Rheumatic Diseases

JUVENILE IDIOPATHIC ARTHRITIS[JIA]

The term arthritis refers to the presence of joint swelling and/ or pain with movement. Joint pain alone is arthralgia.

JIA refers to arthritis in a person aged less than 16 years, for a duration of more than 6 weeks, and of unknown cause.

Therefore JIA is a diagnosis of exclusion, arrived at after all other possible known causes have been excluded.

To be excluded such as malignancies, connective tissue disorders, infections, autoinflammatory disorders, joint hypermobility syndrome, etc.

JIA is heterogenous group of diseases, divided into 7 different types, depending on the number of joints involved, the presence of systemic symptoms, and the presence/ absence of rheumatoid factor.

If not recognized and treated in time, arthritis leads to permanent deformities and associated disability and poor health related quality of life. Therefore, should you suspect arthritis in those under age 16 years, get them to be seen by a rheumatologist, or pediatrician/physician. 

SYSTEMIC  JIA

Systemic type of JIA usually begins in early childhood, mostly ages 0 to 5 years, but can occur at any age up to 16 years. Another name for it is stills disease, and when it first occurs at age beyond 16 years it is called adult onset stills disease.

It is characterized by the presence of features of systemic inflammation such as persistent daily spikes of high fever, usually 39 degrees centigrade and above. Other features include a fleeting pink skin rash, myalgia, sore throat, arthralgia/arthritis, enlarged liver and spleen, enlarged lymph nodes, and serositis[ inflammation of membranes covering the heart, the lungs and peritoneum].Serositis may be accompanied by collection of variable amounts of fluid around these organs that may be life threatening.

Generally, there are three clinical disease course patterns so far recognized in systemic JIA. These are monophasic, polycyclic, and persistent patterns. In the monophasic type the disease occurs as a one off phenomenon, it is recurrent attacks with resolutions in between in polycyclic course, and there is no complete resolution of disease from the onset/first attack in persistent course.

Chronic systemic inflammation may also lead to accumulation of a substance called amyloid in various internal organs including the kidney, the liver, gastrointestinal tract, and the heart, this may lead to failure of the involved organs. 

Mesenteric adenitis can be painful, and at times can be misdiagnosed as acute abdomen leading to inappropriate surgical interventions!!

The other life threatening  complication of this disease is Macrophage activation syndrome which requires emergency management in an ICU setting.

 UVEITIS IN JIA

 JIA is the commonest cause of this disease in children, most commonly pre-school age. It refers to inflammation inside of the eye, which can be anterior/front, intermediate/middle or posterior/at the back. The most common presentation in JIA is anterior uveitis, followed by intermediate. Pan-uveitis [ front through to back] also occurs.

If not recognized and treated early leads to complications such as cataract and glaucoma. Children diagnosed with JIA must therefore be reviewed by an ophthalmologist at baseline, and regularly thereafter. Uveitis is however rare in systemic JIA.

Rheumatic Diseases

The following is a list of some of the rheumatic diseases we treat.

  1. Osteoarthritis.
  2. Gout.
  3. Rheumatoid arthritis.
  4. Lupus.
  5. Seronegative inflammatory arthritis.
  6. Spondyloarthritis.
  7. Scleroderma.
  8. Inflammatory myopathy.
  9. Sjogren's syndrome.
  10. Polymayalgia rheumatica.
  11. Back pain.
  12. Other regional pain syndromes.
  13. Bursitis.
  14. Vasculitis.
  15. Pseudogout.
  16. Raynauds disease.
  17. Sarcoidosis.
  18. Behcets disease.
  19. Mixed connective tissue diseases.
  20. Overlap syndromes.
  21. Undifferentiated connective tissue disorders.
  22. Relapsing polychondritis.
  23. Autoinflammatory disorders.
  24. Antiphospholipid antibody syndromes.
  25. Juvenile Idiopathic arthritis.
  26. Erythema nordosum.
  27. Lipodermatosclerosis.
  28. Fibromyalgia syndrome.
  29. Osteoporosis and other diseases of bone.
  30. Pain management.
  31. Infection related arthritis, eg lyme, HIV, reactive, other viral arthritis.
  32. Septic arthritis.
  33. Lymphoedema.

How do I know if I have Arthritis?

It is important to suspect that you have arthritis if you experience joint pains and, more importantly, joint swelling in one or more of your joints. Arthritis is defined as joint pains and swelling but it may initially present as joint pains without swelling. 

Why is it important to SUSPECT ARTHRITIS AND ACT ON YOUR SUSPICIONS PROMPTLY? Because arthritis, if not diagnosed and managed early, is progressively damaging to the joints and physically disabling to the patient.

However since joint pains, and even swelling, can have causes other than arthritis, it is important to make an appointment with a rheumatologist early so that the true cause of your symptoms can be determined through a detailed history, clinical examination and relevant investigations.

