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BY:HARDINI PRAJAPATI MPT STUDENT CARDIORESPIRATORY
rheumatology, a subspeciality of internal med and ped,is devoted to the diagnosis & therapy of rheumatoid dz. The term originates from greek RHEUMA meaning ³that which flows as a river or stream´ and the suffix-ology, meaning ³the study of´
Rheumatologists, mainly deals with problems involving jts and the allied conditions of connective tissues. Rheumatism, is a nonspecific term used to describe any painful disorder affecting the locomotor system; including jts,ms,connective tissues, soft tissues around jts and bones.
Rheumatism is also used to describe rh fever affecting heart valves. However , the med profession use specific terms to describe rh disorders such as RA,RF,AS,SLE,GOUT and more««««. rheumatic disorder, is a nonspecific form of med problems affecting the heart,bones,jts,kidney,skin and lungs.
Rh disorders are like a common cold and there are more than 100 types of disorders are there, for eg;OA, RA, RF, AS, GOUT, STILL¶S DZ, SLE, SCLERODERMA, SPONDYLITIS, TENDINITIS, VASCULITIS, SJOGREN¶S SYN, BURSITIS, AN, DUPUYTREN¶S DZ, PSORIATRIC ARTHRITIS, PSEUDO GOUT, REITER¶S SYND, INFECTIOUS DISORDERS «««««««. AND MANY MORE.
The term was first used by GARROD in 1858 but was not accepted by ARA as the official terminology untill 1941. definition: it is a systemic connective tissue disorder which affects predominantly the synovial jts, hence the term µRHEUMATOID DZ¶
2. RA is a chronic inflammatory systemic dz of young and middle aged adults, characterized by destructive and proliferative changes in synovial memb, periarticular stru, sk ms and perineural sheaths. Eventually , jts are destroyed, ankylosed and deformed. [ SAMUEL L TUREK] 3. It is a non suppurative systemic infl dz of of unknown cause characterized by a symmetrical polyarthritis affecting peripheral jts and extra articular stur. [TIDY]
1.5 million ppl affected in UK where as 2.1 million are affected in USA. F:M, 3:1. there is a general increase in prevalence for both sexes with incresing age. Age of onset is as young as 16 yrs but is generally in the 20-55 yrs grp. There is some differences in the prevalence of RA in certain subpopulations ,which suggests a possible role for genetic or environmental factor in etiology of the dz. eg
Etiology of RA is still unknown. Current researches into the causes of RA is based on the complex, but as yet incomplete, appreciation of the fun of IMMUNE SYSTEM. IMMUNE THEORY: based on the fact that indi with RA produces antibodies to their own Igs, hence there is some reason to believe that RA is an AUTOIMMUNE DISORDER.
INFECTIOUS THEORY: evidence suggests that a variety of agents may initiate arthritis through a no of mech. THEORY OF RF: RF are found in sera of approx 70% of all pts with RA. RF are antibodies specific to IgG. Current theory suggests that RF arise as antibodies to altered autologous [ pt¶s own] IgG. RA occurs in the -nce of RF in a no of indi. Indi with RF or seropositive , have increased freq of subcutaneous nodules, vasculitis and polyarticular inv.
GENETIC THEORY: RA has been associated with increased HLA-D and HLADR [ D related] antigens suggesting that certain genes determine whether a host is more or less at risk for an immunological response that leads to RA. A µrheumatoid epitope¶ has been identified thr DNA typing of HLA-DR4 as a particular sequence of amino acids common among pts with RA.
The most widely held theory of pathogenesis is that an immunologic response takes place in the synovial tissues. An unknown exogenous antigen encounters the defender cell, the lymphocyte, which is transformed into a larger plasma cell, which manufactures antibodies. Antigen+antibody= complex. Scavenger phagocytic cells engulf this complex.
These phagocytes contain enzyme producing sacs, lysosomes, that destroy the complex. Some of the lysosomes escape from phagocytes and their proteases attack the cartilage and synovium. Destruction of tissues produces debris that calls for more phagocutic activity to remove debris. Consequantly more phagocytes pour outs more enzymez which create more desyruction and further infl and arthritic process becomes selfperpetuating.
