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Human immunodeficiency virus (HIV) is a retrovirus, made up of two copies of single-chain RNA enclosed within a capsid. It is transmitted by blood and genital secretions (unprotected sex) and from mother to foetus during pregnancy or birth or through breast-feeding (where the mother does not have her infection controlled). HIV is NOT transmitted through other channels, such as objects, insects or physical contact without sharing blood or secretions.
HIV can be prevented by using condoms during sex and by not sharing any materials that may contain infected blood.
HIV infects a particular type of the body’s defences, CD4+ lymphocytes. It reduces the number of its host's lymphocytes, thereby increasing the latter’s risk of suffering certain infections from micro-organisms (bacteria, viruses, fungi and parasites) that normally do not cause problems when the immune system is working correctly; these are known as opportunistic infections. In addition, the virus infects the body’s other cells and remains in a latent state in areas such as lymphatic ganglia and intestinal mucus. This latent virus is known as a viral reservoir and is one of the main obstacles to curing this infection.
Acquired immunodeficiency syndrome (AIDS) is diagnosed where the number of CD4+ lymphocytes drop below 200/μl or one of the syndrome's defining diseases (infections or neoplasia) appears. It is for this reason, and for the sake of preventing new infections, that early diagnosis of the infection is very important. Anyone who has been in a risk situation should be tested for HIV (and other STDs), irrespective of the presence or absence of symptoms. Having any other STD raises the risk of acquiring and transmitting HIV.
Acute infection with HIV can manifest itself non-specifically, like any other viral infection such as the flu (fever, general malaise, skin rash, swollen lymph glands, pain in the joints or in swallowing, fatigue etc.,) or may be completely asymptomatic.
Once the infection has become chronic, a variable period of time passes during which patients may be completely without symptoms but can transmit their infection. As the (CD4+) defences drop, clinical symptoms may appear with the associated pathologies, whether infections or neoplasias, which can affect several organs/systems.
Anyone who is sexually active runs the risk of being infected by HIV if they do not know the state of health of the person they are having sexual relations with and do not take the following precautions: use of condoms or pre-exposure prophylaxis (PrEP: taking a combination of two anti-retroviral medicines without being infected with HIV, to prevent such infection in the event of coming into contact with the virus). Fortunately, the risk of transmission through other channels, such as blood or mother to foetus, has dropped significantly in our environment, thanks to harm-reduction and HIV-screening programmes for pregnant women and blood and organ donors, among other measures.
HIV is diagnosed in the laboratory by detecting antibodies the patient creates against the virus (but which are not used for neutralising the virus and curing the infection and which remain positive for life as a marker of the infection) and the direct detection of parts of HIV, whether the virus’ antigens or by determining the number if HIV particles that are circulating through the bloody (viral load). Note that there is a period of time between the virus’ entry into the body and the detection of these antigens/antibodies during which all tests are negative, known as the window period. Today’s new techniques have reduced this period to 2-3 weeks after infection.
The recommendation these days is for all patients infected with HIV to start anti-retroviral treatment irrespective of the number of CD4+ lymphocytes or viral load. The only exception would be elite controllers, that is, people whose viral load remains undetectable without treatment. For all other infected individuals, treatment is started with patients as soon as they are ready to receive it and have the necessary information for choosing the best option possible in each case. An effective treatment makes the viral load undetectable, although it does not eliminate HIV from the body. The immune system can therefore remain intact/recover, reducing the possibility of new infections. In fact, when the virus is undetectable in the blood thanks to this anti-retroviral treatment, the infection is not transmitted to other people (undetectable=untransmittable).
There are various families of medicines that act at several points in the HIV life cycle, halting its replication within the body. So we now have analogue and non-analogue nucleoside reverse transcriptase inhibitors, integrase inhibitors, protease inhibitors and entry inhibitors.
Anti-retroviral treatment is currently administered in pills or in the form of long-acting injectable medicines. Standard treatment involves a combination of 2 or 3 different medicines, which can often be combined in two pills or a single tablet. Today's anti-retroviral treatment is for life, given that, if patients stop their treatment, their latent HIV reservoir will re-activate and replicate. Depending on the drugs patients are taking, the possibility of interactions with any other medications they may receive needs to be monitored and a follow-up analysis or specific explorations may be necessary for certain drugs.
