Forestier's disease, or ligamentosis ossificans, has been known for a long time, but only in 1950, after a detailed description of it by J. Forestier and J. Rots, it began to be called Forestier's disease , although the authors of the description themselves proposed calling it senile ankylosing hyperostosis of the spine. This is due to the fact that the disease is non-inflammatory, but slowly progressive in nature and is externally similar to spondylosis. But unlike the latter, it is not based on excess production of bone tissue, and at the same time, the concept of “ligamentosis” does not accurately reflect the pathomorphology, since it is not based on dystrophic damage to the longitudinal ligaments of the spine.
Since the 70s, the concept of Forestier's disease has not been limited only to local damage to the anterior longitudinal ligament of the spine, but has begun to be interpreted as a more generalized pathological process that also affects the peripheral parts of the skeleton. In this case, a gradual metaplasia of the elements of the connective tissue (ligaments) itself into cartilaginous tissue occurs, this process is accompanied by the phenomena of cystic degeneration, mainly in the places of fixation of tendons and ligaments to the bones with the deposition of calcium there and the subsequent formation of osteophytes, widely known as “spurs” (calcaneal “ spurs” osteophytes of the patellar ligament, osteophytes in places where muscles are attached to the iliac crests, etc.)
Introduction
Back pain is an interdisciplinary medical problem, however, such patients primarily turn to internists and general practitioners, who are responsible for timely differential diagnosis and prescription of a complex of therapy in cases where back pain is not a manifestation of a serious or life-threatening disease .
However, the prescribed therapy is not always successful, which may be due to an insufficient diagnostic search [1]. Thus, osteochondrosis or a disc herniation detected during magnetic resonance imaging (MRI) can mask diseases of an inflammatory, infectious, metabolic, or oncological nature. Assessing “threat symptoms,” or “red flags,” will allow the physician to identify the presence of a disease that requires a slightly different approach to therapeutic interventions.
This article presents a description of a clinical case - management of a comorbid patient with chronic pain syndrome.
Causes
The initiating factors for the development of hyperostosis are considered to be:
- neuroendocrine disorders;
- frequent intoxication;
- age-related changes in connective tissue;
- chronic infectious diseases.
Causes of Forestier's disease
Pain syndrome and ankylosis can be observed in people after 45-50 years of age, and most often such changes were observed in their parents. It is believed that genetic factors are of greatest importance in the development of pathology. Studies have shown that a third of patients had the following antigen: human leucocyte antigen - HLA B27 , the gene of which is responsible for bone formation.
Clinical observation
Patient E., 61 years old, complained of pain in the lumbar and thoracic spine, which appeared and intensified after physical activity, stiffness of the spine after physical activity or prolonged stay in one position, and a decrease in height by 7 cm over her life. The patient also noted pain in the joints of the lower extremities, mainly of a mechanical nature, and pain in the arch of the left foot.
From the anamnesis it is known that episodes of pain in the lower back have been bothering me since the age of 50. At the onset, the pain was acute, lasting up to 1–2 weeks. She was observed by a neurologist at her place of residence with a diagnosis of osteochondrosis; during exacerbations, short courses of therapy with nonsteroidal anti-inflammatory drugs (NSAIDs) and B vitamins were administered, which helped relieve back pain.
Over the past 8–10 years, I have been experiencing aching pain in the knee and hip joints, provoked by physical activity. Swelling of the knee joints was repeatedly noted, accompanied by morning stiffness for up to 30–60 minutes; symptoms of synovitis were relieved by taking NSAIDs. Damage to large joints of the lower extremities was regarded as osteoarthritis of the knee joints in combination with reactive synovitis.
The patient suffers from arterial hypertension and angina pectoris, for which she takes antihypertensive and antianginal drugs.
Due to pain in the spine and recurrent synovitis of the knee joints, she was sent for consultation to a rheumatologist with a referral diagnosis of “unspecified spondyloarthritis.”
During the examination, attention was drawn to excess body weight (body mass index 40.7 kg/m2), varicose veins of the lower extremities, pronounced thoracic kyphosis, limitation of movements in all parts of the spine (rotation in the cervical spine within 30° in both directions , distance “back of the head - wall” - 5 cm, chest excursion - 2 cm, Schober test - 2 cm). When assessing the local status - deformation in the area of the interphalangeal joints of the hands with the formation of Heberden and Bouchard's nodes, slight swelling of the knee joints, as well as in the area of the tarsal joints of the left foot, without signs of local hyperemia and hyperthermia.
