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Clinical Importance Of The Variants Of Rheumatoid Arthritis Relevant To Connective Tissue Disorders

Updated on February 13, 2014

A Patient With Connective Tissue Disorder


Felty's Syndrome


A General Overview

Rheumatoid arthritis is a connective tissue disorder which manifests in various clinical presentations. Before anything is discussed about it, we ought to know what a connective tissue disorder is.

Connective Tissue Disorders

Connective tissues contain mainly collagen, elastin and proteoglycan. Skin and tendons contain abundant collagen which is responsible for the bulk of the connective tissue.

The terms “collagen diseases” was coined originally to denote a group of disorders which showed common features such as multisystem involvement, fibrinoid degeneration of connective tissue demonstrable on histology and caused by immunological processes. Rheumatoid arthritis, systemic lupus erythematosus, polyarteritis, systemic lupus erythematosus, polyarteritis nodosa, progressive systemic sclerosis and dermatomyositis fall under this group. Since no consistent abnormality in the structure or composition of collagen is demonstrable in these cases, the term “Connective tissue disorders” seems to be more appropriate. Broadly, arthropathies may be divided into seropositive and seronegative based on the presence of demonstrable immunological markers in the serum, e.g. rheumatoid factor, antinuclear antibodies, etc. The seropositive arthropathies include rheumatoid arthritis, systemic lupus erythematosus, polyarteritis nodosa, progressive systemic sclerosis and dermatomyositis. The seronegative arthropathies include ankylosing spondylitis, enteropathic arthritis, psoriatic arthropathy, Behcet’s syndrome, Reiter’s syndrome and juvenile chronic polyarthritis.

Still Disease (Juvenile Chronic Polyarthritis


Sjogren’s syndrome


Variants of Rheumatoid Arthritis

The following are the variants of rheumatoid arthritis:

  1. Chronic juvenile polyarthritis (including Still’s disease)
  2. Felty’s syndrome
  3. Sjogren’s syndrome, and
  4. Palindromic rheumatism.

Still’s disease (Juvenile Chronic Polyarthritis): This disorder occurs before the age of 16 years. Girls are affected more than boys. It differs from adult rheumatoid disease in that only one or a few joints are affected. Rash, fever, lymphadenopathy and splenomegaly are characteristic. Subcutaneous nodules are rare and rheumatoid factor is negative. Eye involvement with uveitis and keratopathy is more common. The treatment is along the same lines as for adult rheumatoid arthritis.

Felty’s syndrome: This is seen in older age groups. In addition to the classical features of seropositive rheumatoid arthritis, splenomegaly and neutropenia also occur. Splenomegaly may lead to hypersplenism. Splenectomy may have to be considered to correct hypersplenism in addition to treatment for the rheumatoid state.

Sjogren’s syndrome: This consists of kerato-conjunctivitis sicca, xerostomia and seropositive rheumatoid arthritis in 50-70% of cases. Keratoconjunctivitis sicca (dry eyes) is generally due to atrophy of the lacrimal glands. A filter paper applied to the conjunctiva fails to be wetted by tears and this is the basis of Schirmer’s test. Xerostomia (dry mouth) is due to atrophy of the salivary glands. Dryness may be seen in other areas such as the respiratory tract and vagina. Raynaud’s syndrome and non-thrombocytopenic purpura occur in some cases.

About 30-50% of patients with keratoconjunctivitis sicca and Xerostomia do not have clinical rheumatoid arthritis. These cases are known as “sicca syndrome”. Systemic lupus erythematosus, polyarteritis nodosa, progressive systemic sclerosis and polymyositis may all be associated with “sicca syndrome”.

In Sjogren’s syndrome, systemic steroid therapy may be indicated. Symptomatic relief to the eye is obtained with methylcellulose eye drops. Salivary secretion can be stimulated with lemon juice. In Sjogren’s syndrome, gold salts therapy is generally contra-indicated due to the high incidence of allergic reactions.

Pallidromic Rheumatism: In this condition, repeated attacks of joint pains, redness and swelling occur. These last for a few days and subside without any residual lesions. The ESR is raised during the attacks and remains high even during the intervals. After varying periods of time, typical rheumatoid arthritis supervenes in many cases, whereas spontaneous remission occurs in some.

© 2014 Funom Theophilus Makama


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