Hypertrophic Pulmonary Osteoarthropathy

Hypertrophic pulmonary osteoarthropathy is a paraneoplastic syndrome characterised by

1.grade 4 clubbing of fingers or toes,

2.symmetrical periostitis of long bones distally

3.arthropathy of wrists, ankles, knees

 

Other name

Marie-Bamberger Syndrome

Pierre-Marie-Bamberger Syndrome

 

Symptoms

Swelling and extreme pain of the affected limbs (wrists, ankles, knees and elbows, fingers and toes, long bones distally)

Stiffness of the joints may occur

Edema of the hand and foot can occur

Fever can occur

Gynaecomastia may be present in some cases

Joint (Knee) effusions may occur

Rarely thickening and swelling of the end of the nose

 

Physical Signs

Lovibond’s angle – The angle of the nail with the proximal nail fold is greater than 180 degree

Schamroth sign – The normal diamond shaped window created by placing the dorsal surfaces of opposite terminal phalanges is obliterated in clubbing.

Tenderness may be present in the wrist and the distal long bones.

 

Causes

Bronchogenic Carcinoma

Mesothelioma and pleural malignancies

Pulmonary Tuberculosis

Congenital cyanotic heart disease

Hepatic carcinoma

Colorectal carcinoma

Inflammatory bowel disease

Cirrhosis of liver

Empyema thoracis

Pulmonary fibrosis

HIV infection

Familial

Clubbing due to any cause can also progress to HPOA

 

Predisposition

Males are more commonly affected than females. In bronchogenic carcinoma it is rare with the oat cell type.

 

Pathogenesis

The pathogenesis is unclear and various theories have been postulated.

1) Shunt theory                In diseases with significant arterio-venous shunting, the megakaryocytes bypass the pulmonary circulation,where they usually get fragmented. These unfragmented megakaryocytes gets deposited in the finger tips stimulate the secretion of platelet derived growth factor (PDGF) and vascular endothelial growth factor (VEGF). This results in angiogenesis and endothelial hyperplasia which manifests as clubbing.

2) Hormonal theory        Humoral mechanism – the tumours may secrete substances that may act on the nail bed. Raised growth hormone and oestrogen are suggested by some researchers

3) Neurogenic theory - Reflex vasodilatation of the nail bed mediated through the intercostal nerves or vagus has also been hypothesized. The resolution of the symptoms after vagotomy is claimed as a support of this hypothesis. 

 

Radiology

X rays of the lone bones – subperiosteal new bone formation

Bone Scintigraphy can be done to confirm the diagnosis.It shows increased tracer uptake along the cortical margins.

 

Treatment

HPOA can be very disabling and should be treated even if the primary tumour is inoperable.

Treatment of the primary cause, such us resection of lung tumour usually leads to the resolution of pulmonary osteoarthropathy.

If the tumor is inoperable, chemotherapy and radiotherapy may be given

Non-steroidal anti-inflammatory drugs especially indomethacin can reduce pain

Octreotide subcutaneously

Vagotomy has shown to reduce the symptoms of HPOA

 

Differentials

Rheumatoid arthritis

SLE with polyarthritis

Tuberculous arthritis