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Special Part

Chapter 11. Diseases of Bones, Muscles, Connective Tissue

Special Pathology

Rheumatoid Arthritis

Rheumatoid arthritis (infectious allergic polyarthritis, infectious non­specific deforming polyarthritis) is a systemic disease of the joints, first and foremost of small joints. The incidence among women is higher than in men. Young and middle-aged individuals are usually affected. The in­cidence of the disease is rather high: according to various authors, from 0.8 to 5 per cent of the population are affected by the disease.

Aetiology and pathogenesis are unknown. The onset of the disease is usually associated with the presence of chronic infectious foci (streptococ-cal infection, unknown viruses, possibly mycoplasm). The disease is regarded as an infectious and allergic and is referred to the group of larger collagen diseases. The rheumatoid factor (antibodies to the Fc fragment of immunoglobulin and mainly to the class M immunoglobulin) and an­tibodies to DNA, collagen, and to the formed blood elements are regularly found in the blood of patients with rheumatoid arthritis. It is believed that the rheumatoid factor arises in response to the production of auto-antigens, the proteins of the affected synovial membrane of the joints. The reaction between the antigen and the antibody causes a progressive affec­tion of the joints with further development of pathological proteins (auto-allergens). Hereditary predisposition to the disease is also important.

Pathological anatomy. Osteosynovitis is characteristic of the initial period. Later the car­tilages and periarticular tissues are involved in the process. Fibrous-sclerotic changes develop which finally result in complete or incomplete dislocation of the joints, development of ankylosis and marked deformation of the joints (hence another name: deforming polyar­thritis). In addition to pronounced affections of the joints, connective tissue and vessels are also affected in various organs which makes it possible to regard rheumatoid arthritis as a systemic disease.

Clinical picture. Polyarthralgia with mostly symmetrical affection of minor joints of hands and feet, limitation of movements, which is especial­ly pronounced after prolonged inactivity, and progressive deformation of the affected joints are symptoms characteristic of the disease. But all other joints can also be involved. In some cases, the disease is monoarthritis. The onset of the disease is usually subacute, but it may be acute or prolong­ed Pain in the joints is especially severe in the morning; it decreases at rest. After a night sleep or prolonged inactivity, the movements are especially limited, and articulation is difficult (this symptom is explained by oedema of periarticular tissues). By the night, movements of the joints become easier. The patient may also complain of general fatigue, fever, indisposi­tion, weakness, and loss of appetite.

Inspection of patients with pronounced arthritic changes reveals specific deformation of the joints, their subluxation and ankylosis. The most typical signs of the disease are deviation of the hand in the ulnar direction (seal's fin deformity), flexion contracture of proximal and hyper-distension of distal interphalangeal joints (button-hole deformity), flexion contracture of the metacarpophalangeal joint with hyperdistension in the proximal and flexion in the distal joint (swan-neck deformity of the finger). Inspection is supplemented by palpation of the joints to determine their tenderness, the degree of limitation of active and passive movements.

The typical changes in the hand of patients with rheumatoid arthritis are a peculiar " visiting card" of the disease. It is an important diagnostic sign. Changes in the other joints are difficult to differentiate from similar changes occurring in arthritis of other aetiology.

Palpation reveals a greater or lesser degree of muscular atrophy (due to lack of exercise, i.e. atrophy due to inactivity and due to specific muscular affection). Sometimes firm rheumatoid nodules, 0.5-1.5 cm in diameter, usually mobile and not adherent to the surrounding tissues, can be palpated in subcutaneous connective tissue around the elbow, over the ulnar bone, Achilles tendon, and in the occipital aponeurosis.

Lymphadenopathy, spleno- and hepatomegaly are observed in juvenile rheumatoid polyarthritis. In 80 per cent of cases, rheumatoid arthritis oc­curs in the form of joint affections. The arthrovisceral form of the disease occurs less frequently. In this case, direct examination of the patients and laboratory-instrumental methods reveal changes specific for affections of various organs (subacute or chronic myocarditis, pleuritis, diffuse fibros-ing alveolitis, glomerulonephritis, or amyloidosis of various organs, e.g. kidneys, liver, etc., which attend rheumatoid arthritis).

Laboratory studies reveal increased ESR (to 50-60 mm/h), not infre­quently normochromic anaemia, and also positive non-specific biochemical tests showing the activity of inflammatory processes such as dysproteinaemia (hypergammaglobulinaemia, increased alpha globulins and fibrinogen in the blood serum), and high seromucoid and C-reactive protein in the blood. Detection of the rheumatoid factor in the blood serum and synovial fluid is a more specific laboratory test for rheumatoid arthritis.

X-raying of the joints reveals their specific changes: osteoporosis of bone epiphyses, narrowing and erosion of the joint slit, formation of microcysts in the epiphyses, osteophytes growing by the articulation sur­faces. Complete and incomplete dislocation, marked deformities of the joints, and also complete overgrowth of certain articular slits can be reveal­ed at later stages of the disease.

Special Part

Chapter 11. Diseases of Bones, Muscles, Connective Tissue

Course. The disease is chronic and progressive. In most patients, it is characterized by periodic exacerbations (provoked by infections, overcool-ing, etc.) and remission. The patient can die of a concurrent amyloidosis, affection of the vitally important organs (heart or renal failure), and also complications associated with prolonged (sometimes, uncontrolled) ad­ministration of strong medicinal preparations. These complications may be perforation of steroid ulcer of the stomach, or hypertonic and diabetic complications in prolonged use of glycocorticosteroids and their analogues.

