Multisystem (Complex) Arthritis

by Dr. Vaijayanti Lagu-Joshi

Many of us know that arthritis is a disease of joints but very few of us know that few types of arthritis are not just restricted to joints but they are multi systemic. I will be sharing some information about such multi system arthritis like rheumatoid arthritis, collagen vascular diseases (SLE, Sjoglen's scleroderma, mixed connective tissue disorder) inflammatory myositis (Polymyositis, Dematomyositis) and vasculitis which are rare and complex.

Rheumatoid arthritis (RA) is inflammatory arthritis of small and large joints (like wrists, shoulders, knees, fingers, toes), dominantly seen in ladies ageing 40-60yrs. The disease can affect young females (20-40yrs) and elderly too. The disease activity leads to pain, swelling of joints, deformities as it advances. Not only joints but systems like skin, eyes, lungs, Kidneys, nerves can also be affected by the disease.

Collagen vascular disease is a group of autoimmune disorders, seen in young females. Systemic lupus erythematosus, (SLE) sclerodenma, mixed connective tissue disorders (MCTD) are few types of collagen vascular disease. They show systemic manifestations involving skin, blood vessels, lungs & kidneys, neurological system. The exact etiology of such illness is not known. Genetic predispositions plus some environmental factors like infections, stress, UV rays can lead to trigerring of immune system and formation of 'autoantibodies' giving rise to such complex arthritis.

Polymyositis and dermatomyositis are inflammatory disease of muscles (mainly proximal muscles of thighs, arms) presenting with myalgia, muscle weakness, with arthralgia, skin and other systemic manifestations.

Vasculitis is a group of autoimmune diseases with inflammation of blood vessels-large, medium & small sized arteries, venules with multi system manifestations. It is a rare group of disorders, can be primary or secondary to collage diseases or infections sometimes.

All these arthritis have characteristic "extra-articulas" presentations. Clinical history & examination play an important role in 'Picking Up' such complex arthritis. The manifestations vary is severity. It is a wide spectrum affecting hair to toes.

1) Hair - Hair loss is common complaint of all females but more so with inflammatory arthritis. Excessive hair loss, alopecia total or patchy is classically seen in SLE.

2) Skin -

3) Raynaud's phenomenon - On exposure to cold water / cold weather, patients notice blue / white discoloration of fingers & toe tips associated with numbness and as they warm up, the coloration is back to normal.
This is a classical feature of patient of scleroderma, MCTD, SLE. sometimes seen in RA due to small vessel vasculitis.

4) Eyes - RA produces dryness, congestion of eyes, sometimes complicating to scleral melting and ulcerations in eye. SLE, vasculitis can lead to optic neuritis or retinitis (inflammation of retina, optic Nerve) leading to vision loss.

5) Mouth - Mouth dryness seen in Sjogrens syndrome, RA is due to inflammation of salivaryglands with decrease in secretion of saliva. Recurrent stoma titis, glossitis is a feature of SLE. Mouth opening become impaired with skin tightening in patients of sclerderma.

6) Lymphadenopathy - Lymphnode swelling, painful / painless seen in neck, axilla can also be a manifestation of collagen disease.

7) Lungs - Collagen disease like SLE, Vasculitis can give rise to pneumonias, lung nodules which are non infective. RA, scleroderma can lead to interstitial lung diseases giving rise to chronic dry cough, breathlessness. Pleural effusions can occur due to inflammatory process.

8) Heart & Blood vessels - All inflammatory diseases like RA, SLE, vasculitis can lead to dyslipidemia (lipid profile derangement) due to multiple reasons like ongoing inflammation, obesity, lack of exercise, medications like steroids. Premature atherosclerosis (hardening of arteries), angina, infarcts are on rise in patients of complex arthritis.

9) Gastrointestinal system - Dysphagia, recurrent reflux disease, diarrhoea, malabsorption can be seen in disease like scleroderma. Vasculitic ulcers can lead to bloody diarrhoea.

10) Kidneys - SLE, MCTD, Vasculitis can lead to affection of kidneys in form of protenuria, glomerulonephritis, Leading to renal impairment.

11) Neurological - Peripheral nerves (nerves of extremities) can be affected in RA, SLE or vasculitis. Patient get variable sensory motor loss, numbness in extremities. In few SLE patients continous headache, meningitis, sudden episode of hemiplegia / paraplegia can occur if disease activity is uncontrolled.
Advanced RA patients can have atlantoaxial subluxation (unstability of cervical vertebrae) leading to cord compression & quadriparesis.

12) A part from all these systemic manifestations, unexplained fever, weight loss, fatigue, should raise an alarm towards complex arthritis.
All such extra articular or systemic manifestations make arthritis a complex issue. It is very crucial to diagnose such arthritis as early as possible.
A part from good clinical examination, a few laboratory tests like ANA, dsDNA, antibody panel screening, ANCA can give us diagnostic clues. Radiological tests like chest X-ray, Ultrasound, sometimes CT scan, MRI are needed for diagnosis and assessing extent of the problem. Once the extent of disease and target organs affected by arthritis are known, the treatment is aggressive. Most of the times these complex arthritis need potent, strong immunosuppressants like steroids, methotrexate, azoran. The doses, duration of medications depend on the disease activity and clinical expertise and strictly medical supervision is needed.
As patients, one should not get frightened by these complex arthritis but one should get educated about the disease. If treated at right time, in right way the disease are controllable and respond dramatically.