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CREST syndrome

Scleroderma is a rare, chronic disease characterized by excessive deposits of collagen. Progressive systemic scleroderma or systemic sclerosis, the generalised type of the disease, can be fatal. The localised type of the disease tends not to be fatal. The term 'localised, generalised sclerderma' can be used to describe cases where the disease covers a large area of the body - typically more than 40%. more...

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Signs and symptoms

Scleroderma affects the skin, and in more serious cases, it can affect the blood vessels and internal organs. The most evident symptom is the hardening of the skin and associated scarring. Typically the skin appears reddish or scaly in appearance. Blood vessels may also be more visible. Where large areas are affected, fat and muscle wastage will weaken limbs and affect appearance.

The seriousness of the disease varies hugely between cases. The two most important factors to consider are, the level of internal involvement (beneath the skin), and the total area covered by the disease. For example there are cases where the patient has no more than one or two lesions (affected areas), perhaps covering a few inches. These are less serious cases and tend not to involve the internal bodily functions.

Cases with larger coverage are far more likely to affect the internal tissues and organs. Where an entire limb is affected, symptoms will almost certainly have serious consequences on the use of that limb. The heart and lungs will be affected when the disease covers this area of the torso. Some patients also experience gastrointestinal problems, including heartburn and acid reflux. Internal scarring may sometimes spread beyond what can be seen by the naked eye.

There is discoloration of the hands and feet in response to cold. Most patients (>80%) have Raynaud's phenomenon, a vascular symptom that can affect the fingers, and toes.

Systemic scleroderma and Raynaud's can cause painful ulcers on the fingers or toes, which are known as digital ulcers.

Types

There are three major forms of scleroderma: diffuse, limited (CREST syndrome) and morphea/linear. Diffuse and limited scleroderma are both a systemic disease, whereas the linear/morphea form is localized to the skin. (Some physicians consider CREST and limited scleroderma one and the same, others treat them as two separate forms of scleroderma.)

Diffuse scleroderma

Diffuse scleroderma is the most severe form - it has a rapid onset, involves more widespread skin hardening, will generally cause much internal organ damage (specifically the lungs and gastrointestinal tract), and is generally more life threatening.

Limited scleroderma/CREST syndrome

The limited form is much milder: it has a slow onset and progression, skin hardening is usually confined to the hands and face, internal organ involvement is less severe, and a much better prognosis is expected.

The limited form is often referred to as "CREST" syndrome. CREST is an acronym for:

  • Calcinosis
  • Raynaud's syndrome
  • Esophageal dysmotility
  • Sclerodactyly
  • Telangiectasia

These five are the major symptoms of the CREST syndrome.

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Iliotibial band syndrome: a common source of knee pain
From American Family Physician, 4/15/05 by Razib Khaund

Iliotibial band syndrome is a common knee injury. The most common symptom is lateral knee pain caused by inflammation of the distal portion of the iliotibial band. The iliotibial band is a thick band of fascia that crosses the hip joint and extends distally to insert on the patella, tibia, and biceps femoris tendon. In some athletes, repetitive flexion and extension of the knee causes the distal iliotibial band to become irritated and inflamed resulting in diffuse lateral knee pain. Iliotibial band syndrome can cause significant morbidity and lead to cessation of exercise. Although iliotibial band syndrome is easily diagnosed clinically, it can be extremely challenging to treat. Treatment requires active patient participation and compliance with activity modification. Most patients respond to conservative treatment involving stretching of the iliotibial band, strengthening of the gluteus medius, and altering training regimens. Corticosteroid injections should be considered if visible swelling or pain with ambulation persists for more than three days after initiating treatment. A small percentage of patients are refractory to conservative treatment and may require surgical release of the iliotibial band.

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Iliotibial band; band syndrome is a common knee injury that usually presents as lateral knee pain caused by inflammation of the distal portion of the iliotibial occasionally, however, the iliotibial band becomes inflamed at its proximal origin and causes referred hip pain. The iliotibial band is a thick band of fascia that is formed proximally by the confluence of fascia from hip flexors, extensors, and abductors. The band originates at the lateral iliac crest and extends distally to the patella, tibia, and biceps femoris tendon (Figure 1). (1)

Iliotibial band syndrome occurs frequently in runners or cyclists, and is caused by a combination of overuse and biomechanical factors. The syndrome can cause significant morbidity; however, most patients respond to a conservative treatment approach that involves stretching and altering training regimens.

