Ferri – Basic Calcium Phosphate Crystal Deposition Disease

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Basic Calcium Phosphate Crystal Deposition Disease

  • Zuhal Arzomand, M.D.

 Basic Information

Definition

Basic calcium phosphate crystal deposition disease, commonly known as calcific periarthritis, refers to periarticular and intraarticular deposits of calcific material. Basic calcium phosphate (BCP) comprises crystals of partially carbonate-substituted hydroxyapatite (a form of calcium phosphate), octacalcium phosphate, magnesium whitlockite, and tricalcium phosphate. BCP crystal deposits are often asymptomatic, although intermittently they can become symptomatic and elicit acute and chronic arthritis. Acute attacks can be manifest as hot, swollen, tender joint symptoms appearing clinically similar to gout, pseudogout, cellulitis, and septic arthritis. BCP crystals are frequently found in the synovial fluid of patients with osteoarthritis and are involved in the soft tissue calcinosis seen in scleroderma and dermatomyositis.

Synonyms

  1. Calcific periarthritis/calcific tendonitis

  2. Hydroxyapatite deposition

  3. Apatite-associated destructive arthritis

  4. Idiopathic destructive arthritis of the shoulder

  5. BCP crystal-associated destructive arthropathies

  6. BCPCDD

  7. Milwaukee shoulder syndrome

  8. Apatite-associated destructive arthropathy (AADA)

ICD-10CM CODES
M11.0 Hydroxyapatite deposition disease
M11.8 Other specified crystal arthropathies
M11.9 Crystal arthropathy, unspecified

Epidemiology & Demographics

Prevalence

Often asymptomatic and incidentally found; therefore, prevalence is not established.

Predominant Sex

Females > males

Predominant Age

Incidence increases with age (50-90 yr)

Genetics

Caucasian predominant

Risk Factors

Most cases have spontaneous onset. May arise from trauma, infection, or overuse.

Associated clinical conditions: aging, osteoarthritis, diabetes mellitus, chronic kidney disease, hyperparathyroidism, connective tissue diseases, and autoimmune diseases such as dermatomyositis, scleroderma, CREST syndrome, and SLE.

Physical Findings & Clinical Presentation

  1. •

    Periarticular calcific deposits are often asymptomatic.

  2. •

    Commonly affects shoulder (usually dominant side, although 60% bilateral). Greater trochanter of hips, knee, elbows, wrists, ankle joints, and digits are also common sites.

  3. •

    Acute attacks have a sudden onset of severe pain, swelling, warmth, local tenderness, and associated loss of function. Reduced active ROM in all affected joints is sometimes associated with joint instability. Most present with spontaneous onset but may be preceded by mild trauma or overuse.

  4. •

    Hydroxyapatite pseudopodagra: describes acute calcific periarthritis of the first metatarsophalangeal joint, usually in young women

  5. •

    Milwaukee shoulder syndrome: describes destructive arthropathy of the shoulder (usually the dominant one) from calcium deposits, leading to disruption and loss of function in shoulder, usually in females older than 60 years (Fig. E1 and Table E1)

    FIG.E1 

    Anteroposterior radiographs of a shoulder joint affected by basic calcium phosphate crystal–associated destructive arthritis (Milwaukee shoulder syndrome).
    The extensive destruction of periarticular tissues, including the rotator cuff, has led to instability of the shoulder. A, The upward subluxation of the humerus can be overcome by B, traction on the shoulder. Note the extensive atrophic destruction and loss of bone of both the acromion and the glenohumeral joint.
    Hochberg MC, et al.: Rheumatology, ed 5, St Louis, 2011, Mosby.
    TABLEE1 Clinical Syndromes That Can Be Associated With the Deposition of Basic Calcium Phosphate Crystals in and Around JointsHochberg MC, et al.: Rheumatology, ed 5, St Louis, 2011, Elsevier.
    Subcutaneous deposits (e.g., calcification of hands in scleroderma) Asymptomatic chance finding
    Acute and chronic inflammation
    Skin ulceration
    Secondary infection
    Pressing necrosis of surrounding tissues
    Mechanical interference with function
    Periarticular deposits (e.g., calcification of supraspinatus tendon) Asymptomatic chance finding
    Acute calcific periarthritis
    Chronic periarticular pain and/or dysfunction
    Intraarticular deposits (e.g., synovial and cartilage deposits in damaged joints) Asymptomatic chance finding
    Acute synovitis
    Severe osteoarthritis
    Destructive arthropathies of older people

    BCP, basic calcium phosphate.

Etiology

  1. •

    Idiopathic.

  2. •

    The formation of BCP crystals is multifactorial but largely depends on extracellular inorganic pyrophosphate (PPi) regulating the formation of pathologic and physiologic minerals. Reductions in extracellular PPi promote BCP formation, whereas excess ePPi promotes CPPD and inhibits BCP crystal formation.