Treatment. Therapy includes: (1) sanation of chronic infection foci (carious teeth, tonsillitis, sinusitis, etc.); (2) using non-steroid anti-inflammatory analgesics (acetylsalicylic acid, butadione, bruphen, and the like); (3) using chloroquine derivatives (delagil, plaquenil); (4) using glucocorticosteroids.and their analogues (prednisolone and the like) in grave cases; this should also be supplemented by local therapy (given into the joint cavity); (5) remedial exercises and physiotherapy (mainly ther-motherapy); (6) in individual cases, surgical treatment (synovectomy).

Prophylaxis. Prophylactic measures are not well developed. But it has been shown that timely diagnosis and sanation of chronic infection foci (sanation of the mouth, tonsils, ears, etc.) are very important.

Osteoarthrosis

Osteoarthrosis (deforming arthrosis) is a chronic dystrophy of the joints and periarticular tissues which causes deformation of the joints. The disease usually attacks aged and middle-aged women.

Aetiology and pathogenesis are not yet known. It is believed that osteoarthrosis is associated with metabolic disorders in the cartilage due to its premature ageing. General endocrine and metabolic disorders, chronic microtrauma of the joints, and hereditary predisposition are also impor­tant. Synovitis, the reactive inflammation, is secondary to irritation of the synovial membrane by articular detritus (minute grains of necrotized car­tilage). Compensatory irritation of the cartilage occurs also with formation of osteophytes; subchondral osteosclerosis develops.

Pathological anatomy. Dystrophic changes in the cartilage impair its elasticity. The car­tilage surface becomes dry, opaque and rough. In grave cases, the cartilage may be affected by necrosis and ulceration, deformation of the joint surface, formation of osteophytes, perichondral osteosclerosis, and finally secondary arthroses. Inflammation in the cartilage and periarticular tissues is usually mildly pronounced.

Clinical picture. The patient complains of pain during exercise (walk­ing, stepping on the affected leg) in the spinal column, the large joints of

the lower extremities, and in distal interphalangeal joints of the hands. The pain is abated at rest.

Deformation of the joints and swelling around them can only be reveal­ed in grave forms of the disease. Palpation can reveal mild tenderness of tissues surrounding the affected joint. Movements of the joints are difficult only at the last stage of the disease. It concerns mostly the hip joint (coxar-throsis): patients develop waddling gait due to difficult articulation of the bones. As the disease progresses, walking becomes impossible because of dislocation or marked deformation of the hip joint. Laboratory studies do not reveal any significant changes, except a mildly increased ESR. X-rays reveal narrowing of the joint slit, the presence of osteophytes, subcar-tilaginous osteosclerosis in combination with cyst-like areas of diminished density in the epiphyses, deformities of the joints of various degrees, and in some cases dislocation of the joint.

Course. The disease is chronic and gradually progressive. Dislocation of the damaged joint is the possible complication.

Treatment. Therapy includes prescription of (1) analgesic and anti-inflammatory preparations; (2) intra-articular injection of trasylol (in­hibitor of the proteases that are involved in the degenerative changes in the cartilage) and arteparon (to inhibit splitting of cartilage mucopolysac-charides); (3) remedial exercises and physiotherapy (mainly ther-motherapy).

Haemorrhagic Vasculitis

Haemorrhagic vasculitis is an immuno-allergic disease characterized by affection of the capillaries, minor blood vessels, with subsequent multiple haemorrhages. The disease was first described by Schoenlein in 1832 and then by Henoch in 1868.

Aetiology. Aetiology of the disease is uncertain, but it has been long noticed that haemorrhagic vasculitis often attends certain infectious diseases (influenza, tonsillitis, tuberculosis, etc.) or develops in the presence of hypersensitivity to some foods and medicines. Specific an­tibodies to endothelial cells of the vascular wall are found in the blood of patients with haemorrhagic vasculitis.

Pathological anatomy. There are multiple haemorrhages in the skin, the wall of the gastro­intestinal tract, and less frequently, in other organs. Histological studies of tissues in the zone of haemorrhage reveal affections of the capillaries and minor vessels, necrosis of their vascular wall, thrombosis, proliferation of the intima with narrowed lumen of the vessel, and focal perivascular infiltration.

Clinical picture. The disease is characterized by a sudden multiple haemorrhages in the skin, often at symmetrical points of the right and left

Special Part

part of the trunk and the extremities. The joints may be affected: this is at­tended by pain, limited movements, and swelling of the periarticular tissues. In the abdominal form of vasculitis, haemorrhagic lesions appear on the gastro-intestinal and peritoneal mucosa; the patient complains of severe pain in the abdomen, which is attended by bloody vomiting and stools; the belly is tensed, the general condition grave, like in the acute ab­domen syndrome. In certain cases, haemorrhagic vasculitis can proceed with affections of the kidneys (subacute or chronic glomerulonephritis) and other organs.

In mild cases, there are no changes in the blood, but severe haemor­rhage is attended by hypochromic anaemia. The thrombocyte and fibrinogen content of blood is normal. Blood coagulability, bleeding time and clot retraction time remain normal. The tourniquet and pinch tests, and also the Bittorf-Tushinsky symptom are positive in most cases.

Course. The disease may be acute and continue for several days or weeks, or it may be chronic, with periodic exacerbations. The patient may die of profuse bleeding or haemorrhage into vitally important organs; necrosis of the intestine and kidney affections can be other causes of death.

Treatment. Treatment should be symptomatic. Calcium chloride is given intravenously to cause a weak anti-allergic effect and to strengthen the vascular wall. Salicylates and butadion are given in cases with affected joints. Corticosteroids are effective. They decrease substantially the allergic response of the body. Dimedrol, diprazine and other antihistamine preparations are also efficacious.