Test: Rheumatoid Arthritis - NEET PG MCQ

Choice A refers to a biological treatment, while Choices B and D are DMARDs employed for the treatment of RA. Febuxostat is classified as a hypouricemic agent utilised in the management of gout, and it has the benefit of not necessitating dose adjustments in cases of renal disease.

The image depicts subluxation at the first metacarpophalangeal joint accompanied by extension of the interphalangeal joints. There is a noted deformity at the wrist joint.

• The history indicates that the distal interphalangeal (DIP) joints are spared.
• These characteristics suggest a diagnosis of Rheumatoid arthritis.

Healey Criteria for diagnosing Polymyalgia Rheumatica

• Age > 50 years
• Pain affecting proximal muscles of the limbs in 2 out of 3 regions: neck, shoulder, and pelvic girdle
• Symptoms lasting at least 1 month
• Morning stiffness lasting more than 1 hour
• Elevated ESR (>40 mm/hr)
• Quick response to low doses of steroids

Do not confuse polymyalgia rheumatica with fibromyalgia.

The image depicts a subluxation at the first MCP joint of both hands, accompanied by a deformity in the fingers of the right hand. Considering the clinical history, the diagnosis is identified as a Z line deformity associated with rheumatoid arthritis, characterised by ongoing disease activity.

• She is currently receiving methotrexate and should now be started on a triple therapy regimen that includes methotrexate, sulphasalazine, and hydroxychloroquine.
• Choice A may result in heightened side effects from methotrexate.
• Choice B might lead to a greater risk of osteoporosis.
• Choice D indicates that anti-TNF drugs are not used in isolation but rather in combination with methotrexate.

The previous treatment pyramid for rheumatoid arthritis is now considered outdated, and a more aggressive strategy aimed at achieving clinical remission is advised. If remission is not attained with methotrexate, a change in DMARD therapy is warranted, which can include:

• Oral triple therapy (methotrexate + sulphasalazine + hydroxychloroquine)
• Methotrexate combined with leflunomide
• Methotrexate paired with biological treatments such as certolizumab

The overall mortality rate associated with RA is twice that of the general population, with ischaemic heart disease being the leading cause of death. Life expectancy is diminished by 7 years for men and 3 years for women.

• Mitral regurgitation represents the most prevalent valvular disorder in RA.
• The pericardium is the most common area of cardiac involvement in RA.
• Cardiomyopathy and diastolic dysfunction are also observed.
• Coronary artery disease is another concern.
• In rare cases, the heart muscle may have rheumatoid nodules or be infiltrated by amyloid.

• Anti-CCP is the most specific antibody for rheumatoid arthritis with specificity of 95%.
• 20% patients have elevated ANA and ESR. CRP levels are elevated proportional to disease activity.

Poly-articular onset JIA (20-40%) is frequently observed in girls, with a ratio of 3:1. The peak onset age is around 3 years. It affects 5 or more joints within the initial 6 months of the condition.

• Poly-articular onset JIA typically impacts the small joints of the hand.
• Less commonly, it involves the larger joints such as the knee, ankle, or wrist.

Asymmetric arthritis can be either acute or chronic and may lead to destructive changes in 15% of cases.

• Immunoglobulin M (IgM) rheumatoid factor (RF) is detected in 10% of children within this JIA subgroup.
• This condition is linked with subcutaneous nodules, erosions, and a poor prognosis.

Around 40% of these patients are positive for ANA. Systemic symptoms like anorexia, anaemia, and growth retardation are typically moderate. There is an intermediate risk for uveitis.
TABLE: Criteria tor the Classification of Juvenile Rheumatoid Arthritis
Age at onset: <16 vr
Arthritis (swelling or effusion, or the presence of 2 or more of the following signs: Limitation of range of motion, tenderness or pain on motion.
Duration of disease: ≥ 6 wk
Onset type defined by type of articular Involvement In the 1^st 6 mo after onset:
Polyarthritis: ≥ 5 inflamed joints Oligoarthritis: ≤4 inflamed joints
Systemic disease: arthritis with a characteristic intermittent fever Exclusion of other forms of juvenile arthritis

Paud-articularJRA

1. Onset is most frequent type of JRA accounting for 60% of patients.
2. Four or fewer joints are affected and the involvement is asymmetrical. Joint swelling rather than joint pain is usual complaint.

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Polyarticular onset JRA develops in 30% of patients and more common in girls. Five or more joints are affected within the first 6 months of onset of disease.
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Systemic onset JRA
Occurs in 10% of cases with acute onset with quotidian fever, rash anywhere with central clearing, pericarditis and interstitial lung disease may be present. RA factor is negative

Involvement of the atlantoaxial region of the cervical spine is clinically significant due to its capability to lead to compressive myelopathy and neurological dysfunction. Although neurological symptoms are seldom the initial sign or symptom of atlantoaxial disorders, they can develop over time as instability between C₁ and C₂ progressively increases.

• The joints sustain damage, characterised by peri-articular erosions and osteoporosis.
• Typical joint involvement includes the P.I.P, M.C.P, and wrist joints on both sides.

