The large mouth of largemouth bass is of interest to scientists trying to understand how joints work

Inside the bass’s mouth is a system of linked muscle and bone that resembles the mechanism of an oil rig. NYTimes:



Read more here: https://www.nytimes.com/2017/12/26/science/largemouth-bass-jaw.html?smid=tw-share

Unprecedented range of therapeutic options for rheumatic diseases is now available — challenge is to make them accessible

From The Lancet:

Small molecule inhibitors of Janus kinase (JAK)

Much of the recent knowledge of the underlying mechanisms that drive rheumatoid arthritis and other diseases has come from preclinical studies of key cytokines, including tumour necrosis factor alpha, interleukin 6, and granulocyte–macrophage colony-stimulating factor. New Janus kinase (JAK) inhibitors include tofacitinib and baricitinib which are approved for treatment of arthritis and other rheumatoid diseases.

Aggressive treatment, early

For a complex, progressive, chronic disease such as rheumatoid arthritis, the timing of intervention is critical. In the past, the recommended treatment approach was slow and steady, referred to as “the pyramid”—ie, a base of physical therapy and non-pharmacological interventions, followed by conservative treatment with non-steroidal anti-inflammatory drugs, then glucocorticoid steroids and, finally, administration of a conventional disease-modifying antirheumatic drug (DMARD). This concept is now inverted. Intensive intervention, initiated earlier, with conventional and biological DMARDs is increasingly recommended.

Biologics and small molecule inhibitors are added to the therapeutic arsenal. Focus on early window of opportunity for management, with treat-to-target approaches that include rapid intervention, and adjustment of medications for patients who do not achieve remission within 6 months. The new campaign, “Don't delay, connect today”, highlights this approach.

Affordability

Biosimilars might provide affordable options.

An unprecedented range of therapeutic options for rheumatic diseases is now available—the new challenge is to make them accessible.

References:

A platinum age for rheumatology - The Lancet http://buff.ly/2slCFPw

Fibromuscular Dysplasia - Cleveland Clinic video

Fibromuscular dysplasia (FMD) is an angiopathy that affects medium-sized arteries predominantly in young. Renal involvement occurs in 60-75%, cerebrovascular involvement in 25-30%, visceral involvement in 9%, and arteries of the limbs in about 5%.



Cleveland Clinic physician, Dr. Gornik and Pam Mace from FMDSA answer questions about fibromuscular dysplasia (FMD) on this spreecast video chat (6/2014).

References:

Fibromuscular Dysplasia (FMD): Causes, Types, Symptoms and Treatment - Cleveland Clinic http://bit.ly/1sRTXKw

Elbow Pain in Adults - 2014 review from Am Fam Physician

The elbow is a complex joint designed to withstand a wide range of dynamic exertional forces. The location and quality of elbow pain can generally localize the injury to one of the four anatomic regions: anterior, medial, lateral, or posterior.

The history should include questions about the onset of pain, what the patient was doing when the pain started, and the type and frequency of athletic and occupational activities.

What are the common causes?

- Lateral and medial epicondylitis are two of the more common diagnoses and often occur as a result of occupational activities. Patients have pain and tenderness over the affected tendinous insertion that are accentuated with specific movements.

- If lateral and medial epicondylitis treatments are unsuccessful, ulnar neuropathy and radial tunnel syndrome should be considered.

- Ulnar collateral ligament injuries occur in athletes participating in sports that involve overhead throwing.

- Biceps tendinopathy is a relatively common source of pain in the anterior elbow; history often includes repeated elbow flexion with forearm supination and pronation.

- Olecranon bursitis is a common cause of posterior elbow pain and swelling. It can be septic or aseptic, and is diagnosed based on history, physical examination, and bursal fluid analysis if necessary.

What are the recommended tests?

Plain radiography is the initial choice for the evaluation of acute injuries and is best for showing bony injuries, soft tissue swelling, and joint effusions. Magnetic resonance imaging is the preferred imaging modality for chronic elbow pain. Musculoskeletal ultrasonography allows for an inexpensive dynamic evaluation of commonly injured structures.