The inflamed synovium forms a PANNUS , a granulomatous mass that grows over and destroys cartilage, tendons and lig. This mass consists of 3 types of synovial cells; TYPE A are phagocytic, TYPE B resembles fibroblasts and TYPE C are undifferentiated cells.
The disorder is primarily a synovitis. Early hyperemia, edema occur, lining cells proliferate until they are three or more layers thick and underlying tissue is infiltrated with lymphocytes and plasma cells. Villous processes gradually develop and project into the jt cavity and they may become necrotic and etruded into the jt.
The typical microscopic lesion is an area of fibrinoid necrosis surrounded by fibroblasts conspicuously arranged radially to the surface of necrosis. Beyond this is an enveloping layer of fibrous tissue. The rh units and infiltration of round cells are prominent not only in synovium but also in periarticular str. Leucocytes frequently are aggregated into round collection that may encircle the blood vessel. And increase amt of clear or turbid fluid accumulate in the jt.
The synovium at the periphery forms a pannus that grows progressively and extends over the articular surface, absorbing and replacling the articular cartilage with fibrous connective tissue. Vascular granulation tissue from the marrow extends towards the art surface & destroys the cartilage from within bone. The art cortex becomes thin and deficient so that fibrous pannus forms the main covering of bone.
The granulation tissue extends towards opp art surface, merging with pannus there, bridging the jt with granulation tissue. A fibrous ankylosis sesults. The fibrous tissue may under go metaplasia into bone.within the articulating bones, the no of trabecule become less and thin. Fibrous proliferation thickenes the capsule.
Chronic inflammation can weaken jt capsule and supporting lig, altering jt stru and fun. Tendon rupture and fraying tendon sheaths may produce imbalanced ms pull on these pathologically altred jts. This results in musculoskeletal deformities, seen in advanced RA.
Pathological changes in muscle:
Nodular polymyositis Increase in size and no of sarcolemmic nuclei, loss of striation, swelling of ms fibres and localized collection of lymphocytes. Damaged ms is replaced by fibrous tissue, so loss of elasticity and contractile power will result in restriction of ROM.
They are composed of the typical basic rheumatoid unit, consisting of a central necrotic zone, a surrounding layer of large mono nuclear cells radially arranged and a outer zone of dense connective tissue with marked round cell infilteration. Found in 20% pts and +nt over pressure areas.
Redused blood flow in arterioles results in cold and cyanosed distal extremities. Lymphnodes exhibit follicular hyperplasia and there will be increased RE activity.
For most of the 20th century, criteria had allowed four classification of RA: classical, definite, probable and possible. ARA criteria for diagnosis of RA: Morning stiffness Pain on motion or tenderness in at least one jt Swelling of one jt due either to soft tissue or effusion or both
1. 2. 3.
4. Swelling of at least one other jt with an interval free of symptoms no longer than 3 mths. 5. Symmetrical jt swelling 6. Subcutaneous nodules 7. Typical radiographic changes which must include demineralisation in periarticular bone as an index of infl. 8. Positive RF in serum
9. Synovial fluid showing poor mucin clot formation when added to dilute acetic acid. 10. Histopathology of synovium consistent with RA . 11. Characteristic histopathology of rh nodules. . S/S must be +nt for at least 6 weeks to satisfy criteria 1 to 5.
RA may be classified as: CLASICAL: if 7 criterias +nt DEFINITE: if 5 criterias +nt PROBABLE: if 3 criterias +nt POSSIBLE: any of the following +nt at least for 3 weeks; Morning stiffness, H/O jt pain/swelling, subcutaneous nodules, eievated ESR/CRP
New criteria were tested & established in 1987 based on a combination of signs, sym and lab findings that have persisted for a specific period of time. A diagnosis of RA is now established upon the presentation of four of 7 listed criteria, must lasted for at least 6 weeks.