Today, HIV infection has become a chronic illness and, with the current treatment, people diagnosed with it now have a life expectancy similar to that of the general population. If someone infected with HIV performs their controls correctly and takes their anti-retroviral medication they can lead a completely normal life, and that includes having children without transmitting their infection to them. Routine visits are made to monitor the infection, usually every 3 to 6 months, during which the number of defence cells (CD4+ lymphocytes) and viral load are measured.
That analysis also measures other parameters to monitor any other pathologies which patients may have (blood count, renal function, liver function, lipids). In addition, a series of specific complementary explorations may also be performed, such as early detection of STDs, screening certain neoplasias (cervix, anus), osseous pathology and so on. People living with HIV can also be given advice on certain preventive measures, such as taking vaccinations against influenza and invasive pneumococcal disease.
The inflammation that the virus’ replication in the body causes also increases the risk of suffering diseases we find in the general population, such as cardiovascular, liver, renal and neurological pathologies and certain cancers, which may appear more severely or at younger ages. That is why it is very important for people living with HIV to control conventional risk factors and adopt healthy life habits.
As an STD, HIV infection can be better treated through early detection and prevented through the use of barrier methods during sex, basically male or female condoms. As mentioned above, the last few years have seen studies on the use of PrEPs as a prevention strategy. This strategy has proven to be highly effective in preventing HIV infection, although it has the disadvantage, unlike using condoms, that it does not protect users from other kinds of STDs.
A person who has been exposed to HIV can also undergo post-exposure prophylaxis (PEP), which involves being administered 3 anti-retroviral drugs for 28 days, although this will have to start within the first 72 hours after exposure to the virus.
Infectious Diseases, Pharmacy, Preventive Medicine, Gynaecology/Obstetrics, Internal Medicine, General Laboratory, Microbiology, Immunology, Neurology, Pneumology, Rheumatology, Hepatology, Oncology, Haematology.
Chronic hepatitis usually takes a silent course and causes inflammation of the liver without presenting any serious symptoms.
Whatever the cause of the hepatitis, serious inflammation may overwhelm the capacity of a patient's liver to regenerate. When that happens, scars may appear (known as fibrosis). Where a patient has numerous scars on their liver, this is known as hepatic cirrhosis. Not all cases of hepatic cirrhosis are caused by alcohol abuse.
Chronic hepatitis is an inflammation of the liver that lasts longer than six months. Frequent causes of chronic hepatitis are:
Two thirds of patients show no symptoms of the illness by the time they have developed hepatic cirrhosis. It is at this stage they may present cirrhosis-derived symptoms such as:
Hepatitis B and C viruses are most often transmitted sexually or through intravenous injections among drug addicts. They may also be transmitted from mother to child during birth. Infection through blood transfusion is very controlled these days and practically never occurs
Toxic hepatitis is caused by exposure to toxins, some well known, others by an unexpected reaction to medicines that have no adverse effect on most of the population (idiosyncrasy). Alcohol abuse is the most common toxin.
Hepatic steatosis is directly linked to obesity among the general population.
Diagnosis is based on three sets of features.
1. Family, personal and case histories
Patients suffering from chronic hepatitis usually present histories that help with the diagnosis, such as alcohol abuse or intravenous drug injections, use of certain medicines, being a child of a mother with HCV or HBV, or obesity. As for people with autoimmune hepatitis, they or their direct family members may present other autoimmune illnesses (such as diabetes, ulcerative colitis, lupus, vitiligo.)
2. Physical examination
Patients may show characteristic signs of portal hypertension (ascites, spider angiomas, reddening of the palms of the hands, collateral circulation in the abdomen). In the case of non-alcoholic steatohepatitis, patients are overweight/obese.
