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Internal Medicine, General Hospital
Sjögren's Syndrome is a chronic, systemic autoimmune disease, the cause of which is unknown. Its main symptoms are a dry mouth (xerostomia) and dry eyes (xerophthalmia). In this disease, the cells making up our defence system (immune system) attack parts of the body itself, such as the glands that keep the eyes and mouth moist, and glands in other parts of the body, altering their function. Symptoms in other organs, such as the lungs, joints or nervous system, may also occur.
Patients with Sjögren's Syndrome often have other conditions related to anomalous functioning of the immune system, such as systemic lupus erythematosus, scleroderma and rheumatoid arthritis.
Patients with Sjögren's Syndrome mainly have symptoms related to a lack of tear and saliva production, which causes dryness in the mouth and eyes. The main symptoms suffered by patients are a burning, gritty sensation in the eyes, red eyes, blurred vision, the need to drink water frequently, difficulty swallowing dry food and a higher propensity for tooth decay and mouth infections. This dryness may also affect the skin and vaginal area, which causes painful sexual relationships. Other symptoms that Sjögren's Syndrome patients may show are joint or muscle pain and fatigue. Less frequently, other organs, such as the lungs, kidney and nerve endings, may be affected.
Above all, the disease affects women aged between 40 and 60. It is calculated that the incidence is between 0.5% and 3% of the population.
There is no single test enabling diagnosis of Sjögren's Syndrome. Diagnosing Sjögren's Syndrome in a patient referred with dry eyes and mouth is based on tests that confirm the existence of a deficit in tear and saliva production and that prove that the dryness is associated with an imbalance in the immune system.
Treatment of Sjögren's Syndrome is essentially based on measures that alleviate the feeling of dryness shown by the patient. The treatment includes the use of artificial tears, eye gels, eye drops, nebulisers and tablets to stimulate saliva production. Particular patients may be given treatment to stimulate glandular secretion. In serious cases, where there are symptoms in organs other than the glands (lungs and peripheral nerves, etc) it may be necessary to administer medication that acts to decrease immune system activity (immunosuppressant drugs).
Patients with Sjögren's Syndrome must undergo a series of eye tests, blood and urine tests and, on certain occasions, it is necessary to do a small biopsy of lip mucous to confirm the existence of inflammation in the glands producing saliva.
Sindrome-Sjogren
Sociedad Española de Medicina Interna
American College of Rheumatology: Síndrome de Sjögren
Hip fractures involve a change in the autonomy of elderly patients, negatively affecting their quality of life and altering their family and social environment, as it causes a change in their prior functional capacities, with a high risk of complications.
The femur is the longest bone in the human body. It should be stated that a femoral fracture in elderly people basically affects the end nearest the hip joint. For this reason, the best-known name for this type of fracture is hip fracture. We should not understand this health problem as a fracture of a long bone that affects an elderly person, but rather as a gateway leading to their physiological and functional situation, and their underlying conditions, becoming decompensated.
The classic symptoms are pain in the hip area, reported by the patient themselves, when they can, along with the associated functional impotence. Furthermore, the affected limb tends to be shorter and presents external rotation (with the lateral side of the foot touching the bed). In patients with cognitive deterioration, who cannot communicate the level of pain they are suffering, a decrease in their functional situation can be a symptom that should make us suspect a “hidden” fracture.
The population incidence of hip fractures in Catalonia for patients aged > 65 is around 600 cases / 100,000 inhabitants per year. The perspective is that this figure will increase in coming years, due to the increase in life expectancy and an increasing population.
At Hospital Vall d'Hebron in 2020, the year of the Covid-19 pandemic, we operated on a total of 693 hip fractures. In 2019, surgery was performed on 720 fractures. In other words, these are fractures that occur under normal circumstances of patients falling over.
The average age where this occurs is 85 years old, and it affects women in 75% of the cases. Some of the risk factors for women may be early menopause and hormonal treatment. A prior fracture (radius or spine are the most frequent), obesity, smoking and a sedentary lifestyle are risk factors in general.
The suspected diagnosis can be made from typical clinical signs and symptoms, as stated above.
In regard to treatment, it has been demonstrated that surgery on hip fractures in the elderly population increases survival, when compared to a non-surgical treatment, as it permits the patient's early movement without pain, thereby avoiding added complications. For this reason, at present, the treatment of choice is surgical treatment. However, there are some exceptions.
A multi-disciplinary approach is important, with the collaboration of specialists in geriatrics, internal medicine and anaesthesiology for the preoperative optimisation of the patient and early surgery, as a rule within the first 48 hours.
It is essential to classify the fractures according to their location, in order to decide on the type of surgical treatment. Intracapsular fractures seriously affect the vascularisation of the femoral head, and for this reason, the most popular option for treatment is to replace it with an artificial head, in the form of a hip prosthesis, especially in displaced fractures. Depending on the patient's age and functional situation, we may opt for a partial or total prosthesis, reserving the second option for younger patients with a better quality of life. Some non-displaced intracapsular fractures can be treated percutaneously, using screws. Extracapsular fractures, of which pertrochanteric fractures are the most common, do not compromise the vascularisation of the femoral head, and for this reason it is not necessary to replace it with a prosthesis. Percutaneous treatment with intramedullary nailing is usually the treatment of choice.