The 1987 revised criteria for classification of RA: MORNING STIFFNESS; in and around the jt lasting at least 1 hr before maximal improovement. ARTHRITIS OF 3/MORE JTS; at least 3 jts simultaneously have had soft tissue swelling or fluid observed by physician. The 14 possible areas are rt/lt PIP, MCP, wrist, elbow,knee,ankle & MTP jts
3. ARTHRITIS OF HAND JTS; at least one area swollen in a wrist, MCP , PIP 4.SYMMETRICAL ARTHRITIS; simultaneous involvement of the same jt areas on both sides of body. 5. RH NODULES; subcutaneous nodules over bony prominences or extensor surface or in juxta articular regions, observed by a physician.
6. SERUM RF; demonstration of abnormal am of serum RF by any method for which the result has been +ve in <5% of normal control sub. 7 RADIOGRAPHIC CHANGES; typical of RA on hand and wrist radiographs, which must include erosion or unequivocal bony decalcification localized in/most marked adjacent to the involved jts.
TYPES OF PRESENTATION
CLASSICAL: pain ,stiffness and swelling of small jts of hands and wrists. Symptoms fluctuate in severity from day to day. PALINDROMIC: intermittent episodes of pain, swelling and redness, usually of a single jt followed by rapid return to normal after several days. SYSTEMIC: wt loss, pleurisy and pericarditis but minimal jt involvement.
POLYMYELGIC: pain and stiffness in sh and hips with subsequent synovitis. MONOARTHRITIC: single jt involvement, usually the knee. ACUTE ONSET: sudden overnight onset with stiffness and pain. WITH GENERALISED LYMPHODENOPATHY
STAGES OF RA
From the clinical veiwpoint RA can be divided into 3 stages: STAGE 1: Reversible soft tissue proliferation. STAGE 2: Controllable but irreversible soft tissue destruction and early cartilage erosions. STAGE 3: Irreversible soft tissue and bony changes.
SIGNS AND SYMPTOMS
SYSTEMIC MANIFESTATIONS: Morning stiffness lasting more than three months Anorexia, weight loss and fatigue are +nt during early stages
ARTICULAR FEATURES: RA is marked by a bil and symmetrical pattern of jt involvement. Clinically pt +nt with immobility and cardinal features of infl. Arthralgia O/E crepitation which is audible, palpable Weakness of ms, lig, tendons leads to deformities Fibrous ankylosis
INDIVIDUAL JOINT INVOLVEMENT: CERVICAL SPINE: Atlanto axial and mid cervical region are commonly affected, specially at c1-c2 level Subluxation is common As spine is involved: neck pain, root pain, radicular pain and some time UMN features and vascular symptoms
TEMPOROMANDIBULAR JT: Inability to open the mouth fully Approximation of upper and lower teeths may be altered CRICOARYTENOID JT: Not commonly involved Hoarseness of voice and can lead to stridor
SHOULDER AND SHOULDER GIRDLE: GH, SC AND AC jts are involved Degeneration, pain and loss of ROM Scapular thoracic immobility Capsule and lig weakness Sh jt eventually becomes unstable Bursitis and tendinitis may result
ELBOW: Inflammation, capsular and lig distension, jt surface erosion may lead to instability and irregular/ catching mts Flexion contracture is common Ulnar nerve entrapment at its groove
WRISTS: Pain Dorsal subluxation of distal end of ulna puts the ext tendons of 3,4 and 5 fingers in danger Early synovitis between carpal bones and ulna leads to flexion contracture which ultimately diminishes the ind¶s ability to execute power grasp Chronic infl of proximal row of carpals can lead to volar subluxation of wrist and hand on radius
Chronic infl leads to the loss of radial lig. Support and destruction of ECU and the fibro cartilage on the distal side of the ulna. The attachment of this restraining structures allows the proximal carpals to slide down the distal radius towards the ulna, creating the radial deviation of the distal raw of the carpals in the wrist relative to the 2 bones of the forearm where normally there are 5 to 10 degree of ulnar deviation. Stenosing tenosynovitis of the first dorsal compartment of the wrist (DQ) ,may also occur.