3. Complementary examinations:
- General analysis: Analytical tests can be taken to reveal inflammation of the liver (transaminases) and loss of its synthetic (coagulation and albumin tests) and purification (increased ammonium) functions. At the same time, a systemic detection can be made of the cause of the inflammation (viral serologies in cases of suspected virus illness, autoantibodies and immunoglobulins for autoimmune hepatitis, copper in urine and caeruloplasmin for Wilson’s disease, etc.)
- Imaging tests (abdominal ultrasound and CT scan) show the presence of a heterogeneous liver with probable fibrosis. Nodular margins and indirect signs of portal hypertension (collateral circulation, splenomegaly, etc.,) can be seen with patients presenting hepatic cirrhosis.
- Elastography may reveal the presence of hepatic fibrosis and determine its severity.
- Hepatic biopsy: may be used for helping with a differential diagnosis (accumulation of copper in Wilson's disease, interface hepatitis in autoimmune hepatitis, macrovesicular steatosis in non-alcoholic steatohepatitis, etc.) It will also reveal the extent of the hepatic fibrosis/cirrhosis.
Treatment will depend on the cause of the chronic hepatitis.
- Viral hepatitis; hepatitis B and C viruses require specific antiviral treatment. In the case of hepatitis C, new direct-acting antivirals have radically changed the prognosis for patients, so that it is now a disease which is curable with few side effects.
- Hepatic steatosis requires a change of patient lifestyle (balanced diet and exercise). Several pharmacological treatments are currently being studied which could help to lessen the build-up of fat in the liver.
- Autoimmune hepatitis; this has a specific treatment where the defence system is modulated with corticoids and Azathioprine.
- Wilson's disease; this is an illness that causes copper to build up in the liver and other organs. Treatments are aimed at increasing the elimination of copper through urine (D-penicillamine) or at reducing its absorption (zinc salts)
Mainly analytical tests for diagnosing the cause of the inflammation of the liver and evaluating its dysfunction, and elastography to assess the extent of fibrosis.
Foetal Alcohol Syndrome Disorder (FASD) is characterised by cognitive, behavioural and physical problems caused by exposure to alcohol during pregnancy.
FASD may result in physical symptoms (such as facial abnormalities), growth retardation, damage to the nervous system and cognitive and/or behavioural problems. 90% of people with FASD suffer from psychological disorders, attention deficit hyperactivity disorder (ADHD) being the most common.
The main symptoms of FASD are poor memory and attention span, learning difficulties, problems with recognising cause and effect and lack of social skills and emotional self-regulation. These issues may lead to secondary complications such as poor academic performance, legal issues, inappropriate sexual behaviour, substance abuse and problems finding employment as an adult.
Babies exposed to alcohol in the womb.
FASD diagnosis requires not just physical examination but also neurocognitive and behavioural assessment.
Treatment for FASD is multidisciplinary and often requires a combination of psychology and pharmacology. The psychological approaches shown to be most effective are based on training in social skills, emotional self-regulation and guidelines for parents on how to manage the conflicts involved in having a child with FASD. Appropriate interventions for FASD also involve relevant adjustments to the child’s education.
Psychological monitoring should include both the patient and their parents or guardians. The psychological treatments available include: group treatment (for teenagers and parents), one-to-one psychological treatment and assisted therapy with dogs.
Interventions are based on the age of the child/teenager and their cognitive difficulties. Before any psychological intervention, a neuropsychological assessment must be performed to indicate which cognitive functions the patient has most difficulty with. Treatment can then be adapted to their abilities and carers can manage their expectations and adapt the child/teenager’s environment according to their behaviour.
Clinical history. Psychological interview. Neuropsychological examination. Physical examination and in some cases MRI and EEG tests.
The best way to prevent FASD is to avoid drinking alcohol during pregnancy. Patients with this syndrome have the best prognosis when diagnosed early (before 6 years old) and within a stable family environment.
Your GP or paediatrician can refer you to the Psychiatry Department.
Nuria Gómez-Barros
Raquel Vidal Estrada
Ana Maria Cueto González
Poliomyelitis is a highly contagious disease caused by any of the three human poliovirus serotypes, which are part of the enterovirus family. Europe was certified free of poliomyelitis in June 2002. Immunisation and vigilance of the disease continue to ensure the region is free of poliomyelitis. Post-polio syndrome has no defined causal mechanism but it affects between 20% and 80% of patients afflicted with poliomyelitis.