References:

Evaluation of Elbow Pain in Adults. Kane SF, Lynch JH, Taylor JC. Am Fam Physician. 2014 Apr 15;89(8):649-657.
http://www.ncbi.nlm.nih.gov/pubmed/24784124

Image source: Normal radiograph; right picture of the straightened arm shows the carrying angle of the elbow. Wikipedia, Creative Commons Attribution-Share Alike 2.5 Generic license.
http://en.wikipedia.org/wiki/File:Coude_fp.PNG

Erythromelalgia - The Syndrome of Red, Hot Feet - Mayo Clinic video

Erythromelalgia is the name applied to the clinical syndrome of red, hot extremities. Most often, the feet are involved; less often the hands are also involved; rarely, the face (ears especially) may be involved. This short video describes what this syndrome is, what is known about why it happens, how it is diagnosed and managed. Here is a Mayo Clinic video about erythromelalgia:



Erythromelalgia is a rare disorder that is characterized by burning pain and warmth and redness of the extremities. There is primary (idiopathic) and secondary erythromelalgia (most commonly associated with myeloproliferative disorders).

The name is derived from 3 Greek words: erythros (“red”), melos (“limb”), and algos (“pain”).

Pelated:

JAMA Dermatology | Erythromelalgia: Vasculopathy, Neuropathy, or Both? http://buff.ly/1uTVen2
Erythromelalgia - Medscape http://buff.ly/1uTV7Ii
Erythromelalgia - NIH Genetics Home Reference http://buff.ly/1iFFA5g
Paroxysmal burning pain caused by erythromelalgia : The Lancet http://buff.ly/1nuzN7X

Sarcoidosis - 2014 Lancet review

Sarcoidosis is a systemic disease of unknown cause that is characterised by the formation of immune granulomas in various organs, mainly the lungs and the lymphatic system.

Sarcoidosis might be the result of an exaggerated granulomatous reaction after exposure to unidentified antigens in individuals who are genetically susceptible.

Diagnosis is made by symptoms, PFTs, CXR, CT, endobronchial ultrasound and PET for assessment of inflammatory activity.

Recognition of unexplained persistent disabling symptoms, fatigue, small-fibre neurological impairment, cognitive failure, and changes to health state and quality of life, has improved.

Mortality in patients with sarcoidosis is higher than that of the general population, mainly due to pulmonary fibrosis.

References:

Sarcoidosis - The Lancet http://bit.ly/1eh9I5S
Image source: Lungs, Wikipedia, public domain.

Related:

Fibromyalgia - An Emerging but Controversial Condition - 2014 JAMA Update

Fibromyalgia is not a rheumatological disease but a central pain syndrome. It is a complicated condition that tends to last a long time, although people do get better.

The syndrome termed fibromyalgia or fibrositis has attracted the interest of investigators for quite some time. Although some authors believe that fibromyalgia is not a discrete condition, rheumatologists now report that fibromyalgia is one of the most common diagnoses in ambulatory practice. Recent estimates of the prevalence of fibromyalgia in the United States have ranged from 3 to 6 million.

This JAMA video introduces a free review article on fibromyalgia from April 2014



What is fibromyalgia?

Fibromyalgia can be thought of as a centralized pain state. Centralized pain is a lifelong disorder beginning in adolescence or young adulthood manifested by pain experienced in different body regions at different times.

How common is fibromyalgia?

Fibromyalgia is present in as much as 2% to 8% of the population, is characterized by widespread pain, and is often accompanied by fatigue, memory problems, and sleep disturbances.

How to treat fibromyalgia?