  3. •

    Metabolism of extracellular PPi is directed by several proteins, including ANKH (ankylosis human), TNAP (tissue nonspecific alkaline phosphatase), ENPP-1 (ectonucleotide pyrophosphate phosphodiesterase 1), PC-1 (plasma-cell membrane glycoprotein 1), PiT-1 (sodium-dependent phosphate transport protein 1), TGF B-1, and CD73.

Diagnosis

Differential Diagnosis of Acute Calcific Periarthritis

  1. •

    Gouty arthritis

  2. •

    Pseudogout

  3. •

    Septic arthritis

Differential Diagnosis of Chronic Calcific Periarthritis

  1. •

    Osteoarthritis (unusual sites of osteoarthritis such as shoulder, elbow, ankle joint should lead to further investigation)

  2. •

    Seronegative polyarthritis

  3. •

    CPPD

  4. •

    Trauma, impingement

  5. •

    Inflammatory arthritis

  6. •

    Neuropathic joint disease

Workup

Laboratory Tests

  1. •

    Phosphorus, calcium, magnesium, vitamin D, alkaline phosphatase levels, iron panel, and renal function for any metabolic causes

  2. •

    Synovial or bursal fluid analysis: low cell count in crystal arthropathies, viscous, sometimes blood tinged in destructive arthropathies

  3. •

    BCP crystals are small, less than 0.1 μm (20-100 nm) long, needle shaped, nonbirefringent

  4. •

    Calcium stains: Alizarin red stain (highly sensitive–lacks specificity to qualify for routine use) or oxytetracycline stain with UV light

  5. •

    Labeled diphosphonate binding

Imaging Studies

  1. •

    Plain radiograph: first line. AP and lateral sufficient, but internal or external rotation may be needed for retro humeral deposits.

    1. ○

      Inert periarticular calcium deposits appear dense, homogenous, without trabeculations with well-defined borders

    2. ○

      Acute calcific periarthritis attacks appear fluffy with poorly defined margins as crystals shed into surrounding tissues, with soft tissue swelling

  2. •

    Ultrasonography: highly sensitive; hyperechoic with posterior acoustic shadowing

  3. •

    Scanning and transmission electron microscope—due to small size (20-100 nm)

  4. •

    CT scan or MRI of affected joint (Fig. E2)

    FIG.E2 

    Axial T2-weighted magnetic resonance imaging scan of a shoulder joint affected

Treatment

Asymptomatic deposits: no treatment needed

Nonpharmacologic Therapy

  1. •

    Immobilization, heat application, range of motion exercises

Acute General Rx

  1. •

    Analgesics, nonsteroidal antiinflammatory drugs, or colchicine

  2. •

    Nonselective COX inhibitors (BCP crystals induce both COX-1 and COX-2)

  3. •

    Needle aspiration of the deposits or effusions with or without irrigation

  4. •

    Intraarticular injection of steroids

  5. •

    Pain relief with suprascapular nerve blocks, or transcutaneous nerve stimulation

Chronic Rx

  1. •

    Physical therapy

  2. •

    Pulsed ultrasonography—increased rate of resorption and reduced pain

  3. •

    Surgical therapy (arthroplasty, debridement, or joint replacement) may be warranted for restoration of function and pain relief

  4. •

    Disodium ethylenediamine tetraacetic acid (EDTA)—administered at painful site with mesotherapy

Referral

  1. •

    Rheumatology

  2. •

    Orthopedic surgery if considering joint replacement

Pearls & Considerations

Comments

Basic calcium phosphate deposition should be considered in the differential diagnosis of acute polyarthritis, especially in the setting of chronic severe OA. Specific crystal identification is difficult with methods that are currently clinically available.

Prevention

None known

Suggested Readings

  • J.M. Blair-Levy: Carbonated apatite-induced arthropathy: a consideration in cases of polyarthritis. Nat Rev Rheum. 2 (5):278–283 2006

  • A. Cacchio, et al.: Effectiveness of treatment of calcific tendinitis of the shoulder by disodium EDTA. Arthritis Rheum. 61:1 2009 19116964

  • H.-K. Ea, F. Liote: Calcium pyrophosphate dihydrate and basic calcium phosphate crystal-induced arthropathies. Curr Rheumatol Rep. 6:221–227 2004 15134602

  • H.-K. Ea, F. Liote: Diagnosis and clinical manifestations of calcium pyrophosphate and basic calcium phosphate crystal deposition diseases. Rheum Dis Clin North Am. 40 (2):201–229 2014

  • C. Hochberg, et al.: Rheumatology. ed 6 2011 Elsevier St Louis

  • D.W. Wu, et al.: The crowned dens syndrome as a cause of neck pain. Arthritis Rheum. 53:1 2005 15696572

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