Rheumatoid arthritis

Editor's Notes

1. Rheumatic arthritis: 15 years old male presented with multiple joints pain..ie polyarthritis. The joints involved were the knees,ankle, wrists ,elbow but the nature way migratory. According to the pt, he had pain in right knee that lasted a few days and then as the pain was subsiding he developed right ankle pain in succession and that’s called migratory or .OE the objective signs of inflammation were minimal .Joint pain usually is more prominent than objective signs of inflammation & is almost always transient. Radiography of an affected joint may demonstrate a slight effusion but is usually unremarkable. Analysis of the synovial fluid in RF with arthritis generally demonstrates sterile. The natural history of the polyarthritis in ARF is altered by empiric treatment with NSAIDS. OA: Deep, achy joint (typically weight bearing large joints)pain exacerbated by extensive use - The disease’s primary symptom Reduced range of motion and crepitus - Frequently present Stiffness during rest (gelling) - May develop, with morning joint stiffness usually lasting for less than 30 minutes Osteoarthritis of the hand DIP joints are most often affected PIP joints and the joints at the base of the thumb are also typically involved Heberden nodes, which represent palpable osteophytes in the DIP joints, are more characteristic in women than in men Inflammatory changes are typically absent, less pronounced, or go unnoticed. (Hard bony swelling) Arthrocentesis :The presence of noninflammatory joint fluid helps distinguish OA from other causes of joint pain. Other synovial fluid findings that aid in the differentiation of OA from other conditions are negative Gram stains and cultures, as well as the absence of crystals when fluid is viewed under a polarized microscope. Physical examination findings: mostly limited to affected joints Osteoarthritis (see the image below) is the most common type of joint disease. It is the leading cause of chronic disability in those older than 70 years, OA is characterized by narrowing of joint space due to cartilage loss and osteophytes due to bone remodeling but not erosions or cysts. O is classically aossicated with abcence of RF S and normal level of acute phase reactants but however RFs can be present but usually in low titer corresponding to patients older age.
2. The typical presentation is with pain, joint swelling and stiffness affecting the small joints of the hands, feet and wrists. Large joint involvement, systemic symptoms and extra-articular features may also occur. Clinical criteria for the diagnosis of RA are shown and By convention, symptoms need to have persisted for more than 6 weeks for the diagnosis of RA. 1. Morning stiffness: Morning stiffness in and around the joints, lasting at least 1 hour before maximal improvement. Symptoms present for a minimum of 6 weeks. Morning stiffness is a common feature of those with active RA; it has been defined as the following: "slowness or difficulty moving the joints when getting out of bed or after staying in one position too long, which involves both sides of the body and gets better with movement" . Morning stiffness, or stiffness after any prolonged period of inactivity, is also seen in virtually all inflammatory arthropathies and myopathies . However, morning stiffness lasting more than one hour reflects a severity of joint inflammation that rarely occurs in diseases other than RA. Arthritis of 3 or more joint areas: At least 3 joint areas simultaneously have had soft tissue swelling or fluid (not bony overgrowth alone) observed by a physician; the 14 possible joint areas are right or left proximal interphalangeal (PIP) joints, metacarpophalangeal (MCP)joints, wrist, elbow, knee, ankle, and metatarsophalangeal (MPT) joints. Findings present for a minimum of 6 weeks. Arthritis of hand joints: At least one area swollen (as defined above) in a wrist, MCP or PIP joint. Findings present for a minimum of 6 weeks. Note: Examination of the hands often provides a good reflection of overall disease activity. The typical features are symmetrical swelling of the metacarpophalangeal (MCP) joints and proximal IPJs. These and other joints are actively inflamed if they are tender on pressure, and have stress pain on passive movement or effusion/soft tissue swelling. Erythema is not usually a feature and its presence implies coexistent sepsis. Characteristic deformities develop with long-standing disease, including 'swan neck' deformity, the boutonnière or 'button hole' deformity, and a Z deformity of the thumb. Dorsal subluxation of the ulna at the distal radio-ulnar joint is common and may contribute to rupture of the fourth and fifth extensor tendons. Triggering of fingers may occur because of nodules in the flexor tendon sheaths. In the foot, dorsal subluxation of the MTP joints may result in 'cock-up' toe deformities. This causes pain on weight-bearing on the exposed MTP heads and development of secondary adventitious bursae and callosities. In the hindfoot, calcaneovalgus (eversion) is the most common deformity, reflecting damage to the ankle and subtalar joint. This is often associated with loss of the longitudinal arch (flat foot) due to rupture of the tibialis posterior tendon. Popliteal ('Baker's') cysts usually occur in combination with knee synovitis, where synovial fluid communicates with the cyst but is prevented from returning to the joint by a valve-like mechanism. Rupture, often induced by knee flexion in the presence of a large effusion, leads to calf pain and swelling. Nodules are found in flexor and extensor tendons of hand, also in sacrum, Achilles tendon, even lungs and myocardium. Symmetric arthritis :Simultaneous involvement of the same joint areas (see 2 above) on both sides of the body (bilateral involvement of PIPs, MCPs, or MTPs is acceptable without absolute symmetry). Findings present for a minimum of 6 weeks. Rheumatoid nodules: Subcutaneous nodules, over bony prominences, or extensor surfaces, or in juxta-articular regions, observed by a physician. Serum rheumatoid factor: Demonstration of abnormal amounts of serum rheumatoid factor by any method for which the result has been positive in <5% of normal control subjects. Radiographic changes: Radiographic changes typical of RA on posteroanterior hand and wrist radiographs, which must include erosions or unequivocal bony decalcification localized to or most marked adjacent to the involved joints (osteoarthritis changes alone do not qualify). This criteria demostrated 91-94% sensitivity and 89 5 specificity for RA when compared with non RA rheumatic disease control subjects.
3. Swan neck deformity is a deformed position of the finger, in which the joint closest to the fingertip is permanently bent toward the palm while the nearest joint to the palm is bent away from it (DIP hyperflexion with PIP hyperextension). Boutonniere deformity is a deformed position of the fingers or toes, in which the joint nearest the knuckle (PIP) is permanently bent toward the palm while the farthest joint (DIP) is bent back away (PIP flexion with DIP hyperextension).
4. Target population (Who should be tested?): Patients who 1) have at least 1 joint with definite clinical synovitis (swelling)* 2) with the synovitis not better explained by another disease† Note: * The criteria are aimed at classification of newly presenting patients. In addition, patients with erosive disease typical of rheumatoid arthritis (RA) with a history compatible with prior fulfillment of the 2010 criteria should be classified as having RA. Patients with longstanding disease, including those whose disease is inactive (with or without treatment) who, based on retrospectively available data, have previously fulfilled the 2010 criteria should be classified as having RA. † Differential diagnoses vary among patients with different presentations, but may include conditions such as systemic lupus erythematosus, psoriatic arthritis, and gout. If it is unclear about the relevant differential diagnoses to consider, an expert rheumatologist should be consulted. ‡ Although patients with a score of 6/10 are not classifiable as having RA, their status can be reassessed and the criteria might be fulfilled cumulatively over time. § Joint involvement refers to any swollen or tender joint on examination, which may be confirmed by imaging evidence of synovitis. Distal interphalangeal joints, first carpometacarpal joints, and first metatarsophalangeal joints are excluded from assessment. Categories of joint distribution are classified according to the location and number of involved joints, with placement into the highest category possible based on the pattern of joint involvement. . “Large joints” refers to shoulders, elbows, hips, knees, and ankles. # “Small joints” refers to the metacarpophalangeal joints, proximal interphalangeal joints, second through fifth metatarsophalangeal joints, thumb interphalangeal joints, and wrists. ** In this category, at least 1 of the involved joints must be a small joint; the other joints can include any combination of large and additional small joints, as well as other joints not specifically listed elsewhere (e.g., temporomandibular, acromioclavicular, sternoclavicular, etc.). †† Negative refers to IU values that are less than or equal to the upper limit of normal (ULN) for the laboratory and assay; low-positive refers to IU values that are higher than the ULN but 3 times the ULN for the laboratory and assay; high-positive refers to IU values that are 3 times the ULN for the laboratory and assay. Where rheumatoid factor (RF) information is only available as positive or negative, a positive result should be scored as low-positive for RF. ACPA anticitrullinated protein antibody. ‡‡ Normal/abnormal is determined by local laboratory standards. CRP C-reactive protein; ESR erythrocyte sedimentation rate. §§ Duration of symptoms refers to patient self-report of the duration of signs or symptoms of synovitis (e.g., pain, swelling, tenderness) of joints that are clinically involved at the time of assessment, regardless of treatment status. The 1987 American College of Rheumatology (ACR; formerly the American Rheumatism Association) classification criteria for rheumatoid arthritis (RA) have been criticised for their lack of sensitivity in early disease. This work was undertaken to develop new classification criteria for RA. Compared with the 1987 criteria, the 2010 criteria classify more patients with RA and at an earlier phase of the disease. 
