What is Hypertrophic Pulmonary Osteoarthropathy (Marie-Bamberger Disease)?
Hypertrophic pulmonary osteoarthropathy (Marie-Bamberger’s disease) is a lesion of the musculoskeletal system, manifested by the deflection of fingers in the form of drum sticks, hypertrophic perostomatism of predominantly long tubular bones, arthralgias or arthritis with effusion into the joint cavity.
Causes of Hypertrophic Pulmonary Osteoarthropathy (Marie-Bamberger Disease)
The symptomatology of this process is considered one of the most important signs of bronchogenic lung cancer, especially of the peripheral form. Sometimes hypertrophic osteoarthropathy appears many months before the development of clinical and radiologic symptoms of cancer. Its frequency in bronchogenic lung cancer, according to S. A. Reinberg, is 7.3%. It was also noted that 90% of all hypertrophic osteoarthropathies are caused by bronchogenic cancer. Other possible causal factors in the development of hypertrophic pulmonary osteoarthropathy should be indicated for lymphogranulomatosis or metastases of sarcoma in the mediastinum, tumors of the pleura, gastrointestinal tract, chronic inflammatory processes in the lungs, congenital “blue” heart defects, infective endocarditis, liver cirrhosis, ulcerative colitis, etc. specific changes of the fingers and hyperplastic process of the periosteum, apparently due to the toxic effect of a hypothetical humoral tumor factor, breaking by the vegetative nervous system.
The pathoanatomical basis for the formation of “drum” fingers are soft tissue swelling, narrowing of blood vessels, proliferation of fibroblasts and collagen proliferation. The bone structure of the nail phalanges changes only in extremely difficult cases, when they become thinner and even completely resorbed. The periosteum is affected mainly in the terminal sections of the long tubular bones, as well as the metacarpal, metatarsal bones, the main and middle phalanges. Periosteal stratifications surround the bone on all sides, have a smooth or slightly rough surface. They are quickly ossified, with a narrow, dense strip clearly visible on radiographs, separated from the compact cortical substance of the diaphysis by a light gap. During the long process, the periosteal layers are fused with the diaphysis of the bone into a single bone mass. Histologically, in the periosteal layers in the early stages of the process, the vascular network is strengthened, there is edema and lymphoid infiltration.
The synovium near the affected bones is swollen, infiltrated with a small number of lymphocytes, its vessels are overflowing with blood, and the lumen is sometimes obliterated; there is a mild proliferation of epithelial cells of the synovial membrane. A transparent yellowish fluid may accumulate in the joint cavity.
Symptoms of Hypertrophic Pulmonary Osteoarthropathy (Marie-Bamberger Disease)
Pathological changes characteristic of hypertrophic osteoarthropathy in chronic purulent lung diseases develop imperceptibly over many months and years; on the contrary, with oncological diseases they progress rapidly with the prevalence of articular syndrome. The change in the distal phalanges of the fingers in the form of drum sticks and the shape of the nails according to the type of watch glass is almost always combined with the defeat of the bones. The soreness of the changed fingers during palpation is not detected, except in those rare cases when the deformities occur extremely quickly. The periosteal process, as a rule, is clinically asymptomatic, although it may be manifested by salvage and bone soreness on palpation.
The lesion of the joints develops gradually. Often involved in the process of the knee, ankle, wrist and elbow joints. There is pain, swelling of the joints, usually symmetrical, as are symmetric and bone changes. The affected joints may be warm to the touch, painful on palpation, and movement in them is limited. In some patients with a long process affecting phalanges, changes may occur in the interphalangeal joints, which, together with possible morning stiffness, which sometimes lasts for an hour or more, makes the process very similar to RA.
Of the common symptoms should be noted pronounced sweating of the hands and feet, pallor, hot flashes, a tendency to the development of gynecomastia. An increase in ESR is found, as a rule, in patients with bronchogenic lung cancer. There are no specific laboratory tests for the disease.
Synovial fluid in hypertrophic pulmonary osteoarthropathy is usually transparent, of normal viscosity, contains no more than 1000 cells per ml, among which mononuclear cells predominate. The rheumatoid factor is not detected either in it or in the blood serum.
Diagnosis of Hypertrophic Pulmonary Osteoarthropathy (Marie-Bamberger Disease)
The diagnosis is made on the basis of a characteristic clinical and radiological picture. In all cases of hypertrophic pulmonary osteoarthropathy, especially in the elderly, it is necessary to exclude malignant neoplasms and, above all, the organs of the chest.
With negative clinical and radiological results, it is advisable to repeat studies to identify the underlying disease as soon as possible. It is necessary to bear in mind the possibility of the innate nature of the “drum fingers”. In such cases, the deflection of the terminal phalanges is detected from early childhood, without being accompanied by pain. It is also necessary to exclude idiopathic hypertrophic osteoarthropathy, known as pachydermoperiostasis. This family disease develops gradually, usually in people of young and mature age, more often in men, and is manifested not only by changes in the bone skeleton, but also by compaction and thickening of the skin of the face, early appearance of deep wrinkles, especially on the forehead and cheeks. Sometimes the face resembles the face of a patient with leprosy.
R due to the simultaneous increase in the hands and feet, sometimes an incorrect diagnosis of acromegaly is made.
“Drumsticks” can be formed as a symptom of acropathy in patients with impaired thyroid function, acromemegaly, not combining in such cases with bone lesions (periostitis). However, periostitis can occur in many diseases, for example, in psoriatic arthritis, Reiter’s disease, vasculitis, renal osteodystrophy, and others, which must be considered when recognizing the nature of the process.
Treatment of Hypertrophic Pulmonary Osteoarthropathy (Marie-Bamberger Disease)
Joint processes respond well to treatment with acetylsalicylic acid, indomethacin and other non-hormonal drugs. Extremely rapid (within the next 24 hours) reverse development of the process, as well as the resorption of sometimes massive periosteal layers within a few months, has been described during surgical removal of a lung tumor or successful radical treatment of a chronic inflammatory focus in them. Isolated drumstick syndrome usually does not require therapy.