The correct line of management can thereafter be discussed and adopted. 

Yes I have arthritis, what next?

With the confirmation that one has arthritis comes the decision as to which particular arthritis it is. This is because there are different causes and types of arthritis, which will also determine how each is treated.

Arthritis can be classified as mechanical or inflammatory depending on the underlying cause. 

 

OSTEOARTHRITIS

This is the commonest of all arthritis and the prototype of the mechanical category. It is normally caused by degenerative changes in the involved joints as a result of advanced age. But it can occur earlier as a result of persistent joint overuse, or even prior joint damage.

The most commonly involved joints are the knee and hip joints, and hands, but other joints may be involved, including the spinal column. When it involves the spinal column it is commonly called spondylosis.

Obesity, or more than ideal body weight, is a major factor in the initiation and rate of progression of joint damage in the case of knee and hip osteoarthritis, but obesity is also associated with hand osteoarthritis.

Osteoarthritis is also associated with a feeling of weakness/giving way/loss of balance in the affected joints, especially if the knees are involved.

Treatment will therefore, apart from pain medication, involve majorly physical therapies that improve joint strength, and body weight reduction in knee and hip osteoarthritis.

When joint damage is advanced, and pain and physical disability can not be controlled adequately by medication and physiotherapy, then joint replacement surgery is available option for hip, knee and shoulder joints, and arthroplasty or arthrodesis for some of the other joints.

For more information, advice, and treatment of osteoarthritis consult your rheumatologist.

 

GOUT

 This is the most common form of inflammatory arthritis, although the prevalence will vary from one region of the world to another.

It results from excess of uric acid in the blood of affected persons[hyperuricemia], which then gets deposited in the joints resulting in acute inflammation of the affected joint, with attendant severe/excruciating pain, swelling and warmth in the affected joint

 Acute attack of gout usually occurs in a single joint at a time, most commonly in the big toe, but other joints may be involved. The pain is typically severe and disabling and, although it may resolve on its own after several days or weeks, nobody dares to wait for that to happen because of the severity of the pain!!.

After resolution of an acute attack there may be an asymptomatic interval of varying duration, but recurrent flare ups are likely to occur unless the underlying cause is addressed.

Elevated levels of uric acid may also lead to deposition of uric acid crystals in the kidneys, and as nodules, called tophi[tophaceous gout], in joints and soft tissues in various parts of the body. Such tophi may with time lead to destruction of the adjacent joints.

The elevated serum uric acid levels is mainly as a result of reduced elimination of uric acid by the kidney, caused by some medications  and/or variants in the uric acid transporters in the kidneys of affected patients. The other cause of hyperuricemia is excessive production of uric acid from foods such as red meat, sea food, beer, high fructose corn syrup, other sweetened soft drinks,  and also from endogenous sources such as breakdown of tumor cells.

Gout is common in men after the age of puberty, but it is rare in women of reproductive age. Gout is associated with higher incidence of other metabolic diseases in the same patient, such as obesity, type II diabetes mellitus, hypertension, and high cholesterol levels. These other diseases should therefore be looked for in any patient presenting with acute attack or the other manifestations of gout.

Unless caused by certain removable conditions such as medications and breakdown of tumor cells, gout is a chronic lifelong condition. Part of treatment therefore involves changes in certain aspects of lifestyle and diet to help maintain serum uric acid levels in the normal range and prevent or reduce incidence of acute attacks.

Such modifications include limiting alcohol consumption, limiting consumption of red meat, sea food, and high fructose corn syrup. A weight loss program is also recommended, which includes physical exercise, for the obese, together with other dietary changes relevant to those with co-existing hypertension and/or pre-diabetic/diabetic states. This calls for a consultation with a nutrition expert.

Most important!! avoid prolonged use of over the counter medications and consult a rheumatologist early at disease onset to avoid developing severe complications from misuse of drugs and poorly controlled gout. Your rheumatologist will discuss with you in detail about gout, associated metabolic conditions, and their management, including the use of medications.

 

RHEUMATOID ARTHRITIS

This is a chronic autoimmune systemic inflammatory disease, which commonly presents as arthritis in adults over the age of 16 years. It affects women more frequently than men.

The most common clinical presentation is with multiple symmetrical joint pains and swellings. There is associated stiffness of the affected joints for prolonged durations, especially on waking up in the morning and more so in cold weather. In more severe forms of the disease nodular lesions may occur adjacent to the joints, especially in the posterior aspects of the elbow joints, and also in some internal organs.

Most frequently involved are small joints of the hands and feet, the wrists, the ankle joints, but all the joints in the body may be affected, including the temporo-mandibular joint and the cervical spine, typically at the atlantoaxial joint.