The postoperative period is also very important. Close collaboration with rehabilitation and physiotherapy services is established so that patients do not lose their prior functional capacity. Furthermore, social workers assess the social situation of each patient, in order to ensure an appropriate hospital discharge.
Diagnosis confirmation is usually carried out with a simple x-ray of the pelvis. In cases where the x-ray does not show a fracture, as may occur in the case of some non-displaced intracapsular fractures, tomography (CAT) is usually the technique of choice.
Hip fracture prevention is based on the adoption of healthy habits, such as a balanced diet that is rich in calcium and vitamin D, doing physical activity every day, within the person's functional capacities, and avoiding tobacco and alcohol.
It is also important to prevent falls, by means of appropriate closed footwear with a non-slip sole, standing up slowly, without haste, programming visits to the toilet to avoid rushing, wearing glasses and hearing aids, not walking in the dark or on went floors, etc.
The failure of our immune system to recognise structures, cells or proteins in the body is responsible for the development of systemic autoimmune diseases. We call them ‘autoimmune’ because our immune response mistakenly targets our own body; and ‘systemic’ because they affect different organs and systems such as the lung, kidney or nervous system, in addition to the well-known effects on the musculoskeletal system.
In recent years, improved knowledge of these diseases has made it possible to correctly diagnose autoimmune diseases at an early stage, allowing us to establish appropriate treatment regimens to reduce the damage that they cause.
The primary therapeutic strategy for tackling these diseases revolves around the modulation of the immune system. Various immunosuppressants and the use of molecules targeting key components of the immune system can restore, to some extent, or at least minimise the abnormal immune response associated with these disorders. The considered use of these treatments will make it possible to deliver personalised, precision therapy to patients suffering from these diseases in the near future.
Vasculitis in its different forms (depending on the calibre of the vessels), lupus, antiphospholipid syndrome, scleroderma and inflammatory myopathies are some of the classic systemic autoimmune diseases.
Idiopathic inflammatory myopathies are a heterogeneous group of illnesses whose main feature is muscular weakness and identification of an underlying inflammation in the muscular biopsy. The group includes dermatomyositis, polymyositis and, recently, inclusion body myosotis, which is most probably the least inflammatory, as well being the myopathy most frequently acquired by the over 50s. Although the main target organ is muscle, the skin and lungs, amongst other internal organs, are frequently affected, for which reason inflammatory myopathies are considered to be systemic illnesses.
The most common presentation of these illnesses is muscular weakness, which usually has a characteristic effect on the proximal skeletal muscles, that is to say, on the shoulder and hip girdles, which makes activities needing these muscles to work normally difficult, such as hanging out laundry, combing hair, going up stairs or getting up from a chair. Skin manifestations are characteristic in dermatomyositis and we can identify a wide range of lesions. The majority of them have a certain photosensitivity component which means they usually appear in areas exposed to the sun. Lilac-coloured or heliotrope palpebral oedema are considered to be pathognomic, as are Gottron's papules, which appear on the knuckles of the hands. Similar lesions can be seen in the extension areas, such as elbows and knees, and also on the hair line on the scalp and nape of the neck. Other cutaneous lesions, which are V-shaped on the neckline or shawl-shaped on the back, are also related to the stimulation of the sun.
The most well-known respiratory infection in patients with dermatomyositis and polymyositis is interstitial lung disease. In general, its inception is usually sub-acute or chronic and the clinical findings during the examination can detect dry, crackling rales, in “velcro”, which are characteristic of pulmonary fibrosis. Cardiac and digestive involvement is infrequent and should be assessed in each patient individually.
Inflammatory myopathies can be considered to be within the rare disease group due to their low incidence. Epidemiological studies carried out around the world set out an average annual incidence of 2.1 to 7.7 new cases for every million inhabitants per year.
It is considered to be a universally distributed disease, although it is a little more prevalent in Caucasians and less frequent in black people. It is twice as frequent in women than men. In addition, although a juvenile form exists that usually appears before the age of 16, signs of the illness are more frequent in 30 to 40 year olds.
The diagnosis is made using the convergence of clinical criteria. The most relevant finding in blood tests is elevated muscular enzymes, such as creatine kinase and aldolase, along with acute phase reactors such as the erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). Muscular biopsy is, for some authors, the benchmark test for diagnosing inflammatory myopathies.
In the case of dermatomyositis and, in general, inflammatory myopathies, there are a series of antibodies that help the clinician to classify the disease. Anti-Jo-1, anti-PL-7, anti-PL-12, anti-U1 and anti-PM-Scl, etc, stand out amongst these antibodies. Another complementary test enabling assessment of the extent of the adverse effect on muscles is the nuclear magnetic resonance which, in turn, is valuable when monitoring progress. Depending on whether there is pulmonary, cardiac or digestive involvement, the extra examinations needed to assess each affected organ will be carried out.
