In its more aggravated forms diffuse scleroderma is one of the most terrible of all human ills. Like Tithonus to ‘wither slowly’ and like him to be ‘beaten down and marred and wasted’, until one is literally a mummy, encased in an ever shrinking, slow contracting skin of steel, is a fate not pictured in any tragedy.
SIR WILLIAM OSLER 1898
The inflammatory connective tissue diseases and the related vasculitides are groups of disorders that are difficult to classify because their causation is generally unknown. They all cause joint and soft tissue inflammation and multiple other possible manifestations that create diagnostic difficulties.
These are disorders in which the body’s immune system damages its own specific organs or systems. The connective tissue diseases are a classic subgroup of autoimmune disease. Rheumatoid arthritis is the most common autoimmune disease. Organ-specific autoimmune diseases include diabetes type 1, Hashimoto thyroiditis, pernicious anaemia, IgA glomerulonephritis, Graves disease, autoimmune hepatitis and myasthenia gravis.
It is convenient to consider a working classification of joint pain (see TABLE 32.1) that includes apparent joint pain (arthralgia), as some of the inflammatory disorders cause problems in the soft tissues around joints (e.g. giant cell arteritis and hydroxyapatite crystallopathy of the tendons around the shoulder joint).
Table 32.1A classification of rheumatological pain ||Download (.pdf) Table 32.1 A classification of rheumatological pain
|Hyperacute (red hot) joints ||Crystals ||Urate: gout |
| || ||Calcium pyrophosphate; hydroxyapatite |
| ||Pus ||Example: staphylococcal septic arthritis |
|Inflammation of joints ||Symmetrical ||Example: rheumatoid arthritis |
| ||Asymmetrical ||Example: spondyloarthropathies |
|Non-inflammatory joint disorder ||Typical ||Primary osteoarthritis (e.g. in hands) |
| ||Atypical ||Example: post-trauma, haemochromatosis |
|Joint and soft tissue inflammation ||Connective tissue disorders ||SLE |
| || ||Scleroderma |
| || ||Polymyositis/dermatomyositis |
| || ||Polyarteritis nodosa |
| ||Vasculitides ||Giant cell arteritis |
| || ||Polymyalgia rheumatica |
|Non-articular (soft tissue) inflammation ||Generalised ||Examples: fibrositis, fibromyalgia, polymyalgia |
| ||Localised ||Examples: plantar fasciitis, epicondylitis |
Vasculitis is, in fact, a condition common to the connective tissue disorders and to the so-called vasculitides (see TABLE 32.2).
Table 32.2List of connective tissue disorders and systemic vasculitides ||Download (.pdf) Table 32.2 List of connective tissue disorders and systemic vasculitides
|Connective tissue disorders |
|Rheumatoid arthritis |
|Systemic sclerosis/limited scleroderma |
|Mixed connective tissue disease |
|Sjögren syndrome |
|Raynaud phenomenon (including Raynaud syndrome) |
|Systemic vasculitides |
|Large vessel predominantly: |
|Medium vessel (mainly affects visceral vessels): |
|Small vessel (mainly): |
immunoglobulin A vasculitis (Henoch–Schönlein purpura)
|Antineutrophil cytoplasmic antibody (ANCA) associated: |
A major concern to all is that the diagnosis of these conditions is ...