The multiple joint pains, swelling and stiffness results in various degrees of physical disability depending on the severity of clinical presentation. If this is not diagnosed and competently managed early, within the first two months of onset, then destruction of the affected joints will progress, leading to joint deformities and associated permanent physical disability.

Rheumatoid arthritis may also involve the heart, causing pericarditis, pericardial effusions, and valvular disease, with attendant functional impairment and mortality.

Rheumatoid arthritis may involve the lungs in various ways, including associated interstitial pneumonia, pleural effusions, pulmonary nodules, which may result in increased mortality.

Apart from causing dryness in the eyes, it may also cause inflammation and redness, and even perforation, in the white part of the eye with resultant blindness.

Rheumatoid arthritis may also cause inflammation of blood vessels and damage to peripheral nerves. 

It is very important to consult with your rheumatologist early if you suspect rheumatoid arthritis. This will enable prompt diagnosis and initiation of appropriate treatment to avoid permanent joint damage, joint deformities, permanent physical disability, and other complications due to internal organ involvement in rheumatoid arthritis.

 

LUPUS

This is an auto-immune disease belonging to the group of connective tissue disorders. It has numerous ways of presenting and has therefore been referred to as a disease of a thousand manifestations. It can affect all age groups, but women are affected much more frequently than men.

The two main types of lupus are Systemic Lupus Erythematosus[SLE] and cutaneous lupus. There are various types of cutaneous lupus, and SLE, which is a chronic multisystem autoimmune disease, can involve the skin, the joints, the tendons, peripheral nerves, blood, blood vessels, and internal organs such as the kidneys, the brain, the lungs and its coverings, the heart and its coverings, the abdomen, and the eyes.

The clinical presentation of this disease in a patient is therefore protean, depending on which organ or tissue of the body is attacked, and in what way. Fever, weight loss, and night sweats, as general systemic symptoms, frequently occur.

Some of the commonest clinical manifestations therefore include skin lesions of various types, oral ulcers and classical for SLE is a facial or malar rash that is photo sensitive. Another common presentation is multiple joint pains and swellings, similar to the pattern in rheumatoid arthritis, but different in that no joint erosions occur in SLE. Yet joint deformities and associated physical disability can occur due to damage to tendons by SLE

Lupus disease of the kidneys is called lupus nephritis, and it presents usually with blood and protein in urine, but in severe cases can present in renal failure and/or fluid retention.

In the lungs SLE may present as lupus pneumonitis or interstitial lung disease, either of these can be so severe as to require respiratory support in HDU/ICU setting. The more common respiratory manifestation however is collection of fluid, of varying amounts, in between the lung coverings, termed pleural effusion. Large volume effusions can result in difficulty in breathing requiring surgical drainage for relief. At times characteristic chest pains result from inflammation of the pleura without much effusion.

Lupus may cause inflammation of the membranes covering the heart. Such inflammation, known as pericarditis, presents with chest pain around the heart, and may lead to fluid accumulation around the heart. Such fluid accumulation, known as pericardial effusion, if large, may compress the heart and prevent it from filling adequately and therefore endanger the life of the patient. Lupus may also cause inflammation of the heart muscle and heart valves.

Lupus may cause inflammation of the blood vessels, called vasculitis, which leads to bleeding in, or impaired blood supply to the tissues affected resulting in dysfunction and/ necrosis of such tissues. Symptoms and signs resulting will depend on the tissues involved, e.g skin lesions, peripheral nerve pains/weakness, stroke, abdominal pains/mesenteric ischemia, digital gangrenes etc.

Lupus may also cause various types of peripheral nerve disease presentations resulting into pains, disturbance of sensation, and at times weakness of the muscles supplied by the affected nerves.

Lupus of the central nervous system, also called neuropsychiatric lupus, may present as psychosis, delirium, or seizures.

When lupus affects components of the blood it can cause hemolytic anemia, reduced white blood cell counts, or reduced platelet counts, the latter may present with bleeding disorder.

 These presentations can occur singularly, or in various combinations, and with marked variability in degree of severity. A mild form of the disease may remain so for many years, even for life, and an initially severe disease may persist, worsen, or go into remission on treatment. 

The most important thing therefore is to see a rheumatologist early if lupus is suspected so that the correct diagnosis can be made and appropriate treatment given. Do not pay much attention to what people who are not specialists tell you about this disease, even if they themselves are lupus patients, neither should you rely so much on the information you obtain from the internet. These will only give you more psychological stress. Discuss any questions or concerns you have with your rheumatologist to obtain the correct guidance.

Comprehensive Rheumatology Clinic Nairobi

Corner House, 4th floor, Room A1, Kimathi St.

P.O. Box 2640 - 00202 Nairobi, Kenya. mobile: +254 728 820 209 email: info@crcnairobi.co.ke website: www.crcnairobi.co.ke

CRC NAIROBI