Initial symptoms are those of a influenza-like illness (fever, headache, joint and muscle pain, vomiting, among other things) and can last up to 10 days. Its most serious forms may cause respiratory paralysis leading to death. Post-polio syndrome presents a new neurological weakness that may be progressive or abrupt on muscles previously affected or unaffected. It may or may not be accompanied by new health problems such as excessive fatigue, muscle pain, pain in the joints, intolerance to cold, reduced physical stamina and function, and atrophy.
It mainly affects children and the mechanisms for its transmission may be through faecal-oral channels or a common vehicle (contaminated water or food).
Post-polio syndrome affects patients who have had poliomyelitis for 20 years or more.
Diagnosis is given clinically, supplemented with laboratory and electromyographic (EMG) tests.
Symptomatic treatment with analgaesics, a ventilator where necessary, gentle exercise and possibility of orthopaedic devices to prevent deformities or to enable function.
In acute diagnoses, studying secretions, stools and cerebrospinal fluid. EMG in acute and later stages for diagnosing post-polio syndrome.
Poliomyelitis has no cure but it can be prevent by vaccination.
A urinary tract infection is defined as the presence of invasive bacteria in the urinary system, together with signs of inflammation, such as high temperature and local pain.
Urinary tract infections may be located in the lower urinary tract (bladder and urethra), or the upper urinary system, affecting one or both kidneys. A kidney infection is also known as pyelonephritis.
Infections of the lower urinary tract are characterised by localised pain, which increases when urinating, and sometimes by cloudy or dark urine, usually without high temperatures.
Kidney infections (pyelonephritis) are characterised by high temperatures, acute local pain in the lower back, and pain or irritation when urinating.
Urinary tract infection is characterised by the presence of local pain (lower abdomen or lumbar region), which increases when urinating. The urine is often cloudy, or dark if it contains blood. There may be high fever, especially in the case of pyelonephritis (an infection of the upper urinary tract).
It can affect people at any age, from early childhood to old age. It is more frequent among women and there are factors that make people vulnerable to it (pregnancy for women and enlarged prostate for men) as well as urological anomalies (pre-existing malformation or presence of kidney stones).
Urinary infections are diagnosed by examining urine under the microscope (sediment) to see whether it contains white blood cells and/or bacteria, and by cultivating the bacteria in a microbiological culture to identify the strain and determine the most appropriate antibiotic for treatment (antibiotic susceptibility testing).
Urinary tract infections are usually treated with antibiotics. Treatment is oral in the case of lower-tract infection.
For upper-tract infections (pyelonephritis) it is usually intravenous, although in some cases outpatient oral treatment may be administered.
The standard tests are urine sediment and culture (urine culture with antibiotic susceptibility testing). An ultrasound scan may be indicated for examining the kidney and urinary tract and identifying obstructions or kidney stones that may have brought about the infection.
Ultrasounds are also used to assess the state of the kidneys. A general analysis may also be indicated to see how the urinary tract infection is affecting the rest of the body, and specifically the renal function.
Urinary tract infection can be prevented by frequent urination (every 2 to 3 hours) and, above all, by avoiding the habit of holding in urine, and by going to the toilet whenever the bladder feels full, without waiting too long.
Acute hepatitis consists of an acute inflammation of the liver that makes the liver not work properly. This is normally brought on by a virus, pharmaceuticals or other toxins.
The symptoms are very varied and often go unnoticed. The most common are fatigue, poor appetite, nausea, muscle pain and fever. Occasionally, a yellow tinge appears in the whites of the eyes and the skin (jaundice), and urine becomes dark in colour (choluria). Symptoms may persist for one to three months before recovery. Hepatitis B and C may become chronic.
Hepatitis A affects children and young people. Thanks to improvements in health and hygiene in our country, the disease now quite rare. It may be found in patients who have recently visited countries with greater incidence of the disease and in people who have had contact with them. As previously mentioned, the means of transmission is faecal-oral and it generally clears up with no complications.