Numerous treatments are available for managing fibromyalgia that are supported by high-quality evidence:

- nonpharmacological therapies (education, exercise, cognitive behavioral therapy)
- pharmacological therapies (tricyclics, serotonin norepinephrine reuptake inhibitors, and gabapentinoids)

Free content from JAMA:

JAMA | Fibromyalgia:  A Clinical Review http://buff.ly/1qH5ITI
JAMA Patient Page | Fibromyalgia http://buff.ly/1qH5HiH

Related reading:

Fibromyalgia is not a rheumatological disease but a central pain syndrome http://buff.ly/1qH4CaH
JAMA Network | JAMA | Fibromyalgia Syndrome:  An Emerging but Controversial Condition http://buff.ly/1m6Dv9w

Gout - Patrick's story - NHS video

From NHS Choices YouTube channel: Patrick, 54, was diagnosed with gout (a form of arthritis) 22 years ago. He describes the symptoms, treatment options and how he learned to live with the condition:



Here is a list of some of the new drugs for an old disease (gout):

Febuxostat is a non-purine-analogue inhibitor of xanthine oxidase that opened a new era in the treatment of gout.

Modified uricases

The use of modified uricases to rapidly reduce serum urate concentrations in patients with otherwise untreatable gout is progressing. Pegloticase, a pegylated uricase, is in development.

JAMA update, 08/2011: New Treatment Offers Hope for Patients With Severe Gout: pegloticase (Krystexxa) costs $2,500 per dose (http://goo.gl/gz9sO).

Drugs in development

Transport of uric acid in the renal proximal tubule and the inflammatory response to monosodium urate crystals (shown above) are targets for potential new treatments.

Several pipeline drugs for gout related to the targets above include:

- selective uricosuric drug RDEA594

- various interleukin-1 inhibitors. Canakinumab (trade name Ilaris) is a human monoclonal antibody targeted at interleukin-1 beta. It was rejected by the FDA panel in June 2011.

References:

Gout therapeutics: new drugs for an old disease. The Lancet, Volume 377, Issue 9760, Pages 165 - 177, 8 January 2011.
Diuretics, beta-blockers, ACEi, non-losartan ARBs associated with increased risk of gout vs. CCB lower risk. BMJ, 2012.
With FDA Approval, a Gout Drug Now Costs $5 Instead of Pennies - WSJ, 2011.
FDA Panel Rejects Gout Drug Canakinumab on Safety Concerns http://goo.gl/lO9uy
The strange story that links gout with the birth of the cocktail drinks. Lancet, 2012.

Comments from Twitter:

francis berenbaum @Larhumato: Gout explained by a patient. Very informative for medical students.

Acute low back pain: What to do? What works and what doesn't?

Here is an excerpt from a recent review article in the official AFP journal American Family Physician:

Acute low back pain is one of the most common reasons for adults to see a physician. Most patients recover quickly with minimal treatment.

"Red flags"

Serious "red flags" include:

- significant trauma related to age (i.e., injury related to a fall from a height or motor vehicle crash in a young patient, or from a minor fall or heavy lifting in a patient with osteoporosis or possible osteoporosis)
- major or progressive motor or sensory deficit
- new-onset bowel or bladder incontinence or urinary retention
- loss of anal sphincter tone
- saddle anesthesia
- history of cancer metastatic to bone
- suspected spinal infection

Diagnosis

Without signs of serious pathology, imaging and laboratory testing often are not required.

Treatment

Patient education, nonsteroidal anti-inflammatory drugs (NSAIDs), acetaminophen, and muscle relaxants are beneficial.

Bed rest should be avoided, if possible.

Exercises directed by a physical therapist, such as the McKenzie method and spine stabilization exercises, may decrease recurrent pain.

Spinal manipulation and chiropractic techniques are no more effective than established medical treatments.

No substantial benefit has been shown with:

- oral steroids
- acupuncture
- massage
- traction
- lumbar supports
- regular exercise programs

References:

Diagnosis and treatment of acute low back pain. Casazza BA. Am Fam Physician. 2012 Feb 15;85(4):343-50.

Image source: Different regions (curvatures) of the vertebral column, Wikipedia, public domain.

Social media and me - Rheumatologist shares his experience

This is a presentation by Dr. Ronan Kavanagh, Rheumatologist, Blogger, and Social Media in Healthcare advocate at Western Rheumatology, Galway, Ireland. His website is at ronankavanagh.wordpress.com

Socmed and me

View more presentations from Ronan Kavanagh

Comments from Twitter:

Dr. Ronan Kavanagh @RonanTKavanagh: @DrVes Thanks! You may notice I pinched a slide idea of two from you. Thanks for that too...