HAND: MCP JTS: Soft tissue swelling is common The volar subluxation and ulnar drift of MCP mainly due to laxity of capsule and lig and pulley supporting tendons at MCP which results in BOWSTRING effect This results in volar pull on proximal phalanx. At the same time as the pully loosens the flexer tendons are able to slip sideways typically in ulnar direction. And thus the deformity of ulnar drift with volar subluxesion begins. The tendons of ED also can slide ulnary and contribute additional deformity.
Radial deviation of carpals will furthure enhance MCP ulnar drift as the phalanges try to componset for the loss of normal ulnar deviation at the wrist. This is known as ZIG-ZAG effect. There are additional predesposing factors that contributes to this deformity: The heads of the metacarpals are normally sloped so more ROM in ulnar deviation than radial deviation. In many normal activities the fingers are pushed in ulnar direction by the external forces.
1 2 3
PROXIMAL IP JOINT: Swelling of proximal IP joints produces a fussiform or SAUSAGE like appearance in fingers. There are two characteristic deformities seen at PIP Joints. SWAN-NECK DEFORMITY:PIP hyper extension and DIP flexion. This arises in three distinct ways, depending on the site of initial involvement Initial synovitis of the MCP Volar capsule of the PIP is stretched in the later bands of FDP move dorsally. Rupture of ED at its insertion on DIP.
BOUTONNIERE DEFORMITY:DIP extension with PIP flexion. This results due to chronic synovitis around PIP which lengthens the tendon of ED at middle phalanx and lateral bands slide volary. Bouchard¶s nodes are found at PIP. DIP JOINTS: Heberden¶s nodes are common. Mallet finger deformity due to rupture of tendon of ED.
THUMB: Primary cause of deformity is synovial swelling. The fibers of dorsal hood mechanism over MCP is affected. The exact mechanism of thumb deformities depends on the particular combination of affected structures. Actual presentation depends on the site of initial synovitis, direction of imbalance of muscles and the integrity of the surrounding joint structures.
Type 1 deformity, consisting of MCP flexion with IP hyper extension without involvement of CMC joint. Type 2 deformity is assigned when the CMC is subluxed and the IP is held in hyper extension. Type 3 deformity, there is CMC subluxation and NCP hyper extension.
MUTILANS DEFORMITY/ OPERA GLASS HAND: Grossly unstable thumbs and severely deformed phalanges are indicative of this deformity. The transverse folds of skin of thumb and fingers resembles a folded telescope. Radiographic study of the bones of hand reveal severe bone resorption, erosion and shortening of MCP, PIP, RADIO-CARPAL and RADIOULNAR jts. The negative impact of this deformity on hand fun and ADL is significant.
HIP JOINT: Less commonly affected Severe infl destruction of the femoral head and acetabulam may push acetabulam into pelvic cavity , a condition known as PROTRUSIO ACETABULI.
KNEE JOINT: Most frequently affected Chronic synovitis results in distension of jt capsule, attenuation of lig and destruction of jt surface. Flexion-valgus deformity WIND-SWEPT deformity Backer¶s / popliteal cyst
ANKLE AND FEET: Chronic synovitis accentuates the natural tendency of the talus to glide medially and plantarward, resulting in pressure on the calcaneus and leading to hindfoot pronation. The spring ligaments is also stretched, flattening medial longitudinal arch. Calcaneal spur develops. As synovitis weakens the transverse arch, the metatarsals spread and a splayed forefoot/splayfoot may developed.
Metatarsalgia Hallux Valgus and Bunion(A painful Bursitis over the medial aspect of first MTP joint) When volar subluxation of the MTP combines with flexion of PIP and hyper extension of DIP, this condition is referred to as Hammer toes.
The MTPs may also exhibit volar subluxation of metatarsal head with flexion of PIP and DIP joints, known as COCK-UP or CLAW TOES. As the capsules and intertarsal ligaments are weakened and stretched, the proximal phalanges move dorsally on the metatarsal head. Similar to conditions observed in the hand, the long toe extensors bowstring over the PIP joints while the flexors are displaced into the intertarsal spaces.
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