Hepatitis B is transmitted in the majority of cases through sexual contact or by exchange of blood (drug addicts use of needles). Transmission via blood transfusion is currently very regulated and virtually never happens. In areas where the disease is very prevalent (mainly Asian countries) the most common means of transmission of hepatitis B is from mother to child during pregnancy or childbirth (vertical transmission). In these cases of vertical transmission, if adequate treatment is not administered, hepatitis B becomes chronic in more than 90% of cases.
Toxic hepatitis is caused by exposure to substances which are harmful to the liver. These toxins may be pharmaceuticals, natural products or others. In some instances the connection between the toxin and the hepatitis is very well documented and is predictable. In other instances an unexpected reaction takes place (idiosyncrasy). Finally, there are pharmaceuticals that do not produce hepatic toxicity unless they are used in much higher doses than normal (for example paracetamol).
Diagnosis is mainly clinical (observation of jaundice, dark urine) and from lab results (elevated liver enzymes and positive viral detection).
In addition, during the development of the disease the appearance or development of antibodies specific to each virus is detected, which determine the response of the patient and whether or not the condition becomes chronic.
An abdominal ultrasound allows us to see if there are any complications stemming from the acute hepatitis and to exclude other causes that might produce similar symptoms.
In general no specific treatment is needed for acute hepatitis except in some cases produced by hepatitis B and C viruses. Extreme personal hygiene is important to avoid contagion to others.
No specific diet is recommended (alcohol must always be avoided). Nor is total bed rest necessary (physical activity should be adapted to the patients general condition).
Basically these consist of analyses to show the status and development of the liver and how the patient is responding to treatment. Blood analyses can also reveal to what extent the condition is becoming chronic.
The best possible treatment for the A and B viruses is vaccination (included in the routine vaccination schedule). No vaccine is currently available for the C virus.
Hepatitis A is transmitted by faecal-oral contact (contaminated food and drink and from person to person). Food hygiene is fundamental here.
Barrier contraception methods (the condom) can prevent the transmission of sexually transmitted diseases (including hepatitis B and C).
In countries where the disease is very prevalent, many pregnant women may have the disease and transmit it to their child during the final phase of the pregnancy or the delivery. The use of +/- gamma globulin early vaccination against the hepatitis B virus can prevent infection in children.
Toxic hepatitis is prevented with caution to the exposure of the various toxins involved.
Sun exposure is the major predisposing factor in all types of skin cancer, although in the case of melanoma there may also be a genetic predisposition.
The skin is exposed to all external factors, acting as a barrier and protection. Ultraviolet rays from solar radiation have the capacity to alter skin cells and transform them into cancer cells. There is a correlation between the degree of exposure to ultraviolet rays and the probability of having skin cancer. People with fairer skin also have greater predisposition than those who tan well after sun exposure.
Skin cancer is the most common type of cancer. The main types of skin cancer are:
Basal cell carcinoma (or basal cell cancer) shows slow local growth and, if not treated properly, it can infiltrate the underlying tissues requiring complex interventions. Squamous cell carcinoma (squamous cell carcinoma of the skin or spinocellular carcinoma) can also be locally invasive and in 5% of cases it spreads to the lymph nodes and to other organs.
Melanoma is much less common than other types of cancer, but there is greater risk of it spreading to other parts of the body. It is the main cause of death due to dermatological disease.
In all cases the diagnosis and treatment processes are very important.
Faced with recently appearing skin lesions, growth, bleeding or any change that can be seen, the dermatologist will guide diagnosis through clinical history, physical examination and in most cases a small skin biopsy.
A biopsy makes it possible to distinguish benign and malignant lesions and within these the subtypes of cancer that may have different treatments. Any pigmented skin lesion that has appeared recently or that changes in a short time must be assessed by a dermatologist to rule out melanoma. Fortunately this is not the norm, but it should always be considered because of its severity.