Taking into account the presence of back pain, swelling of the knee joints and tarsal joints, further examination was carried out to exclude the disease from the group of spondyloarthritis. According to laboratory research methods: ESR - 33 mm/h (according to Westergren), C-reactive protein - 8 mg/l, rheumatoid factor - 2.0 U/l, HLA B27 - not detected. A general urine test and a biochemical blood test were unremarkable, with the exception of an increase in serum creatinine to 120 µmol/l.
An X-ray of the pelvic bones including the hip joints (Fig. 1) revealed subchondral osteosclerosis of the articular surfaces of the sacrum and ilium, narrowing of the joint spaces of the sacroiliac joints (SIJ), initial signs of bilateral coxarthrosis, ossification of the greater and lesser trochanters of the femurs, periostosis (enthesophytes ) tuberosities of the ischial bones, wings of the ilium.
Radiographs of the cervical (Fig. 2), thoracic (Fig. 3), lumbar (Fig. 4) spine revealed straightening of the cervical lordosis, pronounced subchondral sclerosis of the endplates of the vertebral bodies in the midthoracic region, a decrease in the height of the intervertebral discs, wedge-shaped deformation of the vertebral bodies in mid-thoracic region, massive marginal bone growths of the anterior corners of the bodies of the cervical, thoracic and lumbar vertebrae with massive calcification of the anterior longitudinal ligament in all parts of the spine along the entire length with coracoid growths in the cervical and mid-thoracic, lower thoracic regions, arthrosis of the facet joints. An ultrasound examination of the knee joints revealed only signs of suprapatellar bursitis on both sides.
Old age, evidence of back pain, the inability to remain in an upright position for a long time, and a decrease in height by 7 cm over a lifetime required caution regarding the exclusion of postmenopausal osteoporosis. According to a biochemical blood test: calcium - 2.19 mmol/l, phosphorus - 0.98 mmol/l, alkaline phosphatase - 219 U/l. The protein profile is unremarkable. X-ray densitometry of the central parts of the skeleton revealed a significant increase in mineral density (BMD) both in the lumbar spine (BMDL1-L4–1.908 g/cm2, T-criterion - 5.8 standard deviations) and in the proximal femur (BMD - 1.340 g/cm2, T-criterion - 2.6 standard deviations). Thus, no laboratory and instrumental data indicating osteoporosis were obtained.
Taking into account the absence of complaints of inflammatory pain in the spine, increased acute-phase blood parameters, arthritis of the knee joints, identified degenerative-dystrophic changes in the SIJ, ossification of the anterior longitudinal ligament of the spine in all parts of the spine, multiple peripheral hyperostoses, according to radiographic examination, it seemed most likely diagnosis of ankylosing diffuse idiopathic skeletal hyperostosis (Forestier disease).
In 1950, the French rheumatologist J. Forestier (in collaboration with J. Rotés-Querol) described in detail not only the radiological changes characteristic of ankylosing hyperostosis of the spine, but also the clinical picture of the disease, which is now called “Forestier disease”. A quarter of a century later, American radiologist D. Resnick published an article in which he showed that ankylosing hyperostosis of the spine is only one of the manifestations of systemic (generalized) ectopic ossification of ligaments, tendons and other connective tissue structures of the musculoskeletal system, and proposed a new term to designate this pathology - “diffuse idiopathic skeletal hyperostosis” [2]. D. Resnick and G. Niwayama in 1982 presented diagnostic signs of diffuse idiopathic skeletal hyperostosis, which were based on radiological data [3]. To make a diagnosis of this disease, all 3 signs must be present:
continuity of ossification of the anterior longitudinal ligament over at least 4 adjacent spinal segments;
absence (or weak severity) of signs of degenerative changes in the intervertebral discs, such as preservation of their height, absence of the vacuum phenomenon and marginal sclerosis of the vertebral bodies in those segments where the anterior longitudinal ligament of the spine is ossified;
absence of radiological signs of ankylosis of the facet joints and sacroiliitis.
Despite quite pronounced radiographic changes, complaints and objective examination data from the spine in diffuse idiopathic skeletal hyperostosis are nonspecific and are observed no more often than in elderly people of the same age, but without ossification of the anterior longitudinal ligament of the spine [4]. Thus, there is no direct relationship between the clinic and the x-ray picture.
