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Background: Systemic lupus erythematosus (SLE) is an autoimmune disease involving multiple organ systems that is defined clinically and associated with antibodies directed against cell nuclei. Its multisystem manifestations and attendant complications from use of immunosuppressive agents make the diagnosis and management of this entity challenging.
Pathophysiology: Autoantibodies, circulating immune complexes, and T lymphocytes all contribute to the expression of disease. Organ systems affected include dermatologic, renal, central nervous system (CNS), hematologic, musculoskeletal, cardiovascular, pulmonary, the vascular endothelium, and gastrointestinal. The revised criteria for SLE must include 4 of the following:
*Presence of anti-DNA antibodies (double stranded
or native) - highly specific for SLE; found in most patients with
active renal disease (90%) and in patients with active disease but without
renal involvement (50%); titres reflect disease activity; these do
not occur in drug-induced LE.
*Antibodies against extractable nuclear antigens.
These antigens include nuclear ribonuclear protein (nRNP) and nuclear nonnucleic
acid glycoprotein (Smith {Sm} antigen). The latter appears to be
highly specific for patients with SLE; present in 25% - 30% of patients
with SLE
*Positive test for LE cells (70% - 85%) specific
for SLE but not as sensitive as the presence of antinuclear antibodies;
LE cells may be seen in synovial, pleural, and pericardial fluid
*Presence of circulating immune complexes - indicates
active disease.
Decreased serum complement (75%) - reflects consumption
by immune complexes and implies active disease
False-positive nontreponemal test for syphilis (15%
- 20%) - reflects increased immunoglobulins
Haematologic Findings
Mild normocytic anaemia (50% - 80%) - usually chronic
disease type; occasionally there is an autoimmune haemolytic anaemia
with a positive direct Coombs' test
Moderate leukopenia - autoimmune mechanism
Lymphocytopenia - due to lymphocytotoxic antibodies;
this is the most frequent initial laboratory finding
Thrombocytopenia - autoimmune mechanism; often
reflects disease activity
Increased fibrin split products - seen in lupus nephritis
Plasma Proteins
Decreased serum albumin (50%-60%) - reflects chronic
disease or loss of albumin in urine in the nephrotic form of lupus nephritis
Increased sedimentation rate and C-reactive protein
- usually reflects disease activity
Urinalysis
Haematuria, cellular casts, and proteinuria reflect
active lupus nephritis.
Synovial Fluid
Low white blood cell (WBC) count (<3000/ul);
predominant lymphocytes LE cells and antinuclear antibodies
Decreased complement
Spinal Fluid
Findings of aseptic meningitis
*Biopsy of Kidney and Skin
Immunogluorescent studies show deposits of immunoglobulins
and complement.
*High titre of antinuclear antibody, which usually
has a speckled pattern
*Very high titre of antibody to extractable nuclear
antigen
*High titres of antibody to nRNP, frequently >1:100,000.
This is the chracteristic serologic finding, occurring in 95% - 100% of
patients with this disorder.
Absent antibody to nuclear Sm antigen
Absent antibody to DNA
Normal serum complement
Increased serum y-globulin (2 g/dl - 5 g/dl)
in 75% of patients
Positive rheumatoid factor in 50% of patients
Increased sedimentation rate reflects active inflammation.
Moderate anaemia, occasional
Moderate leukopenia, occasional
Increased SGOT (AST), aldolase, and CPK reflect muscle
involvement.
Reactive arthritis, also called
Reiter's syndrome, is the most common type of inflammatory polyarthritis in
young men. It is sometimes the first manifestation of human immunodeficiency
virus infection. An HLA-B27 genotype is a predisposing factor in over two
thirds of patients with reactive arthritis. The syndrome most frequently
follows genitourinary infection with Chlamydia trachomatis, but other
organisms have also been implicated. Treatment with doxycycline or its analogs
sometimes shortens the course or aborts the onset of the arthritis. Reactive
arthritis may also follow enteric infections with some strains of Salmonella or
Shigella, but use of antibiotics in these patients has not been shown to be
effective. Reactive arthritis should always be considered in young men who
present with polyarthritis. Symptoms may persist for long periods and may, in
some cases, cause long-term disability. Initial treatment consists of high
doses of potent nonsteroidal anti-inflammatory drugs. Patients with large-joint
involvement may also benefit from intra-articular corticosteroid injection. (Am
Fam Physician 1999;60:499-507.)
