Crains disease

Crains disease

Crains disease is an aggressive subset (5%–10%) of primary OA typically affecting the Distal Inter Phalyngeal joints, Proximal Inter Phalyngeal Joints, and first Carpo metacarpal joints bilaterally.

Prevalence

EOA primarily occurs in Caucasian women

Which age group is affected?

This condition affects the age group of 40 to 50 years

This is rare in men.

What are the symptoms?

The first MTP joints are rarely involved.

In EOA, there is a component of joint inflammation that is superimposed on the degenerative osteoarthritic symptoms.

The clinical course of EOA usually waxes and wanes for up to 5 years with painful inflammatory “flares” leading to joint deformities.

Decreased hand function is more common in patients with EOA than in those with typical nodal OA.

What distinguishes Crains disease from Inflammatory Arthritis?

Patients with EOA may be misdiagnosed as having rheumatoid arthritis (RA) or psoriatic arthritis.

In contrast to inflammatory arthritis, EOA is not accompanied by systemic symptoms, does not involve the MCP joints, wrists, or second to fifth MTP joints, has a normal ESR and C-reactive protein, and negative RF and ANA.

Additionally, radiographs in RA and psoriatic arthritis show synovial-based erosions, which occur in the periarticular “bare areas” and not centrally as seen in EOA.

However, patients with EOA do need to have superimposed crystalline arthritis ruled out (radiographs and synovial fluid analysis during “flares”).

What causes Crains disease?

The etiology of EOA is unknown.

There is a strong family history in 66% of patients.

Genetic studies have reported an association of EOA with a particular IL-1 genotype on chromosome 2.

The centrally eroded cartilage is not caused by synovial pannus invasion.

One theory is that cytokines such as IL-1 from the synovium may signal chondrocyte release of matrix metalloproteinases leading to cartilage destruction.

This may be centrally accelerated where the cartilage is thinner.

Another proposed etiology of EOA suggests a pathologic role of hydroxyapatite crystals and calcium pyrophosphate dihydrate (CPP) crystals.

How is this condition diagnosed?

Radiographs are characteristic, showing osteophytes and central “erosions” with a hallmark “gull wing” or “inverted T” appearance.

Joint ankylosis occurs in 15% of cases.

Synovial biopsies show synovial changes similar to RA with synovial hypertrophy and lymphocytic/neutrophilic infiltration.

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