Syndromes of Rheumatoid Arthritis | Rheumatology

In this article we will discuss about the syndromes of rheumatoid arthritis, mostly seen in humans.

1. Ankylosing Spondylitis (A.S):

Progressive stiffening and finally fusion of the axial skeleton brought about by a chronic inflammatory arthritis usually of the sacroiliac joints and spine.

Young men between 20 and 30 years are most commonly affected and the m-f ratio is 9 : 1, although incomplete variants of the classical disease may equalise the ratio . Certain ethnic groups show prevalence (e.g. Haida Indians), and so does first degree relatives of patients with psoriatic, arthritis, inflammatory bowel disease, Reiter’s syndrome and of course ankylosing spondylitis itself. 90% of patients are HLA-B27 positive, and faecal carriage of Klebsiella species (especially during exacerbations) may be aetiologically significant. Associated prostatitis, may not however show any infective organism in the prostatic fluid.

The pathological hallmark of the disease is the presence of “syndesmophytes” which is new bone formation at the function of vertebral bodies and annulus fibrosus of intervertebral discs, due to the heading of enthesopathic foci, leading to bony ankylosis of the spine (bamboo spine of X-ray). The enthenopathy comprises multiple foci of inflammation containing lymphocytes and plasma cells at ligamentous attachments to bone causing bony erosion.

Low back pain and stiffness of insidious onset and chronic nature with exacerbations and remissions, occasional radiation of pain to thigh or buttock, and early morning stiffness are characteristic of A.S., although some may present with acute symptoms resembling lumbar disc prolapse.

There is pain on sacroiliac compression, all movements of the lumbar spine are restricted. While symptoms or signs of nerve root compression is significantly absent, cases presenting with symptoms referable to thoracic or cervical spine usually display evidence of previous involvement of the costovertebral may cause chest pain aggravated by breathing. Tenderness over prominences such as iliac crest, ischial tuberosity and greater trochanter, Achilles tendinitis and plantar fascitis are characteristic features.

Rheumatoid factors — negative

Antinuclear factors — negative

Synovial fluid complement levels — not reduced. Routine blood examination usually displays raised ESR.

Although early X-rays may be normal, eventually irregularity and marginal sclerosis progressing to fusion is seen, especially in lower parts of joints. Syndesmophyte formation, ossification of anterior longitudinal ligament leading to “squaring” of vertebrae, erosion and sclerosis and anterior corners of vertebrae and factal joint changes are all common and typically, progressive ossifications lead to the “Bamboo spine”, with erosive changes in symphysis pubis, ischial tuberosities and peripheral joints. Osteoporosis and atlanto axial dislocation may occur.

Negative X-rays may prompt a radionuclide bone scan but this is a non-specific test showing blood flow and turnover of bone.

D/D — Prolapsed lumbar vertebral disc, trauma causing low back pain and other spondyloarthrities are to be considered in differential diagnosis. Degenerative joint diseases display no systemic manifestations and occur in elderly.Benign senile hyperostic spondylosis may resemble, but here the osteophites extend horizontally (not vertically from the edges of the vertebral bodies and while the sacroiliac joints is intact the disc spaces are often narrowed. Peripheral joint involvement, such as the M.T.P. and great toe may be confused with gout pseudogout, infective arthritis or any other seronegative inflammatory arthritis.

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2. Joint Disorders Reiter’s Syndrome:

Reiter’s syndrome is suspected if there is a tetrad of arthritis, non-specific urethritis, mucocutaneous lesions and conjunctivitis, especially if it occurs after attack of enteric infection with samlonella chlamydia, shigella or yersine organisms. Sometimes it is called “Reactive arthritis”.

Patients coming to the venereal disease clinic suffering from arthritis are sometimes diagnosed as Reiter’s syndrome but these may be cases of gonococcal arthritis and not Reiter’s syndrome. Male to female ratio is 50 : 1, but the male preponderance may be due to the fact that women often ignore the symptoms of urethritis and the disease is overlooked. Subjects with HLA-B27 positive histocompatibility antigen, have greater susceptibility in contracting this disease.

An acute onset with oral lesions, urethritis, conjunctivitis, and arthritis appear simultaneously about one to three weeks after dysentery or sexual exposure. Systemic symptoms like fever, weight loss and vasomotor changes in the feet are often present, chiefly knee and ankles are involved.

Arthritis may subside spontaneously but in about 15% cases there is recurrence without any relapse of dysentery. Sometimes low back pain and stiffness due to sacro-ileitis may occur.

Serum rheumatoid factor and antinuclear factors are negative but in 75% of cases, HLA, B-27 may be detected.

