Ankylosing Spondylitis

A 23 year old male patient was presented to the medical ward with one year history of joint pains. He was mainly complaining of large joint involvement. He complained of back pain, shoulder, elbow, hip and knee joint pains, but there was no small joint involvement. The pain was more in the morning and there was significant morning stiffness.  He was having the symptoms for a long period of time hasn't got any regular treatment up until now.

On examination, he had restricted movements of his spine, but there were no joint deformities. Schoeber's test was positive indicating the presence of significant spinal stiffness. Schoeber's test is performed by marking a spot on the back, at the mid line, at the level of iliac crest. Then two points vertically 10 cm above and 5 cm below this point is marked while the patient is standing. The the patient is asked to bend as much as he can and the distance between the two marked point is measured. A reading less than 5 cm implies spinal stiffness. The reading of this particular patient was only 2 cm. The patient also had reduced chest expansion as a result of costochondral junction involvement (normal chest expansion should be at least 5 cm at the nipple level).

With all these findings, the patient is clinically diagnosed to have ankylosing spondylitis. Three out of four of following clinical criteria should be there to diagnose a patient as having ankylosing songilitis. These clinical features are,

1. Morning stiffness lasting more than 30 min. 
2. Improvement of back pain with movements but not with rest.
3. Awakening because of back pain during second half of the night only
4. Alternating buttock pain.

Ankylosing spondylitis, as the name implies, is a inflammatory disorder of the spine. This is mainly seen among young males who are in their late teen or early twenties. Even though both men and women (2.5: 1) can be affected but symptoms are more prominent in men. The disease has a strong association with HLA B27 gene. These patients are also more susceptible to develop iritis also.

Investigations are performed to confirm the diagnosis.

1. ESR - elevated
• The patient's ESR was 112 mm/1st hour
2. CRP (C reactive proteins) - elevated
• The patients CRP level was 24
3. HLA B 27 testing 
4. MRI - shows sacroilitis before it is seen on X rays
5. X rays 
• X ray lumbar spine
• The fusion of vertebra leads to a
  bamboo spine
• X ray sacroiliac joint
• The following radiograph is taken from our patient. There is sclerosis of either sides of the joint with irregular joint space suggestive of sacroilitis, but there is no ankylosis of the sacroiliac joint.

Ankylosing sondylitis - Sacroilitis

Treatment

• Exercising - Exercising plays a major role in preventing the progression of the disease and improving the symptoms.
• NSAIDs - Sometimes, the pain makes it impossible for the patient to carryout exercises. This is where NSAIDs come in to play. An evening dose of slow releasing NSAID, oral or as a suppository will reveal pain and help in carrying out an active lifestyle and improve sleep.
• Methrotexate is helpful if there is peripheral joint involvement, but will not improve spinal disease.
• Sustained and dramatic reduction of inflammation can be achieved by TNF alpha blocking drugs.
• Rituximab does not help in seronegative arthritis.

Prognosis

The prognosis is good with adequate exercises. Fifty percent of the HLA B27 positive patients have the risk of transmitting the gene to their offspring. Thirty percents of these offspring may get the disease eventually. 

Psoriasis Treatment- Side Effects of Cyclosporine

Are you a psoriasis patient? Are you given psoriasis? Then you should know about the side effects of cyclosporine. This is a very effective drug in severe psoriasis, but it should be given under specialist care.

Side effects of long term treatment include,

• Hypertension
• Kidney damage
• Viral warts with risk of skin cancer
• Hypertriglyceridaemia
• Hersutism

Therefore the doctor will do the following tests before starting treatments.

• Blood pressure
• Serum creatinine

 

He will check serum creatinine 2 or three times before starting the treatment. Serum creatinine is a good indicator of renal function. Values more than the normal levels, indicate kidney damage.  This is to establish a baseline value for serum creatinine so subsequent values (every other week) after starting the treatments can be used to assess any kidney damage.

Fifty percent of patients who are on cyclosporine can develop systolic blood pressure >160mmHg and diastolic blood pressure >95mmHg. Such patients are given calcium channel blockers like Nifedipine or angiotensin converting enzyme inhibitors if not tolerated. 

A lipid profile will also be done to see any development of Hypertriglyceridaemia.

Purple Discoloration around the Eyes? Is it Dermatomyositis?

Dermatomyositis is a rare condition in which there is purple discoloration and edema around the eyes. They also have purple raised vasculitic patches on the fingers. They may also have Raynaud phenomenon.

