Hypertrophic osteoarthropathy

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Classification according to ICD-10
M89.4 Other hypertrophic osteoarthropathy
ICD-10 online (WHO version 2019)

In the hypertrophic osteoarthropathy is a rare disorder characterized by painful swelling, especially in the area of diaphysis of the long bones   (the limbs ) and drumstick-like widening of the fingers and toes and Uhrglasnägeln .

Classification

A distinction can be made between the following types:

  • Primary (idiopathic) hypertrophic osteoarthropathy (PHO) (of the child), frequency 3%
  • secondary (acquired) hypertrophic osteoarthropathy (adult)
    • Hypertrophic pulmonary osteoarthropathy (outdated term English Hypertrophic osteoarthropathy (HOA) )
    • classic paraneoplastic disease in rheumatology

all referred to as Marie Bamberger Syndrome .

The following is about the adult form.

Sometimes joint involvement occurs. Vasomotor disorders of the hands and feet may be present. Neurovegetative disorders and dysproteinemia are mentioned. Men before middle age are preferably affected.

Synonyms

  • Bamberger syndrome after the German doctor Eugen von Bamberger (1858–1921)
  • Marie Bamberger Syndrome or Marie Bamberger Disease
  • Pierre Marie disease , after the French neurologist Pierre Marie (1853-1940)
  • Pierre Marie Bamberger Disease
  • Ostéoarthropathie hypertrophiante
  • Osteoperiostitis ossificans toxicans

Bamberger first described the disease in detail in 1889 and referred to the connection between the typical widening of the fingers and toes and lung and heart diseases. In 1890 Marie worked out the differences to acromegaly . Since the symptom complex was first described by Nikolaus Friedreich in 1868 by the brothers Karl and Wilhelm Hagner, it is also referred to as Hagner syndrome , the very rare familial occurrence according to Boris Nikitich Mankowsky also called Mankowsky syndrome .

causes

Marie-Bamberger2.jpg
Typical image in skeletal scintigraphy with increased accumulation in the long tubular bones.
Marie-Bamberger PET-CT (NaF) .jpg
Findings in the maximum intensity projection of a PET / CT with 18 F sodium fluoride (NaF).


Since lung tumors can be a cause of Marie-Bamberger syndrome and more than 90% of cases are associated with non-small-cell bronchial carcinoma, Marie-Bamberger syndrome is also considered a paraneoplastic syndrome and must be the subject of an appropriate investigation when making a diagnosis.

In terms of differential diagnosis, other chronic lung diseases such as cystic fibrosis , tuberculosis , bronchiectasis , pulmonary fibrosis , and more rarely diseases of the heart and liver ( liver cirrhosis ), but also chronic inflammatory bowel diseases (such as ulcerative colitis  ) must be considered and looked for if necessary. The clinical picture occurs even more rarely spontaneously or in families.

Pretibial pain and swelling can precede malignant intrathoracic disease by months.

therapy

Aside from treating the underlying disease , nonsteroidal anti-inflammatory drugs are given to relieve pain .

Individual evidence

  1. Periosteal thickening in the x-ray of the distal femur ( Memento of the original from January 11, 2006 in the Internet Archive ) Info: The archive link was inserted automatically and has not yet been checked. Please check the original and archive link according to the instructions and then remove this notice. @1@ 2Template: Webachiv / IABot / www.mevis.de
  2. ... also in 2 levels  ( page no longer available , search in web archivesInfo: The link was automatically marked as defective. Please check the link according to the instructions and then remove this notice.@1@ 2Template: Dead Link / www.mevis.de  
  3. a b emedicine
  4. Pachydermoperiostosis. In: Orphanet (Rare Disease Database).
  5. ^ Cranio-osteoarthropathy. In: Orphanet (Rare Disease Database).
  6. Whonamedit: disease Bamberger-Marie
  7. RW Bettschart, C. Waldherr, W. Wiesner, R. Bingisser: Pretibial pain and swellings. ( Memento of the original from November 7, 2007 in the Internet Archive ) Info: The archive link was inserted automatically and has not yet been checked. Please check the original and archive link according to the instructions and then remove this notice. In: Switzerland Med Wochenschr. 2000; 130, p. 558. @1@ 2Template: Webachiv / IABot / www.smw.ch
  8. Julia Crone, Wolf Dietrich Huber, Rosemarie Resch Walter Ponhold, Gerhard Grandi sealed: Hypertrophic osteoarthropathy in Untreated Ulcerative Colitis . In: Journal of Pediatric Gastroenterology and Nutrition . 2002, 35, pp. 213-215.