Mucocutaneous lesions consist of stomatitis, and keratoderma blenorrhagica.

Sometimes carditis may occur leading to aortic incompetence. However, spontaneous remission may occur though tendency to relapse is well known.

Reduction of joint space, erosive changes, and periarticular osteoporosis are noted especially when the disease is protracted or there is recurrence.

Periostitis is present specially in metacarpal bones and in phalanges. Characteristic “fluffy” calcaneal spurs may be seen. The sacroileitis may be present as in spondylitis but isolated bony spurs and paravertebral ossification is present in the spine unlike ankylosing spondylitis.

Mainly symptomatic and supportive. In acute stage rest and use of nonsteroidal anti-rheumatic drug is resorted to, as in rheumatoid arthritis. Aspiration of joints having effusion and injections of steroids may also be required. Systemic steroids are very occasionally necessary. Anterior uveitis may be a medical emergency and it should be treated by topical sub-conjunctival or systemic corticosteroids.

Rarely severe arthritis and intractable keratoderma blenorrhagica justify the use of cytotoxic drugs. Tetracycline is the drug of choice for the non-specific urethritis but the drug does not affect other symptoms nor does it alter the course of the disease.

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3. Behcet’s Syndrome:

This syndrome was described by the Turkish dermatologist Dr. Behcet. Recurrent oral and genital ulceration associated with uveitis, sero-negative recurrent arthritis are the characteristics of this disease. The major criteria include inflammatory arthritis of large joints, epididymitis, intestinal ulceration, thrombophlebitis and neuropsychiatric symptoms.

If all major criteria are present, it is said to be “complete” syndrome, while if only three major criteria are present it is incomplete. The knees, ankles, wrists and elbows are most frequently involved in arthritis.

Sometimes eyes are badly involved leading to blindness. Uveitis, keratitis, hypopyon and even optic neuritis may occur, CNS involvement consists of encephalitis, cranial nerve palsies and spinal cord lesions. There may be leucocytosis and high ESR.

When arthritis is the dominant presentation treatment is similar as in the case of rheumatoid arthritis. But antibiotics may be necessary for infected ulcerations. Corticosteroids and immuno­suppressive therapy are used in severe and serious cases. Follow-up is essential as there is tendency to relapse and remission.

4. Whipple’s Disease:

The disease is manifested by diarrhoea, pain in the abdomen, arthritis and malabsorption in the gut. Anaemia, gastrointestinal bleeding, lymphadenopathy and fever may occur. Usually there is non-erosive and asymmetrical oligoarthritis, but occasionally some patients develop spondylitis.

Very rarely extraarticular features like meningo-encephalitis, peripheral neuropathy, pericarditis or pleurisy may develop.

ESR is raised, often very high, during the acute phase may persist at high level long after symptoms have subsided.

PAS positive macrophages in small intestinal biopsy confirms the diagnosis of Whipple’s disease. Certain rod-shaped organisms which are as yet not identified have been detected by electron microscopy in the lamina propria and synovial membrane of affected persons.

Treatment consists of supportive measures and use of tetracycline 1.0 to 2.0g per day, which may have to be given for a few months.

Anaemia and hypoproteinemia will need appropriate therapy including blood transfusion.

Enteropathic Arthritis:

Ulcerative colitis and Crohn’s disease display two different types of sero-negative inflammatory arthritis.

Sacroileitis is seen in patients with ulcerative colitis of Crohn’s disease along with ankylosing spondylitis, but they are not affected by the intestinal disease process and pursue an independent course. High incidence of HLA-B27 positive cases is seen.

Entropathic Synovitis:

This is a non-erosive oligo-arthritis, acute and migratory in nature usually affecting the weight bearing joints; sometimes wrists and small joints of the fingers and toes may be involved. Arthritis is primarily dependent on bowel disease, e.g. ulcerative colitis and Crohn’s disease.

Arthritis often occurs in association with aphthous mouth ulcers and erythematous rashes which disappear” following total remission of ulcerative colitis. There is no specific treatment for arthritis, as arthritis settles down bowel disease is controlled.

5. Juvenile Chronic Arthritis:

(Juvenile Chronic Arthritis or Still’s Disease):

Juvenile chronic arthritis may start with severe lymphadenopathy, hepatosplenomegaly, pleurisy, pericarditis and high intermittent fever along with systemic disturbances. There may be a striking degree of retardation of growth, along with weight loss and often transient erythematous rash appearing with rise of temperature.

Leucocytosis, anaemia and raised ESR are noted. Rheumatoid and antinuclear factors are, negative. Usually most children improve after a few months but some cases have recurrent attacks which may progress to a severe chronic polyarthritis.