 But, these are not the only main symptoms in dermatomyositis. People who have dermatomyositis also have fatigue, weight loss, fever and malaise and the cardinal feature, progressive, symmetrical proximal muscle weakness. Their pelvic and shoulder muscles become wasted and movements like squatting and climbing stairs become difficult. Usually the face muscles and distal limb muscles are not affected. 

This is a disease common among females. These patients are in a higher risk of developing pulmonary interstitial fibrosis, Raynaud phenomenon, arthritis and hardening and fissuring of skin over the purple surface of the fingers. 

This can be a part of paraneoplastic syndrome, so the patient should be checked for breast, lung ovarian and stomach cancers.  

The disease is treated with physiotherapy, steroids and immunosuppressive therapy.

Related Topics:

Causes of 'Black Eye' 

More in:

The Official Patient's Sourcebook on Dermatomyositis: A Revised and Updated Directory for the Internet Age

Myositis and You: A Guide to Juvenile Dermatomyositis for Patients, Families, and Healthcare Providers

Hard Skin Lesion? Is it Scleroderma?

Don’t get panic if you also have a similar lesion, because it is a very rare disease with an incidence of 30 cases per 100,000.  It is 4 times commoner in females than in males, so if you are a female, you have a higher chance of getting the disease. People at the age of 30-50 years are affected by this disease.

It can manifest either as localized scleroderma or systemic scleroderma. 

The localized scleroderma can be either linear or morphea, and it only affect the skin.

The systemic disease can be,

1.      Limited
2.      Diffuse
3.     Sine

And it can affect the skin, underlying tissues, and internal organs (heart, lungs, and kidneys).

What are the symptoms?

Initially you may have,

•   musculoskeletal discomfort
•  fatigue
• weight loss 
•   heartburn

The more specific symptoms are referred to as CREST syndrome.

·         Calcinosis

·         Raynaud phenomenon

·         Esophageal reflux

·         Sclerodactily (tightly bound waxy skin on the fingers)

·         Talangiectasias

How to identify a scleroderma skin lesion? 

Thickening or hardening of the skin is the characteristic feature in scleroderma. The area of the body, in which the lesions develop, varies depending on the kind of the disease you have. Those common areas are listed below.

1.      Morphea – face, chest, back, arms, legs

2.      Linear scleroderma – arm, leg, forearm

3.      Limited scleroderma – face, lower arms and legs, hands, fingers

4.      Diffuse scleroderma – face, upper arms, upper legs, hands

·         Course of skin changes in diffuse scleroderma

o   Inflamed fingers – presence of edema and erythema,

o   pigmentory changes are also seen – alternating hyperpigmented and vitiligo like areas slat and pepper appearance)

o   pruritus (itching) in the early phase of the disease

These symptoms may last from weeks to months.

In addition to these, you may have Talangiectasias (dilated capillaries). They occur on the face and palmer surface of the hands. Subcutaneous Calcinosis is also seen along the extensor surface of the forearm and on the fingers.

How the heart is affected

Heart is involved in the late stages of the disease. The disease causes contraction band necrosis and tissue fibrosis, which result in arrhythmias, cardiomiopathy and heart failure. These may manifest as chest pain, palpitation or as dyspnoea on exertion.

How the lungs are affected

The lung involvement may manifest either as interstitial fibrosis or as pulmonary artery hypertension or both. The patient may have dyspnoea on exertion, but this is seen in later stages of the disease. 

How the kidneys are affected

Kidney involvement manifest as sudden onset of malignant hypertension. Earlier, before the drug, angiotensin converting enzyme inhibitors were discovered, renal failure was the cause of death for many scleroderma patients.  Therefore scleroderma patients must have regular blood pressure check ups. These patients may have other symptoms of hypertension such as headache, visual changes or seizures.  

How to diagnose

To diagnose there should be

·         Thickened skin changes proximal to the metacarpophalangial joint

                                   Or two of the following

·         Sclerodactily

·         Digital pitting

·         Bibasilar pulmonary fibrosis

Another way of diagnosing scleroderma is by the presence of 3 of the five features of the crest syndrome.

Presence of Rhaynaud’s phenomenon for longer period indicates limited scleroderma and rapidly progressed Rhaynaud’s phenomenon and other symptoms indicate diffuse scleroderma.

Treatment

Although there is no cure for scleroderma, there are treatment for some of the symptoms of the disease.

•    NSAIDS
•   Immunosuppressant drugs
•   Prostacyclin infusion for digital ulcerations