Ep-PAINE-nym



Behçet’s Disease

Other Known Aliases Silk Road Disease

Definition – systemic inflammatory disorder that commonly effects the eyes, mouth, GI/GU tract, nervous system, and blood vessels

Clinical Significance Pathogenesis is largely unknown, but theorized to have a genetic predisposition. It is rare in the United States and more common in the Middle East and Asia, where it received is other eponym as the “silk road disease” due to the trading routes going through Turkey and the Mediterranean. Onset of the disease in most commonly in 20’s-40’s and is more common in men than women. Treatment is most commonly antiinflammatories and immunosuppressants.

HistoryNamed after Hulusi Behçet (1889-1948), a Turkish dermatologist who received his medical doctorate from the Gülhane Military Medical Academy in Istanbul in 1910. He served as staff physician at the Edirne Military Hospital during World War I and took a special interest in venereal diseases and dermatology. He would go on to become professor in the newly formed republic of Turkey and was the first person in Turkish academia to receive this rank. He took a special interest in the manifestations of syphilis and published extensively on this condition. He described is eponymous condition in 1936 after following several patients with similar symptoms and presenting them at meeting in Paris. It should be noted that several others had described this condition, as early as 1922, but Behçet was the first to recommend it as a previously undiscovered disease process.


References

  1. Firkin BG and Whitwirth JA.  Dictionary of Medical Eponyms. 2nd ed.  New York, NY; Parthenon Publishing Group. 1996.
  2. Bartolucci S, Forbis P.  Stedman’s Medical Eponyms.  2nd ed.  Baltimore, MD; LWW.  2005.
  3. Yee AJ, Pfiffner P. (2012).  Medical Eponyms (Version 1.4.2) [Mobile Application Software].  Retrieved http://itunes.apple.com.
  4. Whonamedit – dictionary of medical eponyms. http://www.whonamedit.com
  5. Up To Date. www.uptodate.com
  6. H. Behçet. Über rezidivierende, aphtöse, durch ein Virus verursachte Geschwüre am Mund, am Auge und an den Genitalien. Dermatologische Wochenschrift, Hamburg, 1937, 105(36): 1152-1163.

Ep-PAINE-nym



Osgood-Schlatter Disease

Other Known Aliases tibial tubercle apophysitis

Definition – inflammation of the patellar tendon at the insertion of the tibial tuberosity

Clinical Significance Seen in adolescent athletes who participates in sports with repeated jumping or overloading of the knee in the bent position. This repetitive trauma on the open growth plates causes the painful separation of the tuberosity from the anterior tibia and manifests as a painful “bump”. Pain will persist until the epiphysis closes, but a persistent bump will remain. Treatment is with rest, NSAIDs, unloading bracing, and potentially casting in knee extension.

HistoryNamed after two physician who contemporaneously studied this condition. Robert Bayley Osgood (1873-1956), an American orthopaedic surgeon who received his medical doctorate from Harvard University in 1899, and Carl Schlatter (1863-1934), a Swiss surgeon who received his medical doctorate from the University of Zurich in 1889. Osgood would spend his career at Harvard University and Massachusetts General Hospital culminating in Professor of Surgery and Chief of Orthopaedic Surgery at both of these institutions. He would help write one of the first textbooks on orthopaedic surgery called “Diseases of the Bones and Joints” and creating one of the first structured orthopaedic residencies in the US at Harvard University. Schlatter surgery training started under Billroth in Vienna and would go on to perform the world’s first gastrectomy with end-to-end esophago-jejunostomy in 1897. Both published case reports and observations of this disease process in 1903


References

  1. Firkin BG and Whitwirth JA.  Dictionary of Medical Eponyms. 2nd ed.  New York, NY; Parthenon Publishing Group. 1996.
  2. Bartolucci S, Forbis P.  Stedman’s Medical Eponyms.  2nd ed.  Baltimore, MD; LWW.  2005.
  3. Yee AJ, Pfiffner P. (2012).  Medical Eponyms (Version 1.4.2) [Mobile Application Software].  Retrieved http://itunes.apple.com.
  4. Whonamedit – dictionary of medical eponyms. http://www.whonamedit.com
  5. Up To Date. www.uptodate.com
  6. Nowinski RJ, Mehlman CT. Hyphenated history: Osgood-Schlatter disease. Am J Orthop (Belle Mead NJ). 1998; 27(8):584-5. [pubmed]
  7. Osgood RB. Lesions of the tibial tubercle occurring during adolescence. Boston Medical and Surgical Journal 1903; 148: 114-117
  8. Schlatter C. Verletzungen des schnabelförmigen Forsatzes der oberen Tibiaepiphyse. Beitrage zur klinischen Chirurgie, 1903; 38: 874-887

Ep-PAINE-nym



Galeazzi Fracture

Other Known Aliases none

Definition – fracture of the distal third of the radius with dislocation at the distal radioulnar joint (DRUJ) often seen after a FOOSH with the forearm in pronation

Clinical Significance Due to the dual injury mechanism, there is a higher than normal risk fo compartment syndrome and nerve injury seen with this injury, specifically the anterior interosseous nerve (AIN). This can lead to to inability to make the “OK” sign with the thumb and index finger. Patients may also present with wrist drop due to injury of the radial nerve. Often described with Monteggia fractures due to the similar, but opposite injury patterns.