5. The disease onset is usually insidious, with the predominant symptoms being pain, stiffness, and swelling of many joints. Up to one-third of patients have the acute onset of polyarthritis associated with prominent myalgia, fatigue, low grade fever, weight loss, and depression. Occasionally, the syndrome of polymyalgia rheumatica may be present. When this occurs in the absence of clinically detectable synovitis, the distinctive clinical features of RA may not develop until months or even years later. Sjogren syndrome The association of keratoconjunctivitis sicca (lack of lacrimal secretion ) and xerostomia (dry mouth due to lack of salivary gland secretion) in association with connective tissue disorder usually RA. Thius may be a/w other autoimmune conditions like autoimmune thyroid disease, myasthenia gravis or ai liver disease. Diagnosis; schirmer filter paper test : less than 5 mm is wet after 5 min . Normally it is >15 mm. Anti Ro (SSA) and anti SS-B antibodies may be found. Rx: artificial tear, nsaid for arthritis. Mononeuritis multiplex is a painful, asymmetrical, asynchronous sensory and motor peripheral neuropathy involving isolated damage to at least 2 separate nerve areas. Multiple nerves in random areas of the body can be affected. As the condition worsens, it becomes less multifocal and more symmetrical. Mononeuropathy multiplex syndromes can be distributed bilaterally, distally, and proximally throughout the body. The most common symptom is dry eyes (keratoconjunctivitis sicca) due to secondary Sjögren's syndrome. Painless episcleritis frequently accompanies nodular seropositive disease; it may cause intense redness of superficial vessels but sight is unimpaired. Scleritis is more serious and potentially sight-threatening; the eye is red and painful, vision is often impaired and the sclera shows a deep red blush beneath the individual red superficial vessels. Scleromalacia is painless bilateral thinning of the sclera, with the affected area appearing blue or grey (the colour of the underlying choroid). Cardiac and pulmonary involvement Cardiac involvement occurs in about 30% of patients with seropositive RA but is usually asymptomatic. Symptomatic pericardial effusions and constrictive pericarditis are rare. Occasionally, granulomatous lesions can cause heart block, cardiomyopathy, coronary artery occlusion or aortic regurgitation. Serositis is commonly asymptomatic but may cause pleurisy or breathlessness. Pulmonary fibrosis can occur in advanced RA and may cause dyspnoea .
6. Neurological complications Peripheral entrapment neuropathies may result from compression by hypertrophied synovium or by joint subluxation. Median nerve compression in the carpal tunnel is most common and bilateral compression can occur as an early presenting feature of RA. Other syndromes include ulnar nerve compression at the elbow or wrist, compression of the lateral popliteal nerve at the head of the fibula, and tarsal tunnel syndrome (entrapment of the posterior tibial nerve in the flexor retinaculum) which causes burning, tingling and numbness in the distal sole and toes. Diffuse symmetrical peripheral neuropathy and mononeuritis multiplex may occur in patients with rheumatoid vasculitis. Cervical cord compression can result from subluxation of the cervical spine at the atlanto-axial joint or at a subaxial level . Atlanto-axial subluxation is a common finding in long-standing RA and is due to erosion of the transverse ligament that is posterior to the odontoid peg. On neck flexion, this allows the peg to move posteriorly and indent the cord. If unrecognised, it can lead to cord compression or sudden death following minor trauma or manipulation. Atlanto-axial subluxation should be suspected in any RA patient who describes new onset of occipital headache, particularly if symptoms of paraesthesia or electric shock are present in the arms. In some patients, the onset may be insidious, with subtle loss of function that is initially attributed to active disease. Reflexes and power can be very difficult to assess in the presence of marked joint damage and therefore sensory or upper motor signs are the most important to elicit. Amyloidosis is a rare complication of prolonged active disease and usually presents with nephrotic syndrome. Generalised and local lymphadenopathy affecting nodes draining actively inflamed joints may both occur. Patients with persistent lymphadenopathy should be biopsied since there is an increased risk of lymphoma in patients with long-standing RA.