• psoriatic arthritis
• Reiter's syndrome
• enteropathic arthritis
• reactive arthritis
• ankylosing spondylitis
• undifferentiated seronegative arthritis
• Whipple's disease
• arthritis linked to pustular acne
• post-intestinal bypass arthritis
• various forms of HIV-associated arthritis

Reactive amyloid A (AA) amyloidosis, a severe complication of RA, is a significant and potentially life-threatening condition resulting from the accumulation of AA amyloid fibrils in various organs. These fibrils originate from the circulating acute-phase reactant serum amyloid A protein (SAA) and can be managed through treatment.
The advent of biological therapies that target specific inflammatory mediators has transformed the management of rheumatoid arthritis (RA). By focusing on crucial elements of the immune system, these therapies effectively inhibit the pathological inflammatory cascade responsible for RA symptoms and the subsequent deterioration of joints.
Milk-alkali syndrome arises from the excessive use of calcium-containing antacids, resulting in hypercalcaemia.

• Felty’s syndrome is characterized by Rheumatoid arthritis with splenomegaly and neutropenia
• Caplan syndrome is Rheumatoid arthritis in association with coal worker pneumoconiosis
• Felty Syndrome affects approximately 1-3% of all patients diagnosed with RA, and RA occurs in about 1% of the general population.
• Many years of aggressive destructive rheumatoid arthritis (RA) precede the onset of Felty syndrome (FS). On occasion, RA and FS develop simultaneously. The extra- articular manifestations of RA (e.g., rheumatoid nodules, pleuropericarditis, vasculitis, peripheral neuropathy, episcleritis, other forms of eye involvement, Sjogren syndrome, adenopathy, skin ulcers) are more common in patients who develop Felty Syndrome)

• HLA-B27: Associated with ankylosing spondylitis, post-gonococcal arthritis, and acute anterior uveitis.
• HLA-B47: Linked to 21-hydroxylase deficiency.
• HLA-DR2: Related to systemic lupus erythematosus.
• HLA-DR3: Connected with autoimmune hepatitis, primary Sjögren syndrome, and type 1 diabetes mellitus.
• HLA-DR4: Associated with rheumatoid arthritis and type 1 diabetes mellitus.
• HLA-DR3 and HLA-DR4 combined: Also linked to type 1 diabetes mellitus.
• HLA-DQ2 and HLA-DOB: Connected to celiac disease.

In decreasing frequency, the metacarpophalangeal (MCP), wrist, proximal interphalangeal (PIP), knee, metatarsophalangeal (MTP), shoulder, ankle, cervical spine, hip, elbow, and temporomandibular joints are most commonly affected.

• Hydroxychloroquine
• Leflunomide
• Methotrexate
• Abatacept
• Anakinra
• Rituximab

Caplan's syndrome is primarily associated with pneumoconiosis, a lung disease caused by inhaling various types of dust, particularly in occupational settings. This condition often develops in individuals who have been exposed to coal dust or silica over extended periods.
Key characteristics of Caplan's syndrome include:

• Formation of nodules in the lungs, visible on X-rays.
• Typically occurs in individuals with a history of coal worker's pneumoconiosis.
• Can lead to respiratory issues and other complications.

In summary, Caplan's syndrome is closely linked to pneumoconiosis and is not directly associated with conditions like COPD, pulmonary edema, or rheumatoid arthritis.

• The image depicts nodules on the extensor distribution, alongside a peripheral smear indicating the presence of normocytic anaemia.
• The primary differential diagnosis to consider is rheumatoid arthritis.
• The nodules are non-tender and represent a late manifestation, thus not included in the diagnostic criteria for rheumatoid arthritis.
• 'Piano-key movement' of the ulnar styloid results from inflammation surrounding the ulnar styloid and tenosynovitis of the extensor carpi ulnaris.
• 'Z-line deformity' arises from the subluxation of the first MCP joint, accompanied by hyperextension of the first interphalangeal (IP) joint due to tendon and joint capsule damage.

• The clinical picture suggests atlanto axial subluxation due to rheumatoid arthritis. Thus the patient fits into Class IIIB ranawat classification of neurological deficit which is Objective weakness and long-tract signs; patient no longer ambulatory.
• Therefore we need to decide whether the patient requires conservative management or surgical intervention. This is decided by MRI spine ideally which is not given in the choices.
• The textbook then mentions that on X-ray, the anterior atlantodental interval (AADI) has been used to monitor patients with rheumatoid arthritis over time. This measures the interval from the posterior margin of the anterior ring of Cl to the anterior surface of the odontoid.
• An interval of more than 3 mm in an adult or 4 mm in a child is considered abnormal. Various authors have recommended surgery for values of more than 8 mm, 9 mm, or 10 mm. Anterior atlantoaxial subluxation may also be assessed by the PADI measurement, as measured from the posterior aspect of the odontoid to the anterior margin of the lamina of Cl.
• Rheumatoid arthritis (RA) of the cervical apophyseal joints produces neck pain, stiffness, and limitation of motion. In advanced RA, synovitis of the atlantoaxial joint may damage the transverse ligament of the atlas, producing forward displacement of the atlas on the axis (atlantoaxial subluxation).
• Surgery should be considered when myelopathy or spinal instability is present. MRI spine (Not Given in Choices) is the imaging modality of choice.