HistoryNamed after Ricardo Galeazzi (1866-1952), an Italian orthopaedic surgeon who received his medical doctorate from the Turin Medical School in 1886. His career would primarily focus on pediatrics making advancements in the understanding in congenital hip dysplasia, scoliosis, and achondroplasia after being appointed Director of the Pius Institute for Crippled Children in 1903, and going on to direct the orthopaedic clinic of the University of Milan for 35 years. He would publish his experience of 18 cases bearing his name in 1935, but it should be noted that it was first published in 1842 by Sir Astley Cooper.


References

  1. Firkin BG and Whitwirth JA.  Dictionary of Medical Eponyms. 2nd ed.  New York, NY; Parthenon Publishing Group. 1996.
  2. Bartolucci S, Forbis P.  Stedman’s Medical Eponyms.  2nd ed.  Baltimore, MD; LWW.  2005.
  3. Yee AJ, Pfiffner P. (2012).  Medical Eponyms (Version 1.4.2) [Mobile Application Software].  Retrieved http://itunes.apple.com.
  4. Whonamedit – dictionary of medical eponyms. http://www.whonamedit.com
  5. Up To Date. www.uptodate.com
  6. Galeazzi R. Archivio di ortopedia pubblicazione ufficiale del Pio istituto dei rachitici. Istituto ortopedico Gaetano Pini. 1935. [link]
  7. SCAGLIETTI O. Riccardo Galeazzi, 1866-1952. J Bone Joint Surg Br. 1953; 35-B(4):679-80. [pubmed]

PAINE #PANCE Pearl – Rheumatology



Question

49yo woman, with a history of hypertension and GERD, presents to your clinic with a six month history of bilateral shoulder weakness that she first noticed when putting on sweaters and jackets and carrying in groceries. She denies any history of pain, repetitive trauma, or weakness in the hands. On physical examination, she has 3.5/5 strength in shoulder abduction and flexion, as well as the below rash. She reports the rash has been present for about the same time, but doesn’t really bother her.

  1. What lab is likely to be profoundly elevated and what lab is most specific to this condition?
  2. What is the most likely diagnosis?


Answer

  1. Due to the inflammatory myopathy, muscle enzymes are often extremely elevated and are helpful in initial screening. Creatine kinase (CK) is most commonly ordered, but lactate dehydrogenase (LDH), aspartate aminotransferase (AST), and alanine aminotransferase (ALT) are often elevated as well. The most common myositis-specific autoantibody is Anti-Jo 1 with others being Anti-SRP and Anti-MI-2.
  2. Given this history and dermatologic “shawl sign”, dermatomyositis is most likely. Polymyositis does not present with skin findings.

Ep-PAINE-nym



Kienböck Disease

Other Known Aliases – none

Definitionavascular necrosis of the lunate

Clinical Significance most often results from trauma with biomechanical and vascular abnormalities that lead to progressive bone death. Patients will report wrist pain with decreased range of motion and grip strength. MRI is best for early diagnosis and treatment depends on the stage of disease using the Lichtman Classification system.

HistoryNamed after Robert Kienböck (1871-1953), who was an Austrian radiologist and received his medical doctorate from the University of Vienna in 1895. He would explore the new and blossoming field of radiology before becoming the head of the radiological department at Vienna General Hospital before becoming professor of radiology in 1926. He was a pioneer in the use of x-rays for medical diagnosis and would co-found the Vienna Radiology Society in 1923. He would publish his eponymous condition in 1910 in his treatise Über traumatische Malazie des Mondbeins und ihre Folgezustände (Traumatic malacia of the lunate and its consequences).


References

  1. Firkin BG and Whitwirth JA.  Dictionary of Medical Eponyms. 2nd ed.  New York, NY; Parthenon Publishing Group. 1996.
  2. Bartolucci S, Forbis P.  Stedman’s Medical Eponyms.  2nd ed.  Baltimore, MD; LWW.  2005.
  3. Yee AJ, Pfiffner P. (2012).  Medical Eponyms (Version 1.4.2) [Mobile Application Software].  Retrieved http://itunes.apple.com.
  4. Whonamedit – dictionary of medical eponyms. http://www.whonamedit.com
  5. Up To Date. www.uptodate.com
  6. Orthobullets. Kienbock Disease. https://www.orthobullets.com/hand/6050/kienbocks-disease
  7. Wagner JP, Chung KC. A Historical Report on Robert Kienböck and Kienböck Disease. The Journal of Hand Surgery. 2005;30(6):1117-1121. [link]
  8. Kienböck R. Über traumatische Malazie des Mondbeins und ihre Folgezustände: Entartungsformen und Kompressionfrakturen. Fortschritte auf dem Gebiete der Röntgenstrahlen. 1910–1911; 16: 77-103.