Rheumatoid is a medical inflammatory disease of the lining of the joint rather than a wear and tear problem.
A typical joint in the body is made up of 2 bones that move against each other. These two bones are held together by ligaments and a sheath that surrounds the entire joint like the walls of a balloon or bag. This sheath or wall is called the capsule of the joint. The inner lining of this joint wall or capsule is called the synovium and is made of a special tissue that does many things including producing a special joint-lubricating nutrient fluid called synovial fluid.
In rheumatoid arthritis, the synovium of the joint becomes aggressive and inflamed. It enlarges and erodes (eats into) the neighboring bones, ligaments and joint cartilage coating damaging the joint's smooth surfaces. The end result is similar to osteoarthritis in that the joint surfaces are destroyed and painful, although rheumatoid arthritis starts differently and has additional problems both in the joint and elsewhere in the body.
ANKYLOSING SPONDYLITIS - Disussion:
- patients with ankylosing spondylitis are prone to C-spine fracture following minor trauma;
- acute neck pain in a patient with ankylosing spondylitis is usually caused by a fracture through the ankylosed spine;
- site of involvement is most commonly near cervicothoracic junction (or C6-7);
- the fracture may extend thru the disc space or may extend thru all columns;
- there may be significant change in neck alignment;
- epidural hematoma may contribute to spinal cord injury;
POLYMYALGIA RHEUMATICA Polymyalgia Rheumatica and Giant Cell Arteritis
Polymyalgia rheumatica is a condition that affects people over the age of 50 and more commonly women. The words mean "rheumatic pain in many muscles".
It is the name given to a condition that results in severe stiffness and pain in the muscles of the neck, shoulders, lower back, buttocks and thighs. The muscles of the forearms, hands, calves of the legs and the feet are usually not affected. In addition people affected by polymyalgia rheumatica may experience weariness and loss of energy, night sweats and fevers, weight loss and depression. These symptoms are often worse in the morning hampering early morning functions such as rising from bed, dressing and washing. Often tasks cannot be undertaken until the afternoon.
Often the onset is dramatic. A previously fit person can retire to bed in good health and awake to find themselves "cast" with severe stiffness and pain. In other cases the onset can be more gradual. It may start with some stiffness in one group of muscles (eg the shoulders) and spread over the next few weeks to involve the back, buttocks and thighs. If it is not treated, the severity and site of the symptoms may vary over several months.
The cause of polymyalgia rheumatica is not known. It does only occur in older people and it may be triggered by virus which alters the way in which the body's immune system works. It responds well to treatment although for some people it may develop into a form of rheumatoid arthritis.
OSTEOMYELITIS
Osteomyelitis is an infection of the bone that occurs most frequently in the lower extremities. Most commonly, it develops after severe local trauma
with an associated open fracture. The adjacent soft tissue structures are injured together with the bone, and can form a poorly vascularized and
scarred tissue bed. Simple debridement and antibiotic therapy are often unsuccessful in treating lower extremity osteomyelitis. As a result, patients
frequently present after multiple failed treatments and with resistant or polymicrobial bacterial infection.
Increased serum alkaline phosphatase, marked - this
characteristic finding is the result of excessive new bone formation, which
reflects the activity of the disease; a rapid rise occurs if osteogenic
sarcoma develops
Increased urinary hydroxyproline - this is the result
of accelerated bone collagen resorption, breakdown, and excretion
Increased serum alkalkine phosphatase - this reflects
new bone formation by the tumour. It is usually not more than two
to three times higher than normal for the age of the patient.
Increased alkaline phosphatase after surgical removal
of the tumour suggests spread or recurrence of the tumour.