Patients may complain of discomfort, stiffness, and, less commonly, pain in the thoracic spine, which intensifies in the morning after sleep, at the end of the working day, after physical activity, prolonged stay in one position, or in the cold. If the process spreads to the cervical and lumbar spine, these sensations may also be noted in these sections. The severity of pain ranges from mild to severe. Often, especially in old age, there may be no complaints [5].
Back fatigue, changes in posture, i.e. a rather poor clinical picture, allow one to suspect osteoporosis, which requires additional research. Therefore, if the disease seems asymptomatic, special attention should be paid to the significant thickening of the anterior and posterior longitudinal ligaments in the cervical spine. Severe ossification of the anterior longitudinal ligament can lead to compression of the esophagus, larynx, trachea and the development of dysphagia and dysphonia [6–8], paresthesia or a sensation of “lump in the throat” [9], and the posterior longitudinal ligament can lead to the threat of compression of the spinal cord with the development of myelopathy , paresis and paralysis [4].
In Forestier disease, ossification of the anterior longitudinal ligament always begins in the thoracic region (and in 99% of cases - in the middle and lower thoracic regions and on the right), as a result of which, to identify such changes, it is enough to perform an x-ray of the thoracic spine in a lateral projection. This will allow one to clearly see the rough ossification of predominantly the anterior longitudinal ligament. Later, the lumbar and cervical spine are involved in the process.
According to N.V. Bunchuk, X-ray changes in the spine in diffuse idiopathic skeletal hyperostosis are often interpreted as a picture of a “bamboo stick”, typical of ankylosing spondylitis [4]. Previously, it was mistakenly assumed that syndesmophytes in ankylosing spondylitis are formed due to ossification of the anterior longitudinal ligament. It has now been established that the formation of syndesmophyte in ankylosing spondylitis begins with inflammation of the attachment sites of the fibers of the fibrous ring of the intervertebral disc to the bodies of the above and underlying vertebrae, i.e., with enthesitis. Subsequently, chondroid metaplasia and ossification of the outer layers of the fibrous disc occurs, the front of which gradually spreads from the vertebral bodies to the middle of the disc. Thus, syndesmophytes typical for ankylosing spondylitis are located inside the disc and usually do not protrude beyond the lateral contours of the vertebrae (Fig. 5).
Ossification of the ligaments located in the posterior parts of the SIJ, as well as their capsule, can simulate partial ankylosis of these joints on a radiograph. Ossification of the SIJ ligaments in degenerative pathology (like arthrosis) up to ankylosis is observed only in the anterior sections.
The main differential diagnostic signs of diffuse idiopathic skeletal hyperostosis and ankylosing spondylitis are presented in Table 1.
In the treatment of spondyloalgia resulting from ankylosing hyperostosis of the spine, local thermal procedures and massage of the back muscles are usually effective. Regular exercise is helpful to maintain adequate mobility of the spine. The use of NSAIDs, effective for ankylosing spondylitis, in diffuse idiopathic skeletal hyperostosis leads to only a moderate reduction in pain [10]. Knowing this will allow you to avoid uncontrolled use of NSAIDs, given the likelihood of side effects. For the treatment of “peripheral” manifestations of the disease, the cause of which is reactive inflammation of structures undergoing calcification and ossification (enthesopathies, tendonitis, ligamentitis), according to N.V. Bunchuk, local methods of influence should be used, for example, injection of glucocorticoids under the control of ultrasound navigation [4].
Diet for Forestier hyperostosis
Hypocaloric diet
- Efficiency: 3-6 kg in 30 days
- Terms: 14-30 days
- Cost of products: 1500-1600 rubles. in Week
The fundamental factor in improving the condition of patients with hyperostosis is weight normalization, which can be achieved through a lifelong diet. Most often, a hypocaloric diet is prescribed, which can reduce the number of calories consumed, but at the same time provide the body with all the necessary substances and vitamins. It is recommended to add more to your diet:
- fresh vegetable and fruit salads;
- light soups;
- flour products made from whole grain or wholemeal flour;
- porridge;
- seafood;
- teas and compotes.
It is advisable to completely eliminate alcohol, flour, sweets, fried and fatty foods, as well as harmful foods such as smoked meats, canned food, semi-finished products, fast food, etc.
Treatment
There are no specific therapeutic measures for this disease. If there is discomfort, massage, physical therapy, heat treatment, and physiotherapy are prescribed. The patient is recommended to visit balneological hospitals.
If there is pain, use NSAIDs (non-steroidal anti-inflammatory drugs) - indomethacin, diclofenac and others. It should be remembered that these drugs have a pronounced ulcerogenic effect (promote the formation of ulcers), so they should not be taken by patients with peptic ulcers.
