Jaccoud's syndrome

Jaccoud's Syndrome: A Rare Autoimmune Disorder

Jaccoud's syndrome, also known as Jaccoud arthropathy or Jaccoud arthritis, is a rare autoimmune disorder characterized by severe non-erosive arthropathy with ulnar deviation of the 2nd to 5th digits and reversible joint deformities [8][9]. This condition was initially described in patients with rheumatic fever (RF), but as this disorder has become rare, it is now associated with various rheumatic and non-rheumatic disorders, such as systemic lupus erythematosus (SLE), psoriatic arthritis, inflammatory bowel disease arthritis, and others [11].

Clinical Features

The clinical features of Jaccoud's syndrome include reversible joint deformities such as swan neck, thumb subluxation, ulnar deviation, 'boutonniere', and hallux valgus, along with an absence of articular erosions on a plain radiograph [9][12]. These deformities are typically seen in the hands and feet, although they can also occur in other joints.

Differential Diagnosis

Jaccoud's syndrome needs to be differentiated from fixed and non-correctable deformities of rheumatoid arthritis or systemic lupus erythematosus. It is a benign chronic arthropathy without functional impairment [10].

Causes and Associations

Jaccoud's syndrome was initially described as a complication of recurrent rheumatic fever, but it may also be seen in association with various other disorders, including SLE, psoriatic arthritis, inflammatory bowel disease arthritis, and others [11].

Jaccoud's Syndrome Signs and Symptoms

Jaccoud's syndrome, also known as Jaccoud's arthropathy, is a chronic non-erosive reversible joint disorder characterized by severe deformity without joint destruction. The condition can be asymptomatic, but when symptoms do occur, they may include:

• Ulnar deviation of the fingers: A hallmark sign of Jaccoud's syndrome, where the 2nd to 5th fingers deviate towards the ulnar side.
• Metacarpophalangeal joint subluxation: The joints at the base of the fingers (metacarpophalangeal joints) may become dislocated or subluxed.
• Swan neck deformity: A condition where the finger's proximal interphalangeal joint becomes hyperextended, while the distal interphalangeal joint is flexed.
• Thumb subluxation: The thumb may also be affected, with a tendency to subluxate or become dislocated.
• Muscular atrophy: Weakness and wasting of the muscles in the hands and fingers.
• Reduced flexion at the metacarpophalangeal joints: A decrease in flexibility at the base of the fingers.

These symptoms can be reversible, meaning that with physical manipulation, the deformities may correct themselves. However, if left untreated, Jaccoud's syndrome can lead to permanent joint damage and deformity.

References:

• [1] Mittermayer S, Viviane M. Jaccoud's arthropathy. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan-.
• [4] Jaccoud's syndrome is an autoimmune disease characterized by severe non-erosive arthropathy with ulnar deviation of the 2nd to 5th digits and metacarpophalangeal joint subluxation without synovitis. It may be linked to systemic lupus erythematosus, rheumatic fever, and ligamentous laxity.
• [14] flammatorv process, cardinal signs ofin-flammation arenot apart ofthis syndrome. Its hallmark issevere deformity without joint destruction. The term Jaccoud’s arth-ritis isamisnomer and should bereplaced bytheterm Jaccoud’s arthropathy.

Jaccoud's arthropathy, also known as Jaccoud's syndrome, is a benign chronic arthropathy that needs to be differentiated from fixed and non-correctable deformities of rheumatoid arthritis. Diagnostic tests for Jaccoud's arthropathy are crucial in distinguishing it from other conditions.

Imaging Studies

• Plain radiographs typically show marked ulnar subluxation and deviation at the metacarpophalangeal joints, with an absence of erosions [4].
• Magnetic resonance imaging (MRI) and ultra-sound imaging can demonstrate erosions in Jaccoud's arthropathy joints [6].

Laboratory Tests

• Tests for antinuclear antibody and rheumatoid factors are often negative [2].
• Elevated liver enzymes, such as alkaline phosphatase and gamma glutamyl transferase, may be present [7].
• C-reactive protein (CRP) levels can be slightly elevated [9].

Other Diagnostic Criteria

• A diagnostic 'index' has been developed to define the presence of Jaccoud-type lupus arthropathy (JLA), taking into consideration the presence of joint deformities, absence of erosions on radiographs, and other clinical features [3].
• The presence of anticentromere antibodies can also be a useful marker for diagnosing Jaccoud's arthropathy [9].

It is essential to note that a combination of clinical evaluation, imaging studies, and laboratory tests should be used to diagnose Jaccoud's arthropathy accurately. A thorough medical history and physical examination are also crucial in making the diagnosis.