Symptomatic knee osteoarthritis (OA) increased during the past 20 years but radiographic OA did not

A recent surge in knee replacements is assumed to be due to aging and increased obesity of the U.S. population.

This cross-sectional study used data from 6 NHANES (National Health and Nutrition Examination Survey) surveys between 1971 and 2004 and from 3 examination periods in the FOA (Framingham Osteoarthritis) Study between 1983 through 2005 of the U.S. population.

Prevalence of knee pain increased by 65% in NHANES from 1974 to 1994.

In the FOA Study, prevalence of knee pain and symptomatic knee osteoarthritis doubled in women and tripled in men over 20 years.

However, no such trend was observed in the prevalence of radiographic knee osteoarthritis.

Prevalence of knee pain has increased substantially over 20 years. Obesity accounted for only part of this increase.

Symptomatic knee osteoarthritis increased but radiographic knee osteoarthritis did not. Why the patients are more symptomatic now than 20 years ago?

Research Finds Exercise is Good for Arthritis (a Cleveland Clinic video):



What is Boomeritis?

In 2006, the NYTimes described the health problems of aging baby boomers who continue to exercise: osteoarthritis which needs "knee and hip replacements, surgery for cartilage and ligament damage, and treatment for tendinitis, arthritis, bursitis and stress fractures." Some doctors call this phenomenon "boomeritis" or "Generation Ouch."

References:

Increasing Prevalence of Knee Pain and Symptomatic Knee Osteoarthritis: Survey and Cohort Data. ANN INTERN MED, December 6, 2011, vol. 155 no. 11 725-732.

What is Boomeritis?

Image source: OpenClipart.org, public domain.

Osteoarthritis at the base of the thumb has a 15-30% prevalence in adults

What is it?

Patients with osteoarthritis of the thumb carpometacarpal joint, or base of the thumb, commonly seek help for their symptoms. Arthritis at the base of the thumb causes functional disability and pain, particularly with “pinching” actions.


A hand with arthritic changes. Image source: Cicadas, a Creative Commons license.

How common is osteoarthritis of the thumb?

The prevalence of this condition increases with age and is greatest in postmenopausal women. It ranges between 15% prevalence in adults in Finland and a 33% prevalence in postmenopausal women. This is likely to increase as populations age and people stay active for longer.

How to diagnose it?

Pain reproduced on the axial grind test localizes pathology to the base of the thumb.

Trapeziometacarpal and scaphotrapeziotrapezoid joints should be assessed with plain radiographs (X-rays) that typically show degenerative changes. However, X-rays may underestimate the extent of the disease.

What to do?

Non-operative treatments can ameliorate symptoms and delay surgery in most patients with osteoarthritis of the thumb:

- behaviour modification
- pain relief
- splinting
- corticosteroid injections

No single operative procedure has been shown to be superior:

- simple trapeziectomy has the lowest complication rate
- arthrodesis may be the best option for patients who value pain relief and reliable strength and stability more than mobility (such as younger manual workers)

Piano lesson: "Rachmaninov had big hands". See how one gets 4 million views on YouTube:



References:

Osteoarthritis at the base of the thumb. BMJ, 2011.

Paget's disease - NHS Choices video

From the NHS Choices YouTube channel: An expert describes the various areas of the body that can be affected by Paget's disease - a condition in which the normal cycle of bone growth is disrupted.

The earliest references to journal clubs are in the memoirs and letters of Sir James Paget, a British surgeon, who described a group at St. Bartholomew’s Hospital in London in the mid-1800s as “a kind of club … a small room over a baker’s shop near the Hospital-gate where we could sit and read the journals.”

Sir Paget (11 January 1814 – 30 December 1899) is best remembered for Paget's disease and is considered, together with Rudolf Virchow, as one of the founders of scientific medical pathology.