Massage is used as an additional treatment method
If pain occurs in the peripheral parts of the skeleton, infiltration of damaged areas with anesthetics and glucocorticosteroids is prescribed. Phonophoresis is carried out with hydrocortisone, applications are made with anti-inflammatory ointments and dimexide. In general, the prognosis for life is favorable if the posterior spinal ligament is not affected.
What is ankylosing spondylitis
This term refers to chronic progressive joint disease. In 80% of cases it begins with back pain (intervertebral joints are damaged), much less often with peripheral arthritis in the joints. The inflammation begins where tendons and ligaments attach to bone and spreads to the small joints between the vertebrae. Gradually the bones fuse and the spine slowly turns into one solid bone.
Since even the ligaments ossify, the person completely loses flexibility and cannot move. The inflammatory process spreads to the peripheral joints, which may already be affected by arthrosis, as well as to the eyes, lungs, kidneys and heart. Without timely treatment, the prospects are very sad.
When should you see a doctor?
If a person suddenly experiences numbness or tingling in the shoulder, arms or legs, or if the person has lost bowel or bladder control, then seek medical help as soon as possible!
If discomfort and pain begin to interfere with daily activities, it is necessary to consult a neurologist. Despite the fact that spondylosis is part of involutional changes, there are nevertheless various treatment methods that can improve well-being and reduce symptoms.
Physical examination
First, the doctor finds out the presence of symptoms, their intensity, location, and history of the disease. A neurological examination is then performed, which includes examining reflexes, testing muscle strength, and determining sensory deficits and range of motion in the neck. A doctor may perform a gait analysis to determine how much damage to the spinal cord is present.
If a doctor suspects cervical spondylosis, he will order imaging tests and neurophysiological studies to verify the diagnosis.
Development mechanism
The anterior longitudinal vertebral ligament can, under some circumstances, produce bone tissue. This occurs when the ligament is torn from the place of fixation, resulting in transformations called spondylosis. In Forestier disease, the ligament also begins to produce bone tissue. The pathological process develops near the intervertebral discs and can affect several levels of the spine at once.
As the bone tissue grows, the ligament begins to move away from the spinal column, while covering it. These bone layers grow tightly together with the vertebrae, cover them from the sides and in front and ultimately deprive them of mobility. First, with Forestier's disease, the thoracic region is damaged (mainly in the middle on the right), then the ossification process spreads to the remaining segments.
Active ossification is observed in the lumbar region (mainly on the left): massive growths are formed next to the intervertebral discs, which emanate from the bodies of neighboring vertebrae and go around the discs. Sometimes this phenomenon is mistaken for spondylosis. The movement continues until complete fusion has occurred. The disease progresses with age.
X-ray shows significant ossification along the spine
Diagnostics
On X-ray, hyperostosis of the spinal column at the initial stage of the disease is similar to the manifestations of spondylosis. However, in the latter case, ossification affects only one level and soon stops. Symptoms of Forestier disease progress steadily. At an early stage, the disease cannot be distinguished from spondylosis and osteochondrosis if we consider only one level and in a direct projection. The task is facilitated by assessing all parts of the spinal column, and in lateral projections.
With spondylosis and osteochondrosis, only structures located at the level of the intervertebral discs undergo ossification. In this disease, ossification occurs both at the level of the disc and at the level of the vertebral bodies. At a later stage, powerful bone formations are visualized, covering the vertebrae from the sides and in front. The thickness of the bone layer can reach one and a half centimeters.
Radiography is the most reliable and accessible diagnostic method
Symptoms of previously existing osteochondrosis remain: decreased disc height, the presence of marginal osteophytes, subchondral osteosclerosis. These formations cover bone layers; the height of the discs at this stage does not change.
In advanced cases, x-rays show how the spinal column is surrounded on the sides by ribbon-like bone layers, as if flowing down along the spine. The formations curve at the level of the discs and come into contact with the vertebral bodies. The spinal column in some sections becomes as if constrained by the formed bone layers.
Features of clinical manifestations at different levels of the spine:
- when the cervical spine is affected, the layers are visualized not as a continuous strip, but as separate fragments in the interval C3–T3;
- the lesion at chest level is most pronounced in the middle and on the right side;
- The entire length of the lower back is affected, but ossification first occurs on the left side.
Laboratory tests have no diagnostic value. There are no signs of inflammation in the blood; hyperglycemia may be observed. The diagnosis is made according to X-ray examination.