Polyarticular onset juvenile rheumatoid arthritis is defined by the involvement of five or more joints, both large and small, within the initial six months.

• Rheumatoid nodules occur in approximately 25% of patients with RA, but they occur in fewer than 10% of patients during the first year of the disease. These lesions are most commonly found on extensor surfaces or sites of frequent mechanical irritation.
• The olecranon process, the proximal ulna, the back of the heel, the occiput, and the ischial tuberosities are common periosteal sites for rheumatoid nodule development. Nodules may also form in subcutaneous tissues of the fingers, in toe and heel pads, in tendons, and in viscera.

Axial involvement in rheumatoid arthritis (RA) typically restricts itself to the upper cervical spine. Inflammation from the synovial joints and bursae in this region can result in atlantoaxial subluxation.

• The primary acquired cause of craniovertebral junction (CV) abnormalities is RA (according to the Merck manual).
• Options C and D represent congenital malformations that can cause craniovertebral anomalies.

The most suitable choice is ankylosing spondylitis, as it is the least likely to be linked with a CV junction anomaly.

• Hypoandrogenism is seen in RA. CRP can be either normal or elevated in RA, Normal CRP and normal ESR are scored as 0 points.
• A score of ≥ 6 is a must for diagnosis of RA.

The defining characteristic of rheumatoid arthritis (RA) is the ongoing symmetric polyarthritis (synovitis) that impacts the hands and feet, though any joint encased by a synovial membrane can be involved. Typically, the smaller joints of the hands and feet are impacted in a fairly symmetrical manner.

• The most frequently affected joints, in order of decreasing occurrence, include:
• metacarpophalangeal (MCP)
• wrist
• proximal interphalangeal (PIP)
• knee
• metatarsophalangeal (MTP)
• shoulder
• ankle
• cervical spine
• hip
• elbow
• temporomandibular joints

Anti Mi-2 antibodies are useful of diagnosis of dermatomyositis Potentially useful laboratory studies in suspected RA fall into 3 categories--markers of inflammation, hematologic parameters, and immunologic parameters-and include the following:

• Erythrocyte sedimentation rate (ESR)
• C-reactive protein (CRP) level
• Complete blood count (CBC)
• Rheumatoid factor (RF) assay
• Antinuclear antibody (ANA) assay
• Anti cyclic citrullinated peptide (anti-CCP) and anti mutated citrullinated vimentin (anti-MCV) assays (currently used in the 2010 American College of Rheumatology [ACR]/European League Against Rheumatism [EULAR] classification criteria)

• Pericarditis
• Cardiomyopathy

The question is based on acquired hemophilia which is seen with autoimmune disorders. For management of acquired factor VIII inhibitors, human factor VIII concentrate and recombinant factor VII concentrate is used. Aspirin and antiplatelet drugs are avoided and acetaminophen is recommended.

The newly developed anti-mutated citrullinated vimentin (anti-MCV) assay demonstrates a diagnostic performance comparable to that of the anti-CCP2 ELISA. It is particularly beneficial for diagnosing RA in patients who are anti-C.C.P2 seronegative.

• The combined use of anti-C.C.P2 and anti-M.C.V assays enhances the laboratory diagnostics for RA.
• A key benefit of testing for anti-M.C.V is the early detection of anti-M.C.V antibodies, which facilitates the identification of early RA and allows for appropriate therapy immediately following the onset of the disease.
• Additionally, anti-M.C.V titers exhibit a strong correlation with disease activity, severity, and the effectiveness of treatment.

Rheumatoid factor is an autoantibody, typically IgM, that targets the Fc region of IgG (for screening purposes). Despite its designation, rheumatoid factor is not exclusive to rheumatoid arthritis; it can also be present in a variety of autoimmune disorders, inflammatory diseases, and chronic infections. The anticitrullinated peptide antibody test (anti-CCP) is more specific than rheumatoid factor for diagnosing rheumatoid arthritis. It can yield positive results very early in the disease's progression, thus it is regarded as the ideal or initial test of choice (IOC).

DR4 is linked to:

• Extra-articular rheumatoid arthritis
• Hydralazine-induced female systemic lupus erythematosus
• Pemphigoid gestationalis
• Pemphigus foliaceus
• Obstructive hypertrophic cardiomyopathy
• IgA nephropathy

Anemia associated with chronic illness has historically included any long-term inflammatory, infectious, or malignant condition. The contemporary definition now also encompasses:

• Rheumatoid arthritis
• Severe trauma
• Heart disease
• Diabetes mellitus

In these disorders, there is primarily a reduced availability of iron, a relatively lower level of erythropoietin, and a slight decrease in the lifespan of red blood cells (RBCs) to 70-80 days. Individuals with early onset rheumatoid arthritis and a positive rheumatoid factor are more prone to developing normocytic normochromic anemia.