References:

When was the earliest journal club?
James Paget, Wikipedia.

How to Stay Active With Osteoarthritis: "Motion is Lotion"

Dr. Daniel Montero, a sports-medicine physician within the Department of Orthopedics at Mayo Clinic in Florida discusses what kind of exercises are you should take part in if you suffer from joint pain. Remember, "motion is lotion", says Dr. Montero.

Exercises you may need to avoid if you have moderate or severe osteoarthritis of the knee or hip include:

- Running and jogging. The difference between how much force goes through your joints jogging or running, as opposed to with walking, is sometimes more than 10-fold your whole body weight

- Jumping rope

- High-impact aerobics

- Any activity where, at any time, you have both feet off the ground at once, however briefly (basketball, jumping)

Fortunately, that leaves a lot of activities that are OK for people with knee and hip osteoarthritis and that can help keep you mobile - see the list at WebMD.

References:

Knee and Hip Exercises for Osteoarthritis. WebMD.

Stress fractures

From a 2011 review in the journal American Family Physician:

Stress fractures are common injuries in athletes and military recruits. These typically affect lower extremities.

Symptoms of stress fractures

Stress fractures should be considered in patients who present with tenderness and/or edema after a recent increase in activity or repeated activity with limited rest.

The Barefoot Professor says barefoot running could minimize injuries although this approach is still experimental:



The differential diagnosis of stress fractures includes:

- tendinopathy
- compartment syndrome
- nerve or artery entrapment syndrome
- medial tibial stress syndrome (shin splints) can be distinguished from tibial stress fractures by diffuse tenderness along the length of the posteromedial tibial shaft and a lack of edema

Diagnosis of stress fractures

When stress fracture is suspected, plain radiography should be obtained initially and, if negative, may be repeated after 2-3 weeks for greater accuracy.

If an urgent diagnosis is needed, triple-phase bone scintigraphy (bone scan) or magnetic resonance imaging (MRI) should be considered. Both modalities have a similar sensitivity, but MRI has greater specificity.

Treatment of stress fractures

Treatment of stress fractures consists of:

- activity modification - nonweight-bearing crutches if needed for pain relief
- analgesics
- pneumatic bracing

After the pain is resolved, patients may gradually increase their level of activity.

Surgical consultation may be appropriate for patients with:

- stress fractures in high-risk locations
- nonunion
- recurrent stress fractures

References:

Stress fractures: diagnosis, treatment, and prevention. Patel DS, Roth M, Kapil N. Am Fam Physician. 2011 Jan 1;83(1):39-46.

The Barefoot Professor says barefoot running could minimize injuries

Related reading:

The Barefoot Running Revolution - INFOGRAPHIC  http://goo.gl/7SUGs

Sciatica: Robert's story

From NHS Choices YouTube channel: Robert's sciatica caused him intense pain in his right leg. He describes how the condition affected his life and mobility and what treatment options were available to him.

New Complication from Contaminated Cocaine - Bilateral Necrosis of the Ear Lobes and Cheeks

Interesting fact: Traces of cocaine taint up to 90% of paper money in the United States. Paper money become contaminated with cocaine during drug deals and directly through drug use, such as snorting cocaine through rolled bills. Amounts of cocaine found on U.S. bills ranged from 0.006-1,240 micrograms of cocaine per banknote (50 grains of sand) (http://bit.ly/27V5Yt).

Since 2005, levamisole (commonly used as to treat worm infections in humans and animals), has increasingly been used to mix cocaine for street use.

In 2009, 70% of cocaine seized at U.S. borders contained levamisole, causing an increase in cases of neutropenia among cocaine abusers.

Recently, researchers observed a new complication of levamisole contamination – vasculitis. Two cocaine abusers with similar cases of neutropenia and vasculitis presented to the University of Rochester Medical Center within 8 days of each other - with purplish plaques on their cheeks, earlobes, legs, thighs and buttocks. While the patients were not tested for levamisole levels, exposure was likely due to recent cocaine use.