Etiology

Iliotibial band syndrome is caused by excessive friction of the distal iliotibial band as it slides over the lateral femoral epicondyle during repetitive flexion and extension of the knee resulting in friction and potential irritation. In patients with iliotibial band syndrome, magnetic resonance imaging (MRI) studies have shown that the distal iliotibial band becomes thickened and that the potential space deep to the iliotibial band over the femoral epicondyle becomes inflamed and filled with fluid. (2)

Despite a clear pathophysiology, it is unclear why this syndrome does not affect all athletes. Few studies (3-7) have shown any direct relationship between biomechanical factors and the development of iliotibial band syndrome. Excessive pronation causing tibial internal rotation and increased stress in the iliotibial band was believed to be a factor in the development of iliotibial band syndrome; however, the literature does not support this theory.

Some observational studies (4,6) have identified potential risk factors for the development of iliotibial band syndrome, including the following: preexisting iliotibial band tightness; high weekly mileage; time spent walking or running on a track; interval training; and muscular weakness of knee extensors, knee flexors, and hip abductors. Hip abductor weakness seems to contribute to the development of iliotibial band syndrome. Strengthening of the hip abductors has led to symptom improvement. (6)

Clinical Presentation

The primary initial complaint in patients with iliotibial band syndrome is diffuse pain over the lateral aspect of the knee. These patients frequently are unable to indicate one specific area of tenderness, but tend to use the palm of the hand to indicate pain over the entire lateral aspect of the knee. With time and continued activity, the initial lateral achiness progresses into a more painful, sharp, and localized discomfort over the lateral femoral epicondyle and/or the lateral tibial tubercle. Typically, the pain begins after the completion of a run or several minutes into a run; however, as the iliotibial band becomes increasingly irritated, the symptoms typically begin earlier in an exercise session and can even occur when the person is at rest. Patients often note that the pain is aggravated while running down hills, lengthening their stride, or sitting for long periods of time with the knee in the flexed position. (7) The differential diagnosis for lateral knee pain is listed in Table 1.

Physical Examination

Patients with iliotibial band syndrome often demonstrate tenderness on palpation of the lateral knee approximately (2) cm above the joint line. Tenderness frequently is worse when the patient is in a standing position and the knee is flexed to 30 degrees. At this angle, the iliotibial band slides over the femoral condyle and is at maximal stress, thus reproducing the patient's symptoms. (1,6) Swelling may be noted at the distal iliotibial band and thorough palpation of the affected limb may reveal multiple trigger points in the vastus lateralis, gluteus medius, and biceps femoris. Palpation of these trigger points may cause referred pain to the lateral aspect of the affected knee. Strength of the lower extremity should be assessed with particular emphasis on examining the knee extensors, knee flexors, and hip abductors. Weakness in these muscle groups has been associated with the development of iliotibial band syndrome. (4,6,7)

The Ober's test can be used to assess tightness of the iliotibial band (Figure 2). With the patient lying on the side with the unaffected side down and the unaffected hip and knee at a 90-degree angle, the examiner stabilizes the pelvis, then abducts and extends the affected leg until it is aligned with the rest of the patient's body. The affected leg is lowered into adduction. If the iliotibial band is normal in length and unaffected, the leg will adduct and the patient will not experience pain. If the iliotibial band is tight, the leg will remain in the abducted position and the patient may have lateral knee pain. (1,6,8) A tight iliotibial band contributes to the excess friction placed on the iliotibial band as it slides over the femoral condyle during flexion and extension of the knee.

[FIGURE 2 OMITTED]

A clinical diagnosis is based on the history and physical examination. If the diagnosis is in doubt or other joint pathology is suspected, MRI can aid in the diagnosis and provide additional information about patients considered for surgery. In patients with iliotibial band syndrome, MRI shows a thickened iliotibial band over the lateral femoral epicondyle and often detects a fluid collection deep to the iliotibial band in the same region. (2)

Treatment

Treatment requires activity modification, massage, and stretching and strengthening of the affected limb. The goal is to minimize the friction of the iliotibial band as it slides over the femoral condyle. The patient may be referred to a physical therapist who is trained in treating iliotibial band syndrome. Most runners with low mileage respond to a regimen of anti-inflammatory medicines and stretching; however, competitive or high-mileage runners may need a more comprehensive treatment program.

The initial goal of treatment should be to alleviate inflammation by using ice and anti-inflammatory medications. Patient education and activity modification are crucial to successful treatment. Any activity that requires repeated knee flexion and extension is prohibited. During treatment, the patient may swim to maintain cardiovascular fitness. If visible swelling or pain with ambulation persists for more than three days after initiating treatment, a local corticosteroid injection should be considered (6) (Figure 3).