Skin cancer is very common and non-melanoma skin cancers have a very good prognosis. There is increased risk of suffering some types of skin cancer with age, possibly due to the fact that sun exposure accumulates over the years. Hence the importance of sun protection, especially starting early in life, given that the accumulation factor of repeated exposure to ultraviolet rays from the sun adds up.
Diagnosis is done by a dermatologist through inspection andbiopsy. You should consult a dermatologist when a skin lesion is persistent or if a pigmented area changes shape or colour.
In the case of basal cell carcinoma or squamous cell carcinoma, treatment is preceded by a biopsy to be followed by local removal surgery, destruction with cold (cryotherapy) or heat (electrocoagulation) and in some cases it may be sufficient to apply a medical treatment in the form of ointment with different active ingredients. Sometimes radiotherapy is also useful.
ºThe case of melanoma is very different because after the biopsy, the extent of the cancer must be evaluated and local treatment (surgery) or general oncology treatment administered to treat possible spread to other organs (metastasis).
In the case of non-melanoma skin cancer, the most common test is a skin biopsy. In the case of melanoma an investigation into the extent may be indicated to rule out other organs being affected (sentinel node biopsy and imaging tests).
Adequate protection from the sun's rays is very important to prevent skin cancer. You are advised to limit direct exposure to a few minutes or by using physical means (shirt, hat) or using creams or lotions with high sun protection factor (greater than 30), but these creams do not by any means offer total protection.
Pneumonia is an infection of the lung tissue.
Depending on the extent of pneumonia in the respiratory tract, different types are identified:
It can be caused by many different microorganisms, although the most common causes are S. pneumoniae (pneumococcus) and Mycoplasma.
Other microorganisms that can also cause pneumonia include Haemophilus, Klebsiella, Staphylococcus aureus, Legionella pneumophila, Chlamydia pneumoniae and some viruses.
Characterised by high fever, coughing, with or without sputum, and often chest pain, which may increase with respiratory movements. Sometimes sputum has a brownish or rusty appearance, which points to pneumonia caused by pneumococcus.
The so-called atypical pneumonia, caused by Mycoplasma or Chlamydia among others, is often characterised by fever with very few respiratory symptoms.
Pneumonia is a very common disease (350,000 cases/year in Spain) and is a significant cause of mortality in the general population. It can affect all age groups.
In previously healthy people it is a disease of mild or moderate severity. It can even be treated at home or in outpatient care, but in patients with previous pathology (immunocompromised, heart failure, previous respiratory failure), it is generally serious.
The appropriate use of antibiotics, together with occasional respiratory support measures (oxygen therapy or even intubation), contributes significantly to improving the chances of cure in the most severe cases.
It is performed based on the patient's clinical history (age, previous pathology, evolution time and type of symptoms), auscultation, chest radiography and blood and sputum cultures to identify the causative organism.
Antigens can also be detected in urine for pneumococcus and Legionella.
The treatment is antibiotic, based on a clinical estimate of the possibility of it being caused by one germ or another (in many cases treatment is started immediately without knowing the causal organism). Treatment is later maintained or changed according to the cultures and the patient's evolution.
The criterion for inpatient or outpatient treatment depends on the estimation of the risks that may occur (older age, previous pathology, impairment of respiratory function).
In a previously healthy patient, treatment may be in outpatient care.
Chest x-ray, blood and sputum or respiratory secretion cultures and determination of antigens in urine.
There are two very different types of otitis, both of which children can suffer from: external otitis and middle ear infection.
External otitis affects the auditory canal and is above all related to exposure to swimming pool, bath and fresh water in general. It is most common in summer.
Middle ear infection is related to infections in the upper respiratory tracts, and can be self-limiting (it resolves itself in most cases) or purulent requiring antibiotic treatment It is most common in winter.
b) Middle ear infection: there is usually a history of catarrh in the respiratory tract, suddenly accompanied by pain (in small children this can manifest as intense crying), with or without fever. It does not hurt when the external ear is touched or moved. Sometimes the ear drum may be perforated due to inflammation and pressure, which may result in a purulent secretion, generally associated with the pain (and crying) disappearing.