Treatment of inflammatory myopathies is based on administering glucocorticoids and immunosuppressants, without forgetting physical therapy or rehabilitation, even in the acute phase. A third of patients respond to a single glucocorticoid treatment, but the majority need the addition of a new immunosuppressant, such as disease modifying drugs, including methotrexate, ciclosporin, cyclophosphamide, azathioprine, mofetil mycophenolate and tacrolimus. Intravenous immunoglobulins may also be used as they act to improve muscle weakness. Biological therapies, such as etanercept, infliximab and rituximab, have been shown to be effective in some clinical cases or observational studies. In any event, the treatment must be individual for each patient.
Miopatías Inforeuma
SLE is a systemic autoimmune disease. Under normal conditions, the immune system produces proteins (antibodies) to protect us from bacteria, viruses, and other foreign substances (what we call antigens). In autoimmune diseases like SLE, the immune system gets "confused" and cannot distinguish between foreign particles and our own cells, so it produces antibodies against our own body, which causes inflammation and damage to different organs.
It being a systemic disease means that it can affect most parts/organs of our body: skin, joints, kidney, lungs, etc. It is a chronic disease that has flares or flare-ups, meaning that it goes through periods where it is more active (flare-ups) and periods of inactivity.
Antiphospholipid syndrome is characterized by the appearance of thrombosis (blood clots) in any area of the body, complications during pregnancy (especially recurring miscarriages and premature births), and the presence of antibodies against phospholipids. Half of the cases of APS are associated with SLE.
SLE can affect almost every organ in the body. The most frequent symptoms are:
The most characteristic clinical manifestations of APS are thrombosis (blood clots) and serial miscarriages.
This thrombosis can trigger deep vein thrombosis in the legs, strokes or brain bleeding, myocardial infarctions, pulmonary thrombosis, ocular thrombosis, etc.
Among the complications that APS may cause during pregnancy, the most common are repeated miscarriages (mostly before week 10 of gestation), although it can also give rise to premature births (before week 34 of gestation).
Apart from the thrombosis and obstetric complications, patients with APS may also present with anaemia, kidney problems, convulsions, arrhythmia, and different kinds of skin lesions.
SLE is a disease that predominantly affects women; one man is diagnosed with this illness for every nine women. It can appear at any age, although most cases manifest between 17 and 35 years of age.
What exactly triggers the disease is unknown. It is believed that an infectious agent may initiate the disease, but at the same time, the individual must present genetic and hormonal factors for this to occur.
APS also affects women (60-80%) more than it affects men. It is unknown exactly why there are individuals who test positive for antiphospholipid antibodies but have never had thrombosis or a miscarriage, while others who are positive do have these issues.
The diagnosis for SLE is clinical and is based on three main aspects: the symptoms the patient reports, the alterations observed on the physical examination, and the results of the blood and urine analysis. There is no single test to diagnose SLE.
There are some abnormalities on a blood test that can make us suspect that a patient may have SLE. It is common for these patients to have a low number of leukocytes, lymphocytes, and/or platelets. We can also detect the presence of antinuclear antibodies (ANAs). Almost 100% of patients with SLE are positive for these, but this does not constitute a diagnosis of the disease, since healthy people or those with other illnesses can test positive.
Diagnosing APS is based on the combination of clinical characteristics mentioned previously (presence of thrombosis or obstetric problems like miscarriages or premature births) and the presence of one or more of the antibodies typically associated with the disease (lupus anticoagulant, cardiolipin antibodies, and anti-beta 2-glycoprotein antibodies).
There is no single cure for SLE, since treatment will vary for each patient based on the clinical manifestations they present. It is usually a long-term, chronic treatment. Generally speaking, anti-inflammatory agents and corticosteroids are prescribed to treat flares and immunosuppressants (hydroxychloroquine, methotrexate, azathioprine, mycophenolate, etc.) are used, based on the areas/organs affected, to treat and prevent new flare-ups. Currently, we only have one biological pharmaceutical approved for SLE, belimumab, although numerous studies are underway that aim to find new, effective drugs to treat this disease.
Treatment for APS mainly includes the administration of an anti-clotting treatment (aspirin) or anticoagulant, based on the clinical manifestations and antibody profile of the patient.
The most common diagnostic test used for suspected SLE and APS is the blood analysis, which includes testing for auto-antibodies.
Other studies and tests can also be used, based on the patient's symptoms: urinalysis to evaluate whether the kidney is affected, chest X-rays and echocardiogram when the heart or lungs are involved, renal biopsy if kidney problems are detected, skin biopsy, head CT or MRI if neurological symptoms appear, etc.
There is no specific measure we can take to prevent the appearance of SLE and APS.
Once manifested, an early diagnosis is essential for both illnesses in order to begin treatment quickly and thus avoid possible complications. Patients must regularly see and work in close collaboration with their specialists.
In addition, for both SLE and APS, it's very important to strictly control the classic cardiovascular risk factors (high blood pressure, diabetes, dyslipidaemia, obesity, and tobacco use), because they can very negatively affect the prognosis of both diseases.
Lupus-Eritematoso
American College of Rheumatology: Lupus
American College of Rheumatology: Síndrome Antifosfolípido
American College of Rheumatology: Tratamientos
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