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Giant cell arteritis (GCA)

Giant cell arteritis is a vasculitis of large and medium size vessels. Although it can affect arteries in the neck, upper body and arms, it occurs most often in the arteries in the temples. For this reason, giant cell arteritis is sometimes called temporal arteritis or cranial arteritis.

Giant cell arteritis is also known as granulomatous arteritis — a reference to a particular type of inflammation it causes.

Epidemiology & Aetiology

• Adults older than age 50 are at greatest risk of giant cell arteritis.
• Women and caucasians are most commonly affected.
• The exact cause isn't known, but researchers believe that genetic, viral and environmental factors may play roles in the inflammation.

Clinical presentation

Giant cell arteritis frequently causes headaches, jaw pain, and blurred or double vision, but the most serious potential complications are blindness and, less often, stroke. These problems occur when swelling in the arteries impairs blood flow to the eyes or brain.

The onset of the symptoms tends to be gradual and includes low grade fever, fatigue, weakness and weight loss.

• A new headache, mild or severe, occurs in at least two-thirds of patients with the pain tending to be located over the sides of the head in front of the ears but may be frontal or other located.
• Nearly one-half of patients suffer from jaw claudication after chewing.
• Impaired vision is often an early manifestation of the disease.
• Permanent partial or complete loss of vision in one or both eyes has been observed in 15-20 % of patients. It is rare for patients to become completely blind in both eyes.
• Polymyalgia rheumatica, which is characterized by pain in the shoulders and hips, is closely linked to GCA, occurring in about 40-50 % of patients.
  

Investigations

• ESR – elevated in most patients with GCA.
• Temporal artery biopsy

Suggested in all cases of suspected GCA even if the diagnosis may appear "classic".

The biopsy is of low risk, causes very little pain, and often leaves little or no scar.

After the use of a topical numbing medication (the same one used by a dentist), a small part of the temporal artery from under the scalp is removed.

• Other ways to diagnose GCA include: ultrasonography, angiographic examination, CT scanning and MR angiography, high resolution MRI and position emission tomography (PET).

Management

• Although there's no cure for giant cell arteritis, immediate treatment with corticosteroid medications usually relieves symptoms and prevents loss of vision.
• Glucocorticoid treatment should be instituted immediately once the diagnosis of GCA is established.
• Daily dosing is more effective than alternate day dosing. The response usually occurs within two to four weeks after the institution of therapy.
• The diagnosis should be reevaluated in patients who are resistant to adequate steroid therapy.
• Steroid withdrawal can begin once clinical remission has been induced.
• Relapses are seen more frequently in the first year or two of the disease.
• Relapses often necessitate increased dosage or prolonged steroid treatment. Some researchers have suggested that the addition of methotrexate may be steroid-sparing while others have not demonstrated any benefit. However the routine addition of methotrexate to glucocorticoid therapy for GCA is not recommended. The efficacy of other cytotoxic drugs, dapsone, antimalarials, etanercept, and penicillamine has not been studied adequately although they have been reported to be helpful in some case reports.
• The finding of an increased risk of visual loss in patients with GCA and thrombocytosis (increase of the number of platelets in the blood), has led some to suggest the addition of drugs like aspirin for patients with high platelet counts, but there is not a lot of data to prove that this may reduce brain/skull problems.

References:

• “Giant cell arteritis”, mayoclinic.com, http://www.mayoclinic.com/health/giant-cell-arteritis/DS00440
• “Giant Cell Arteritis (Temporal Arteritis)”, Vasculitis Foundation, http://www.vasculitisfoundation.org/giantcellarteritis

Reactive arthritis

Reactive arthritis aka Reiter's syndrome aka Reiter's disease aka arthritis urethritica, venereal arthritis, seronegative spondyloarthropathy, polyarteritis enterica.

Reactive arthritis is a RF-seronegative, HLA-B27-linked spondyloarthropathy with symptoms similar to arthritis or rheumatism. It is caused by genitourinary or gastrointestinal infections, and is thus "reactive", i.e. dependent on the other condition.

Reactive arthritis is the combination of three seemingly unlinked symptoms:

1. an inflammatory arthritis of large joints
2. inflammation of the eyes (conjunctivitis and uveitis), and
3. urethritis.