[FIGURE 3 OMITTED]

As the acute inflammation diminishes, the patient should begin a stretching regimen that focuses on the iliotibial band as well as the hip flexors and plantar flexors. The common iliotibial band stretches (Figure 4) have been evaluated for their effectiveness in stretching the band. The stretch shown in Figure 4C was consistently the most effective in increasing the length of the iliotibial band in a study (9) of elite distance runners. Although this study (9) demonstrates the effectiveness of stretching the iliotibial band, participants in the study did not have iliotibial band syndrome and studies have not demonstrated that stretching hastens recovery from the syndrome.

[FIGURE 4 OMITTED]

Once the patient can perform stretching without pain, a strengthening program should be initiated. Strength training should be an integral part of any runner's regimen; however, for patients with iliotibial band syndrome particular emphasis needs to be placed on the gluteus medius muscle. (6) A strengthening exercise geared toward the gluteus medius is shown in Figure 5.

[FIGURE 5 OMITTED]

Running should be resumed only after the patient is able to perform all of the strength exercises without pain. The return to running should be gradual, starting at an easy pace on a level surface. If the patient is able to tolerate this type of running without pain, mileage can be increased slowly. For the first week, patients should run only every other day, starting with easy sprints on a level surface. Most patients improve within three to six weeks if they are compliant with their stretching and activity limitations. (1)

For patients who do not respond to conservative treatment, surgery should be considered. The most common approach is to release the posterior 2 cm of the iliotibial band where it passes over the lateral epicondyle of the femur. In a retrospective study (10) of 45 patients who underwent surgical release of their iliotibial band, 84 percent of the patients reported that their surgery results were good to excellent.

REFERENCES

(1.) Panni AS, Biedert RM, Maffulli N, Tartarone M, Romanini E. Overuse injuries of the extensor mechanism in athletes. Clin Sports Med 2002;21:483-98.

(2.) Ekman EF, Pope T, Martin DF, Curl WW. Magnetic resonance imaging of iliotibial band syndrome. Am J Sports Med 1994;22:851-4.

(3.) Taunton JE, Ryan MB, Clement DB, McKenzie DC, Lloyd-Smith DR, Zumbo BD. A retrospective case-control analysis of 2002 running injuries. Br J Sports Med 2002;36:95-101.

(4.) Messier SP, Edwards DG, Martin DF, Lowery RB, Cannon DW, James MK, et al. Etiology of iliotibial band friction syndrome in distance runners. Med Sci Sports Exerc 1995;27:951-60.

(5.) Messier SP, Pittala KA. Etiologic factors associated with selected running injuries. Med Sci Sports Exerc 1988;20:501-5.

(6.) Fredericson M, Cookingham CL, Chaudhari AM, Dowdell BC, Oestreicher N, Sahrmann SA. Hip abductor weakness in distance runners with iliotibial band syndrome. Clin J Sport Med 2000;10:169-75.

(7.) Orchard JW, Fricker PA, Abud AT, Mason BR. Biomechanics of iliotibial band friction syndrome in runners. Am J Sports Med 1996;24:375-9.

(8.) Fredericson M, Guillet M, DeBenedictis L. Quick solutions for iliotibial band syndrome. Phys Sportsmed 2000;28:53-68.

(9.) Fredericson M, White JJ, Macmahon JM, Andriacchi TP. Quantitative analysis of the relative effectiveness of 3 iliotibial band stretches. Arch Phys Med Rehabil 2002;83:589-92.

(10.) Drogset JO, Rossvoll I, Grontvedt T. Surgical treatment of iliotibial band friction syndrome. A retrospective study of 45 patients. Scand J Med Sci Sports 1999;9:296-8.

RAZIB KHAUND, M.D., is clinical assistant professor of medicine in the Department of Orthopedic and Internal Medicine at Brown University School of Medicine, Providence, R.I., a physician in internal medicine at the Hughston Clinic in Columbus, Georgia, and a sports medicine specialist at the New England Center for Athletes in Providence. Dr. Khaund received his medical degree from New Jersey Medical University, Newark. He completed a fellowship in sports medicine at the Hughston Clinic.

SHARON H. FLYNN, M.D., is a hospitalist at the Oregon Medical Group/Hospital Service, Eugene, Ore., and has a special interest in sports medicine. She received her medical degree from George Washington University Medical Center, Washington, D.C., and completed a residency in internal medicine at Rhode Island Hospital/Brown University School of Medicine.

Address correspondence to Sharon H. Flynn, M.D., Oregon Medical Group/Hospital Service, 1200 Hilyard St., Suite S-140, Eugene, OR 97401 (e-mail: sflynn@peacehealth.org). Reprints are not available from the authors.

The authors indicate that they do not have any conflicts of interest. Sources of funding: none reported.

Figures 2 through 5 used with permission from Sharon H. Flynn, M.D.

COPYRIGHT 2005 American Academy of Family Physicians
COPYRIGHT 2005 Gale Group

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