Doctors should suspect levamisole exposure in patients presenting with both neutropenia and necrotic skin lesions.

See the dramatic photos from a similar case published in the NEJM here: Toxic Effects of Levamisole in a Cocaine User

References:
Bilateral Necrosis of Earlobes and Cheeks: Another Complication of Cocaine Contaminated With Levamisole. Ann of Int Med, June 1, 2010,  vol. 152  no. 11  758-759.

Exercises to Avoid with Osteoarthritis of the Knee or Hip

Participation in sport is associated with a with a 20—40% reduction in all-cause mortality compared with non-participation. Exercise might also be considered as a fifth vital sign, according to the Lancet: http://goo.gl/gyxYf

Exercises to Avoid with Osteoarthritis of the Knee or Hip include:

- Running and jogging. The difference between how much force goes through your joints jogging or running, as opposed to with walking, is sometimes more than 10-fold your whole body weight
- Jumping rope
- High-impact aerobics
- Any activity where, at any time, you have both feet off the ground at once, however briefly (basketball, jumping)

Fortunately, that leaves a lot of activities that are OK for people with knee and hip osteoarthritis and that can help keep you mobile - see the list at WebMD.

Research Finds Exercise is Good for Arthritis (a Cleveland Clinic video):



References:

Knee and Hip Exercises for Osteoarthritis. WebMD.
Image source: OpenClipart.org, public domain.

Porphyrias

From a Lancet review:

Hereditary porphyrias represent a group of 8 metabolic disorders of the haem biosynthesis. They are characterised by acute neurovisceral symptoms, skin lesions, or both.

Every porphyria is caused by abnormal function of a separate enzymatic step, resulting in a specific accumulation of haem precursors:

- 7 porphyrias are the result of a partial enzyme deficiency

- a gain of function mechanism is present in one new porphyria

Acute porphyrias present with acute attacks - severe abdominal pain, nausea, constipation, confusion, and seizure - and can be life-threatening.

Cutaneous porphyrias present with painful photosensitivity, skin fragility and blisters.

Porphyrias are still underdiagnosed. Screening of families to identify presymptomatic carriers and avoidance of precipitants is important.

References:

Porphyrias. The Lancet, Volume 375, Issue 9718, Pages 924 - 937, 13 March 2010.

Celiprolol as treatment of choice to prevent complications in vascular Ehlers-Danlos syndrome

Vascular Ehlers-Danlos syndrome is a rare hereditary connective tissue disorder caused by mutations in the collagen type III gene ( COL3A1 ), which leads to a loss of tissue integrity in many organ systems.

Patients with vascular Ehlers-Danlos syndrome have weakened blood vessels and an increased risk of arterial dissection or rupture that can lead to early death.

The researchers assessed the ability of celiprolol, a β1-adrenoceptor antagonist with a β2-adrenoceptor agonist action, to prevent arterial dissections and ruptures in vascular Ehlers-Danlos syndrome.

Patients with clinical vascular Ehlers-Danlos syndrome were randomly assigned to 5 years of treatment with celiprolol or to no treatment.

33 patients were positive for mutation of collagen 3A1 (COL3A1). Celiprolol was uptitrated every 6 months by steps of 100 mg to a maximum of 400 mg twice daily. The primary endpoints were arterial events (rupture or dissection, fatal or not).

Mean duration of follow-up was 47 months, with the trial stopped early for treatment benefit.

The primary endpoints were reached by 20% in the celiprolol group and by 50% controls (hazard ratio [HR] 0·36).

Celiprolol might be the treatment of choice for physicians aiming to prevent major complications in patients with vascular Ehlers-Danlos syndrome.

References:

Effect of celiprolol on prevention of cardiovascular events in vascular Ehlers-Danlos syndrome: a prospective randomised, open, blinded-endpoints trial. The Lancet, Volume 376, Issue 9751, Pages 1476 - 1484, 30 October 2010.

Celiprolol therapy for vascular Ehlers-Danlos syndrome. The Lancet, Volume 376, Issue 9751, Pages 1443 - 1444, 30 October 2010.