7. AS: 4As ,apical fibrosis, anterior uveitis, aortic regurgitation, Achilles tendonitis . (back pain and stiffness)with loss of lumbar lordosis.bamboo spine. Spinal and axial involvement Reiter’s syndrome : conjunctivitis, urethritis nongonococcal and Asymmetric oligoarthritis Psoriatic arthritis: nail pitting, psoriatic plaques and dip arthropathy. Pencil in a cup deformity.
8. Osteoarthritis (see the image below) is the most common type of joint disease. It is the leading cause of chronic disability in those older than 70 years, OA is characterized by narrowing of joint space due to cartilage loss and osteophytes due to bone remodeling but not erosions or cysts. O is classically aossicated with abcence of RF S and normal level of acute phase reactants but however RFs can be present but usually in low titer corresponding to patients older age.
9. The presence of nodules indicates seropositive and more aggressive arthritis.
10. Infectious arthropathies fever and polyarthritis. Infections often result in a transiently positive RF, so this is not helpful in differentiation. If bacterial arthritis is suspected, joint aspiration and synovial fluid cultures and blood cultures are often helpful in establishing the diagnosis. exception is gonococcal arthritis and Lyme disease ( endemic region where tick exposure was likely). Viral infections, such as parvovirus B19 infection, (accompanying rash, and their self-limited course). systemic lupus erythematosus (SLE) can have a similar distribution of joint involvement but rarely have erosive disease. Jaccoud's arthropathy often leads to deformities that are similar in appearance to those in RA, but these changes result not from joint destruction but from tendon and ligament laxity. These deformities, unlike those in RA, are readily reducible on examination. In most cases, the other clinical manifestations of SLE and serologic findings are helpful in establishing the diagnosis. Polymyositis and dermatomyositis can manifest with arthralgias, arthritis, and joint stiffness, similar to RA. Muscle weakness and antibodies associated with these disorders most often readily distinguish these disorders from RA. Polyarticular crystal arthropathies can also mimic RA. Radiographs might also show joint erosions, but in calcium pyrophosphate dihydrate deposition disease, chondrocalcinosis is often apparent. Synovial fluid analysis is diagnostic of crystal arthropathy. Polymyalgia rheumatica and giant cell arteritis can manifest with symmetrical polyarthritis. In these patients, a detailed history can help identify the characteristics of these disorders (e.g., a new, unrelenting headache, predominant shoulder and hip girdle involvement). In cases where history does not make the distinction, careful observation of the evolution of the disease over time is key, because the clinical courses and potential serious complications associated with these disorders are quite different. Fibromyalgia (FMS) can manifest with diffuse symmetrical arthralgias and stiffness at rest, but the absence of synovitis, the lack of pain on motion, and normal laboratory and imaging studies confirm the diagnosis of FMS. It is important to remember that FMS is present in 10% to 15% of patients with rheumatic diseases such as SLE and RA; hence, its diagnosis does not exclude other concomitant disease.19,20 Sarcoidosis can manifest with polysynovitis and is not infrequently RF-positive, but it has other distinguishing characteristics that often help to differentiate it from RA. In Löfgren's syndrome, the acute onset of polyarthritis is accompanied by erythema nodosum and hilar adenopathy. Chronic sarcoid arthropathy may clinically closely mimic RA, and on radiographs it can show bony destruction similar to that in RA. Tissue biopsy may be necessary in these instances if no other manifestations of sarcoidosis are present to establish the diagnosis.