A 42 y.o. male comes to the ED with hand pain
What do you notice on his xrays?
What causes this problem?
Our patient had hypertrophic osteoarthropathy(HOA) with acro-osteolysis. Note the clubbing of the finger tips and digital osteonecrosis. This is a syndrome with periostitis of the bones and arthritis. Skin also is thickened and it is also known as pachydermoperiostosis.
clubbinb
HPO can be primary or secondary. The primary syndrome is genetically transmitted when VEGF(vascular endothelial growth factor) is induced by a malfunction of the HPGD gene. However, the majority of cases, 80%, are secondary to VEGF overproduction by paraneoplastic syndromes, lung diseases, chronic inflammation or congenital heart disease. All these conditions induce tissue hypoxia and VEGF is measurably increased.
The most common condition causing HOA is is non-small cell lung cancer and those presenting with new clubbing should be screened for this if there is no other obvious cause. 5-15% of patients with lung cancer develop clubbing.
lung cancer
THE PATHOPHYSIOLOGY OF HYPERTROPHIC OSTEOARTHROPATHY
In addition to chronic hypoxia from congenital heart disease, a second factor may contribute to overproduction of VEGF. VEGF is a platelet derived cytokine it is thought that conditions in which platelet clusters bypass the lung: right-to-left shunts and tumors make it more likely to develop
Once it is produced, VEGF interacts with endothelial cells and stimulates both new vessel formation, and production of osteoblasts resulting in new bone formation.
FUN FACTS
While hypertrophic osteoarthopathy is a specific syndrome referring to symmetrical periosteal reaction along the long bones, periosteal reactions ( both localized and generalized) occur in many other situations. A periosteal reaction can occur in trauma (ballet dancers) , inflammatory bowel disease, vitamin A excess, fluorosis, and even inflammation in a dental abscess. Rheumatic diseases and endocrine disorders(hyperparathyroidism and hyperthyroidism) have also been associated with acro-osteolysis.
Occupational acro-osteolysis has been reported with workers exposed to vinyl chloride in the production of PVC. They usually present with scleroderma -like symptoms rather than clubbing.
periosteal reaction from fluorosis on the right
Our patient had lung cancer.
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