Hypertrophic osteoarthropathy is a disease of the joints and bones.
Hypertrophic osteoarthropathy is characterized by clubbing of the digits of the hands and feet, enlargement of the extremities as a consequence of periarticular and osseous proliferation, and painful and swollen joints. Two categories are recognized, primary (hereditary or idiopathic) and secondary.
Primary hypertrophic osteoarthropathy
, also called pachydermoperiostosis, is inherited as an autosomal dominant trait. Patients may have the complete syndrome (with pachydermia, periostitis and cutis verticis gyrata), the incomplete form (with sparing of the scalp), or the forme fruste (with pachydermia and minimal or absent periostitis). On radiographs, periostitis predominates. Poorly defined bone outgrowths in the epiphyses of tubular bones and sometimes also in the axial skeleton are especially characteristic of this disease. Diaphyseal expansion in tubular bones and sclerosis of the spongiosa in both appendicular and axial skeletal sites are evident. Other features are prominent sinuses, moderate enlargement of the mandible, and calcification and ossification of ligaments.
Secondary hypertrophic osteoarthropathy
occurs as a consequence of another disease process (Table 1).
Osteoarthropathy, Table 1. Some causes of secondary hypertrophic osteoarthropathy.
| Pulmonary | |
| Bronchogenic carcinoma | |
| Abscess | |
| Bronchiectasis | |
| Emphysema | |
| Hodgkin's disease | |
| Metastasis | |
| Cystic fibrosis | |
| Pleural, diaphragmatic | |
| Mesothelioma | |
| Cardiac | |
| Cyanotic congenital heart disease | |
| Abdominal | |
| Portal or biliary cirrhosis | |
| Ulclerative colitis | |
| Crohn's disease | |
| Dysentery | |
| Gastrointestinal polyposis | |
| Neoplasms | |
| Biliary atresia | |
| Miscellaneous | |
| Nasopharyngeal carcinoma | |
| Oesophageal carcinoma | |
| Infected aortic or axillary artery grafts | |
In the secondary form, digital clubbing (at times associated with focal areas of tuftal hypertrophy or resorption), joint pain, and tenderness are frequent manifestations. Initially periosteal bone deposition occurs in the diaphyses of the tibiae, fibulae, radii, ulnae, and sometimes the femora, humeri, metacarpals, metatarsals and phalanges. Although periostitis becomes prominent in metaphyses, it does not usually extend into the epiphyses. Among the types of periostitis are a single layer of new bone, laminated or onion peel pattern, and irregular areas of periosteal elevation. A diffuse, symmetric increased uptake on bone scans in the diaphyses and metaphyses of tubular bones along their cortical margins creates a distinctive parallel track sign.
Neuropathic osteoarthropathy occurs in patients with a number of diseases, including syphilis, diabetes mellitus, and syringomyelia. This form of osteoarthropathy is discussed under its specific name.