#63 – Osteoarthritis Treatment



***LISTEN TO THE PODCAST HERE***



Quick Osteoathritis Review

  • Signs and Symptoms
    • Progressive joint pain
      • Stage 1 – Intermittent, Predictable, limits only high-impact activities
      • Stage 2 – Constant, effects daily activities
      • Stage 3 – Constant with intermittent, unpredictable, intense pain with severe limitations
    • Worse in the afternoon
    • Decreased ROM
    • Joint-line tenderness
    • Swelling and effusions
  • Radiography
    • 4 Radiographic Criteria of Osteoarthritis
      • Diminished joint space
      • Bony sclerosis
      • Osteophytes
      • Subchondral cyst


General Osteoarthritis Treatment Principles

  • Education
    • Discuss modifiable risk factors
    • Prognosis
    • Treatment options and timeline
  • Goal Setting
    • Identify current issues
    • Set priorities
    • Develop realistic plan
      • Multiple short term goals to achieve long term goal
      • Directed at minimizing pain, optimizing function, and modify joint damage
  • Clinical Assessment and Follow-up
    • Should be every 3 months by provide
    • Factors to be addressed and discussed during visits:
      • Impact of pain on daily living and quality of life
      • Functional limitations
      • Recreational and/or occupational aspirations
      • Sleep disturbances
      • Fall risk assessment
      • Expectations of treatment

Updated Guidelines from ACR/AAF

2019 updated guidelines from American College of Rheumatology and American Arthritis Foundation


Non-Pharmacologic Treatment

  • Should be first line either alone or with pharmacologic therapy
  • Physical therapy is the mainstay of non-pharmacologic treatment
    • Usually 6 weeks
  • Weight loss
    • Loss of 10% of body weight equals 50% reduction in pain scores
    • Adipokines (leptin and adiponectin) released by adipose tissue are known inflammatory factors
    • Consultation with dietician can be helpful
  • Exercise
    • Exercising have comparable effects on pain and function compared to NSAIDs
    • Low-impact is best, but tailor to patient’s function and limitations
    • Activities to help with core strength and balance can have significant reductions in falls
  • Braces and Splints
    • When possible, these aids can have significant benefit in pain reduction during activities

Pharmacologic Treatments

  • Topical NSAIDs should be considered prior to oral NSAIDs
    • Knee > hand efficacy
    • Diclofenac 1% gel – 4g (large joints) or 2g (small joints) applied 3-4x/day
      • Now available OTC
  • Oral NSAIDs > acetaminophen
    • COX-2 selective NSAID
      • Celecoxib 100-200mg daily or BID
      • Diclofenac 75mg BID
      • Meloxicam 10-15mg daily
  • Duloxetine can be helpful
    • Desensitizes central nociceptive pain processing
    • 60-120mg daily
  • Intrarticular glucocorticoid injections have limited role
    • Can be used for short-term relief
      • Most helpful with the hip
      • Long-term use can damage cartilage
    • Triamcinolone 40mg
  • Hyaluronic acid is controversial with limited data
  • Avoid recommended glucosamine, chondroitin, vitamin D, and fish oil due to lack of clear data showing benefit

Surgical Indications and Management



Cottage Physician (1893)



References

  1. Hawker GA, Stewart L, French MR, et al. Understanding the pain experience in hip and knee osteoarthritis–an OARSI/OMERACT initiative. Osteoarthritis Cartilage. 2008; 16(4):415-22. [pubmed]
  2. French SD, Bennell KL, Nichols PJ, Hodges PW, Dobson FL, Hinman RS.  What do people with knee or hip osteoarthritis need to know? An international consensus of essential statements for osteoarthritis.  Arthritis Care Res (Hoboken). 2015;57(6):809. [pubmed]
  3. Bodenheimer T, Lorig K, Holman H, Grumbach K. Patient self-management of chronic disease in primary care. JAMA. 2002; 288(19):2469-75. [pubmed]
  4. Messier SP, Mihalko SL, Legault C, et al. Effects of intensive diet and exercise on knee joint loads, inflammation, and clinical outcomes among overweight and obese adults with knee osteoarthritis: the IDEA randomized clinical trial. JAMA. 2013; 310(12):1263-73. [PDF]
  5. Zhang W, Nuki G, Moskowitz RW, et al. OARSI recommendations for the management of hip and knee osteoarthritis: part III: Changes in evidence following systematic cumulative update of research published through January 2009. Osteoarthritis Cartilage. 2010; 18(4):476-99. [pubmed]
  6. 2019 American College of Rheumatology/Arthritis Foundation Guideline for Management of Osteoarthritis of the Hand, Hip, and Knee.  Arthritis Care Res (Hoboken). 2020;72(2):149-162. [pubmed]

PAINE #PANCE Pearl – Rheumatology



Question

43yo woman, with a history of hypertension and GERD, presents to your clinic with a six month history of bilateral shoulder weakness that she first noticed when putting on sweaters and jackets and carrying in groceries. She denies any history of pain, repetitive trauma, or weakness in the hands. On physical examination, she has 3.5/5 strength in shoulder abduction and flexion, as well as the below rash. She reports the rash has been present for about the same time, but doesn’t really bother her.

  1. What lab is likely to be profoundly elevated and what lab is most specific to this condition?
  2. What is the most likely diagnosis?