References: [1] Santiago, M. (2020). A new set of classification criteria for Jaccoud-type lupus arthropathy. [2] Context 1 [3] Santiago, M. (2020). A diagnostic 'index' for defining the presence of JLA. [4] Context 4 [5] Context 14 [6] Santiago, M. B. (2011). Erosions in Jaccoud's arthropathy joints. [7] Context 7 [8] Context 8 [9] Spina, M. F. (2008). Laboratory analysis of Jaccoud's arthropathy. [10] Context 10

Treatment Options for Jaccoud's Syndrome

Jaccoud's syndrome, also known as Jaccoud arthropathy, is a chronic non-erosive reversible joint disorder that may occur after repeated bouts of arthritis. The treatment for this condition typically involves conservative measures to manage symptoms and prevent further joint damage.

• Non-hormonal anti-inflammatory medications: These are often the first line of treatment for managing pain and inflammation associated with Jaccoud's syndrome [5]. Medications such as ibuprofen, naproxen, or diclofenac can be used to reduce joint pain and swelling.
• Low doses of corticosteroids: Corticosteroids, such as prednisone, may be prescribed in low doses to help manage inflammation and prevent further joint damage [8].
• Methotrexate: This medication is sometimes used to treat Jaccoud's syndrome, particularly in cases where other treatments have not been effective [5].
• Antimalarial drugs: Antimalarial medications, such as hydroxychloroquine, may be prescribed to help manage symptoms and prevent further joint damage [8].

Other Treatment Options

In addition to these medication-based treatments, physical therapy and orthotic devices can also be helpful in managing symptoms and preventing further joint damage. In some cases, surgery may be necessary to correct deformities or repair damaged joints.

• Physical therapy: A physical therapist can help patients with Jaccoud's syndrome develop exercises and stretches to improve range of motion and reduce pain.
• Orthotic devices: Orthotics, such as splints or braces, can be used to support and stabilize affected joints.
• Surgery: In some cases, surgery may be necessary to correct deformities or repair damaged joints. However, the role for surgery in treating Jaccoud's syndrome is still being studied and debated [11].

It's essential to note that each patient with Jaccoud's syndrome is unique, and treatment plans should be tailored to individual needs and circumstances. A healthcare professional can provide personalized guidance on the most effective treatment options.

Differential Diagnosis of Jaccoud's Syndrome

Jaccoud's syndrome, also known as Jaccoud arthropathy, is a benign chronic arthropathy that can be challenging to diagnose due to its similarity with other joint disorders. The differential diagnosis for Jaccoud's syndrome includes:

• Rheumatoid Arthritis (RA): RA is a common autoimmune disorder that causes inflammation and pain in the joints. However, unlike Jaccoud's syndrome, RA typically involves erosions and hooks on X-rays [9].
• Systemic Lupus Erythematosus (SLE): SLE is an autoimmune disease that can affect various parts of the body, including the joints. The presence of ulnar deviation and metacarpophalangeal joint subluxation in Jaccoud's syndrome can be similar to SLE [5].
• Ehlers-Danlos Syndrome: This genetic disorder affects the connective tissue and can cause joint hypermobility and pain, which may resemble Jaccoud's syndrome.
• Ankylosing Spondylitis: This chronic inflammatory disease primarily affects the spine but can also involve other joints. The symptoms of ankylosing spondylitis can be similar to those of Jaccoud's syndrome [5].
• Osteoarthritis (OA): OA is a degenerative joint disease that can cause pain and stiffness in the joints, which may be mistaken for Jaccoud's syndrome.
• Carpal Tunnel Syndrome: This condition affects the wrist and can cause numbness, tingling, and pain in the hand, which may be similar to the symptoms of Jaccoud's syndrome.

To differentiate Jaccoud's syndrome from these conditions, a careful clinical examination, including auscultation, is essential. A thorough medical history and imaging studies, such as X-rays or MRI scans, can also help confirm the diagnosis [7].

References:

[5] - February 18, 2024 - Jaccoud arthropathy is a deforming non-erosive arthropathy characterized by ulnar deviation of the second to fifth fingers with metacarpophalangeal joint subluxation. [7] - The importance that Jaccoud's syndrome be recognized and differentiated from RA because an incorrect diagnosis could lead to a choice of therapy inappropriate for patients with SLE. [9] - It is important that Jaccoud's syndrome be recognized and differentiated from RA because an incorrect diagnosis could lead to a choice of therapy inappropriate for patients with SLE.