Hypertrophic pulmonary osteoarthropathy is a secondary lesion of the osteoarticular apparatus that develops against the background of chronic inflammatory or neoplastic processes. Hypertrophic pulmonary osteoarthropathy is characterized by deformation of the nail phalanges by the type of “drumsticks”, bone pain, arthralgia, swelling and stiffness of the joints, vegetative disorders (sweating of the palms and feet, pallor of the skin, hot flashes, etc.). Diagnosis of hypertrophic pulmonary osteoarthropathy is based on clinical and radiological data and the identification of the underlying disease. Therapeutic tactics include the appointment of NSAIDs, treatment of a chronic inflammatory focus or surgical removal of the tumor process.
Meaning
Hypertrophic pulmonary osteoarthropathy (HPOA, Marie-Bamberger’s disease) is a paraneoplastic syndrome that includes pin–shaped deformity of the fingers, periostosis of long tubular bones, arthritis of the ankle, knee and wrist joints. There is no reliable information about the frequency of the syndrome; it is known that bronchogenic lung cancer is the cause of 90% of cases of hypertrophic pulmonary osteoarthropathy. Men predominate among patients with HPOA (5:1), the average age of patients is 40-50 years. Secondary damage to the musculoskeletal system can occur in chronic inflammatory and tumor processes of the lungs and mediastinum, therefore, hypertrophic pulmonary osteoarthropathy is of practical interest not only for rheumatology, but also for oncology and pulmonology.
Causes
In the overwhelming majority of cases, hypertrophic pulmonary osteoarthropathy serves as an early marker of peripheral lung cancer and occurs even during the absence of clinical and radiological signs of the oncological process. Also, HPOA can accompany other tumor processes: lymphogranulomatosis, pleural mesothelioma, mediastinal sarcoma, esophageal cancer, atrial myxoma, thyroid cancer, metastases of malignant tumors of various localizations in the mediastinum.
Possible causes of hypertrophic pulmonary osteoarthropathy include inflammatory pathology of the lungs and pleura (bronchiectatic disease, chronic pneumonia, lung abscess, pleural empyema), heart (infectious endocarditis), gastrointestinal tract (chronic hepatitis, ulcerative colitis). In rarer cases, Marie-Bamberger’s disease may be associated with primary biliary cirrhosis of the liver, Crohn’s disease, congenital heart defects of the “blue” type, cystic fibrosis, idiopathic fibrosing alveolitis, hyperthyroidism, etc.
Primary hypertrophic osteoarthropathy (pachydermoperiostosis) should be distinguished from Marie-Bamberger’s disease as a secondary process – a disease with an autosomal recessive type of inheritance characterized by periostosis, pachyderma, CHD, hyperhidrosis, cranial bone defects, early formation of deep wrinkles on the forehead and cheeks.
Pathogenesis
It is believed that the pathogenesis of hypertrophic pulmonary osteoarthropathy is associated with damage to the pulmonary capillaries, as a result of which the process of detaching platelets from megakaryocytes is disrupted, platelet growth factor is released in large quantities, which eventually leads to the occurrence of a periosteal reaction. Toxic effects of inflammatory and tumor factors, violation of vascular tone regulation play a role in the development of arthropathy.
The pathomorphological basis of the deformation of the fingers are: swelling of the soft tissues of the nail phalanges, capillary spasm, fibroblast proliferation and increased collagenogenesis. Periosteal layers are formed mainly in the area of the diaphyses and metaphyses of the long tubular bones, metatarsal and metacarpal bones, middle phalanges. In the early stages of hypertrophic pulmonary osteoarthropathy, the periosteal layers have a smooth or slightly rough surface, but they are quickly ossified, practically merging with the end sections of the bone into a single mass. Simultaneously with periostosis, synovitis phenomena develop with the accumulation of a small amount of transparent exudate.
Symptoms
Pathological changes, and, consequently, clinical manifestations of hypertrophic pulmonary osteoarthropathy accompanying the course of chronic inflammatory diseases, develop gradually, over many months. In oncological processes, on the contrary, the symptoms progress rapidly, outstripping the development of the tumor.
Hypertrophic pulmonary osteoarthropathy is characterized by a club-shaped deformity of the fingers (“Hippocratic fingers”, fingers in the form of “drumsticks”, nails in the form of “watch glasses”). There may be pain in the nail phalanges and bones of the hands. Defiguration of the fingers in almost all cases is combined with bone damage. Clinical manifestations of the periosteal process are ossalgia and increased bone soreness during palpation. The articular syndrome that develops with Marie-Bamberger’s disease resembles that of rheumatoid arthritis. Arthropathy usually involves the interphalangeal, wrist, elbow, knee, and ankle joints. Joint changes (swelling, pain, local hyperthermia, stiffness and restriction of movement) are symmetrical.
Extra-articular manifestations of hypertrophic pulmonary osteoarthropathy may include various vegetative disorders: pallor of the skin, sensations of hot flashes, hyperhidrosis of the palms and feet. Men have a tendency to develop gynecomastia.
Diagnosis and treatment
The main criteria for the diagnosis of Marie-Bamberger’s disease are clinical and radiological data. Distinctive clinical signs of hypertrophic pulmonary osteoarthropathy are thickening of the terminal phalanges of the fingers, polyarthritis of large joints and periostitis of tubular bones. Patients with these symptoms in most cases initially turn to a therapist or rheumatologist.
X-ray examination of tubular bones reveals the phenomena of osteoporosis and periosteal reaction; scintigraphy in these areas determines the pathological accumulation of isotope. Radiography of the fingers of the hand reveals hypertrophy of the distal phalanges, rarely their osteolysis. With the help of ultrasound, in some cases it is possible to detect a small accumulation of effusion in the joint cavity. Examination of the synovial fluid obtained during the diagnostic puncture of the joints reveals the non-inflammatory nature of the effusion.
When obtaining data indicating the presence of hypertrophic pulmonary osteoarthropathy, the diagnostic search should be focused on identifying the latent oncological process. For this purpose, first of all, chest x-ray, CT of the thoracic cavity, ultrasound of the pleural cavity and ultrasound of the mediastinum, as well as, if there are grounds, bronchoscopy, thoracoscopy or mediastinoscopy are performed. As part of the differential diagnosis, other diseases occurring with the phenomena of periostitis should be excluded: psoriatic arthritis, Reiter’s disease, vasculitis, renal osteodystrophy, etc.
The management tactics of patients with hypertrophic pulmonary osteoarthropathy consists of symptomatic therapy and treatment of the underlying disease. Depending on the specific clinical situation, oncologists, thoracic surgeons, pulmonologists, gastroenterologists, cardiac surgeons, endocrinologists, etc. participate in the fate of such patients.
Articular syndrome in most cases lends itself well to therapy with nonsteroidal anti-inflammatory drugs, analgesics. The reverse development of arthropathy and periostosis is noted after radical surgical removal of the tumor of the mediastinum, lungs, pleura or the cessation of the chronic inflammatory process. In the case of a widespread metastatic process, treatment can only be palliative. Hypertrophic pulmonary osteoarthropathy is a secondary syndrome, therefore it has no independent prognostic value. The course and outcome of paraneoplastic syndrome are closely related to the underlying disease.