Epidemiology

• Most commonly strikes individuals aged 20-40.
• More common in men than in women.
• More common in white men than in black men due to white individuals being more likely to have tissue type HLA-B27 than black individuals.
  
• People with HIV have an increased risk of developing reactive arthritis.

History

Reactive arthritis was first described by Hans Reiter, a German military physician, who in 1916 described the disease in a World War I soldier who had recovered from a bout of diarrhea.

The term Reiter's syndrome is being phased out, partly due to a move in the field of medicine to give descriptive names, rather than personal names, to conditions, and partly due to Dr. Reiter's experiments in Nazi concentration camps.

Causes

• Reactive arthritis is set off by a preceding infection, the most common of which would be a genital infection with Chlamydia trachomatis.
• Other bacteria known to cause reactive arthritis are Neisseria gonorrhoeae, Ureaplasma urealyticum, Salmonella spp., Shigella spp., Yersinia spp., and Campylobacter spp..
• A bout of food poisoning or a gastrointestinal infection may also trigger the disease.
• Reactive arthritis usually manifests about 1-3 weeks after a known infection.

Pathophysiology

The mechanism of interaction between the infecting organism and the host is unknown.

Synovial fluid cultures are negative, suggesting that RA is caused either by an over-excited autoimmune response or by bacterial antigens which have somehow become deposited in the joints.

Signs and symptoms

• Symptoms generally appear within 1-3 weeks but can range from 4-35 days from the onset of the inciting episode of the disease.
• The classical presentation is that the first symptom experienced is a urinary symptom such as burning pain on urination (dysuria) or an increased need to urinate (polyuria or frequency).
• Other urogenital problems may arise such as prostatitis in men, and cervicitis, salpingitis and/or vulvovaginitis in women.
• The arthritis that follows usually affects the large joints such as the knees causing pain and swelling with relative sparing of small joints such as the wrist and hand.
• Eye involvement occurs in about 50% of men with urogenital reactive arthritis and about 75% of men with enteric reactive arthritis. Conjunctivitis and uveitis can cause redness of the eyes, eye pain and irritation, and blurred vision.
• Roughly 20 to 40 percent of men with reactive arthritis develop penile lesions called balanitis circinata (circinate balanitis) on the end of the penis. A small percentage of men and women develop small hard nodules called keratoderma blennorrhagica on the soles of the feet, and less often on the palms of the hands or elsewhere. In addition, some people develop mouth ulcers that come and go. In some cases, these ulcers are painless and go unnoticed.
• About 10 percent of people with Reactive Arthritis, especially those with prolonged disease, will develop cardiac manifestations including aortic regurgitation and pericarditis.

Commonly remembered with the mnemonic "Can't See, Can't Pee, Can't Climb a Tree"

Diagnosis

There are countless clinical symptoms, but the clinical picture is dominated by polyarthritis. There is pain, swelling, redness, and heat in the joints. MRI's are effective for diagnosis.

The urethra, cervix and throat may be swabbed in an attempt to culture the causative organisms. Cultures may be carried out on urine and stool samples.

Synovial fluid from an affected knee may be aspirated to look at the fluid under the microscope and for culture.

A blood test for the gene HLA-B27 may be given to determine if the patient has the gene. About 75 percent of all patients with Reiter's Syndrome have the gene.

Treatment

• The main goal of treatment is to identify and eradicate the underlying infectious source with the appropriate antibiotics.
• Treatment is symptomatic for each problem. Steroids and analgesics may be given for severe joint inflammation.
  
• Immunosuppressants may be needed for patients with severe reactive arthritis who do not respond to any other treatment.

Prognosis

• Reactive arthritis may be self limiting, frequently recurring or develop continually.
• Most patients have severe symptoms lasting a few weeks to six months.
• Approximately 15 to 50 percent of cases have recurrent bouts of arthritis.
• Chronic arthritis or sacroiliitis occurs in 15-30 percent of cases.
• Repeated attacks over many years is common, and more than 40 percent of the patients end up with chronic and disabling arthritis, heart disease or impaired vision.
• However, most people with reactive arthritis can expect to live normal life spans and maintain a near-normal lifestyle with modest adaptations to protect the involved joints.

References:

• http://en.wikipedia.org/wiki/Reiter%27s_disease