diagnosis is clinical + imaging + synovial fluid + exclusion of other causes

Synovial fluid analysis

  • Large-volume effusion, low WBC count (<2000/mm³)
  • No crystals seen on polarised light
  • Can use alizarin red staining to identify calcium-containing crystals
  • Electron microscopy or X-ray diffraction can identify BCP (rarely used clinically)

Imaging

  • X-ray
    • Superior migration of the humeral head
    • Severe humeral head and glenoid destruction
    • Soft‑tissue calcifications and joint space narrowing
    • Intra‑articular loose bodies and periarticular osteopenia
  • MRI
    • Massive rotator cuff tear – especially supraspinatus and infraspinatus, often with tendon retraction
    • Superior migration of humeral head
    • Large joint effusion
    • Articular cartilage loss and bony erosions – destructive arthropathy of humeral head and glenoid
    • Minimal synovial thickening
  • Ultrasound
    • May show calcification and effusion

Remember

Always aspirate and analyze synovial fluid in an elderly patient with large shoulder effusion before assuming OA or RA.

Remember

Fluid is non-inflammatory yet damaging — unlike gout or septic arthritis.

Crystal Arthropathy Clinical Comparison Table

FeatureGoutPseudogout (CPPD)BCP / Milwaukee ShoulderHADD (Hydroxyapatite)
Typical Age30–50 (M > F)>60>70 (F > M)30–60 (F > M)
Crystal Type & ShapeMonosodium urate, needleCalcium pyrophosphate, rhomboidBCP (hydroxyapatite), amorphousHydroxyapatite, amorphous
BirefringenceStrongly negativeWeakly positiveNoneNone
Common Joint Involved1st MTP, midfoot, ankleKnee, wristShoulder (glenohumeral joint)Shoulder (supraspinatus tendon)
Synovial Fluid WBCHigh (2,000–50,000+)Moderate (2,000–50,000)Low (<2,000), non-inflammatoryNormal to mildly elevated

Treatment

Conservative (mainstay)

  • Joint aspiration (for symptomatic relief)
  • NSAIDs (for mild pain)
  • Physiotherapy (passive ROM to prevent contractures)
  • Intra-articular corticosteroids (if inflammation suspected)

Surgical (if severe)

  • Reverse total shoulder arthroplasty in cases of joint collapse or significant dysfunction
  • Rotator cuff repair rarely indicated due to chronicity

Complications and Prognosis

Complications

  • Progressive joint destruction
  • Loss of function and disability
  • Recurrent large joint effusions
  • Prosthetic failure if arthroplasty performed late

Prognosis

  • Prognosis poor without early supportive management
  • Disease is not systemically inflammatory, but causes major mechanical disability

References

  1. Dieppe PA, Swan A. Identification of calcium crystal deposition diseases. Best Pract Res Clin Rheumatol. 2005;19(6):983–1000.
  2. Halverson PB, et al. Milwaukee shoulder syndrome: clinical and laboratory findings in 38 patients. Arthritis Rheum. 1986;29(5):567–574.
  3. Tanimoto K, et al. Milwaukee shoulder syndrome: destructive shoulder arthropathy associated with apatite crystals. Mod Rheumatol. 2006;16(2):86–92.
  4. Oliviero F, et al. Basic calcium phosphate crystals in osteoarthritis: morphology, localization and association with clinical parameters. Clin Exp Rheumatol. 2008;26(3):410–416.
  5. McCarty DJ, et al. BCP crystal-associated arthritis: pathogenesis and management. Curr Rheumatol Rep. 2003;5(3):221–227.

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