In both cases, diagnosis is clinical: medical history, assessing symptomatology and exploration using an otoscope.
Otoscope exam. Occasionally, if there is suppuration, cultivation of the pus.
Scleroderma is an autoimmune disorder characterised by increased collagen in various body tissues, structural alteration of microcirculation and certain immune abnormalities. The term scleroderma comes from the Greek “skleros”, which means hard, and “derma”, which means skin. This indicates that skin hardening is the most characteristic feature of the condition. As well as the skin, it can also affect the digestive tract, lungs, kidneys and heart. The prognosis varies. There is currently no cure, but the condition can be treated with general measures and treatment of symptoms, depending on the organs affected.
Raynaud syndrome: one of the most characteristic manifestations of the condition (97% of cases), it is the first clinical expression in most patients. It is caused by vasoconstriction of the capillaries. Patients report that with the cold their fingers change colour and turn pale (like wax) first, then turn blue after a while and finally turn reddish. The presence of Raynaud syndrome is not always an indication of scleroderma. In reality, only 5% of people with Raynaud syndrome later develop the condition. Almost half of sufferers may have digital ulcers, as an expression of a severe microcirculatory injury.
The most peculiar manifestation of the disease is the way it affects the skin. It is hard, tight and wrinkle-free (hard to pinch). The extent of the skin condition varies and is related to the prognosis. Two clinical forms are distinguished: limited (distal skin condition to elbows and knees) and diffuse (distal and proximal skin condition to elbows and knees, and torso). The face can be affected equally in both clinical forms. The limited subtype has a better prognosis than the diffuse one. Reduced aperture of the mouth (microstomy) may also be seen. In the skin there are hyperpigmented and coloured areas, telangiectasia (accumulation of small blood vessels) and sometimes subcutaneous calcium deposits can be felt (calcinosis).
Most patients experience joint and muscle pain, and in extreme cases contraction and retraction of the fingers are observed. When the digestive tract is affected, which often happens, the patient complains of a burning sensation and difficulty swallowing, as the oesophagus has lost its ability to move food towards the stomach. Pulmonary disease is the leading cause of death and may occur in the form of fibrosis or pulmonary hypertension; coughing, choking and heart failure are the main manifestations of lung involvement. When the heart is affected, heart rhythm disturbances and in some cases symptoms of angina pectoris are detected, due to the involvement of the small coronary vessels. In a small percentage (about 5%) scleroderma alters the kidney (scleroderma renal crisis) and manifests itself as malignant arterial hypertension and kidney failure.
It should be noted that not all patients with scleroderma present all the manifestations described above. It can also be concluded that there is great, almost individual, variability in the clinical expression of the disease.
Scleroderma is a rare disease with an incidence of 4-18.7/million/year and a prevalence of 31-286/million. It is more common in females, with a variable ratio, depending on the series, ranging from 3:1 to 14:1 (female/male). The age at which it presents is around 30-40 years.
When the above symptomatology is clear, the diagnosis does not offer too much room for doubt. Various complementary tests are helpful in confirming diagnosis and in assessing the degree of involvement of the various organs that may be affected.
“An incurable, but not untreatable condition”. There is currently no treatment for scleroderma that has satisfactory results, but this does not mean that it cannot be treated. Treatment is symptomatic, depending on the organ affected. For Raynaud syndrome: vasodilators, antiplatelets; gastro-oesophageal reflux: proton pump inhibitors; renal crisis: angiotensin converting enzyme inhibitors/dialysis; pulmonary fibrosis: immunosuppressants/lung transplant; pulmonary hypertension: vasodilators/lung transplant. In patients with the diffuse form and less than three years of evolution, immune modulators such as mycophenolate sodium (or mycophenolate mofetil) or methotrexate may be indicated as a basic treatment.
The most common tests to confirm and/or assess the degree of involvement of the various organs are: general analyses and immunological data (specific antinuclear antibodies); capillaroscopy, high-resolution computerised axial tomography scan of the chest, respiratory functional tests, oesophageal manometry and echocardiogram. In the follow-up for these patients, respiratory functional tests and an echocardiogram should be performed annually.
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