3 Systemic diseases with oral manifestations Scleroderma



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3 Systemic diseases with oral manifestations


Scleroderma
Scleroderma is a chronic autoimmune disease characterised by fibrosis, vascular alterations, and autoantibodies. Activation of the immune system causes injury to tissues. This injury is similar to scar tissue formation. The disease is more frequent in females than males. There are autoimmune diseases that can be associated with scleroderma such as Sjogren’s syndrome, systemic lupus erythmatosus, mysthenia gravis, and acquired haemolytic anaemia.

There are 3 major forms of scleroderma; diffuse, limited (previously called CREST syndrome in reference to calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasias), and morphea/linear. Diffuse and limited sclerodermas are both a systemic disease, whereas the morphea/linear form is localised to the skin. Diffuse scleroderma is severe, has a rapid onset and progression, with widespread skin hardening and will generally cause much internal organ damage. It can be fatal, as a result of heart, kidney, lung or intestinal damage. However, the limited type is much milder and has a slow onset and progression; skin hardening is usually confined to the hands and face; internal organ involvement is less severe, and a much better prognosis is expected. In typical cases of limited scleroderma, Raynaud's phenomenon may precede scleroderma by several years.

Most patients with systemic sclerosis have waxy, tight, smooth facial skin (Mona Lisa face) and skin tightening can cause the mouth to become smaller (microstomia) with difficulty in opening the mouth wide. It can cause numerous oral and dental problems when it affects the mouth and face. The oral mucosa will probably reveal atrophic epithelium and the tongue and soft palate are often partially immobilised giving rise to difficulty in eating, talking and swallowing. In the early stages, macroglossia is usual, but as fibrosis occurs the tongue becomes small, hard and immobilised (chicken tongue). The systemic disease is often accompanied by Sjogren's syndrome and the resulting dry mouth leads to increased cavities, gum disease, and candida infections. It can also loosen teeth by causing the ligament around the teeth to expand due to collagen deposition. When the ligament expands, the teeth are less supported by bone structure. Function is further inhibited by progressive involvement of the facial skin, muscles of mastication, and the TMJ. The localised type of the disease, known as morphea/linear, while disabling, tends not to be fatal and involves isolated patches of hardened skin, with no internal organ involvement. Individuals with morphea or limited scleroderma have a relatively positive outlook. Those with diffuse systemic scleroderma have a negative prognosis, and although more females are affected, the disease kills more men. Following diagnosis, two-thirds of patients live at least 11 years. The higher the patient's age at diagnosis, the more likely they are to die from the disease.
Aetiology


  • It is an autoimmune disease.

  • It could be inherited, but the environment seems to also play a role.

Diagnosis





  • It is essentially a clinical one.

  • Blood analysis is non-contributory in localised scleroderma.

  • The anti-topoisomerase antibody is most often seen in patients with the diffuse form of scleroderma, whereas anti-centromere antibody is found almost exclusively in the limited form.

  • Nearly all patients with scleroderma have ANA. Other tests are used to evaluate the presence or extent of any internal disease. These may include GIT tests, chest x-ray, lung function testing and CT scanning, ECG, echocardiogram (Echo), and sometimes heart catheterisation to evaluate the pressure in the arteries of the heart and lungs.

Treatment





  • There is no cure for scleroderma, and treatment aims at ameliorating the symptoms.

  • Raynaud's phenomenon may be treated with agents to increase blood flow to the fingers.

  • Fibrosis of the skin has been treated with drugs that soften the skin and reduce inflammation. Some patients may benefit from exposure to heat.

  • Because scleroderma is an autoimmune disease, one of the major pillars of treatment involves the use of immunosuppressive agents such as methotrexate, azathioprine, cyclophosphamide, and mycophenolate.



Amyloidosis
Amyloidosis is the abnormal deposition of amyloid protein in various body tissues, leading to organ or tissue damage. Most often, amyloid protein comes from cells in the bone marrow. Deposition of amyloid in a localised area or a single tissue of the body is called localised amyloidosis, causing relatively few symptoms. Often with aging, amyloid can be locally produced and deposited to cause tissue injury. Amyloidosis that affects tissues throughout the body is referred to as systemic amyloidosis, and can cause serious changes in virtually any organ of the body. It has been classified into 3 major types that are very different from each other. These are distinguished by a 2 letter code that begins with an A (for amyloid). The second letter of the code stands for the protein that accumulates in the tissues in that particular type of amyloidosis. The types of systemic amyloidosis are currently categorised as primary (AL), secondary (AA), and hereditary (ATTR). Primary amyloidosis or AL is of unknown cause and occurs when a specialised cell in the bone marrow (plasma cell) spontaneously overproduces a particular protein portion of an antibody called the light chain. Primary amyloidosis can occur with a bone marrow cancer of plasma cells called multiple myeloma. It is not associated with any other diseases, but is a disease entity of its own. Secondary amyloidosis or AA is more common than AL and occurs secondarily as a result of another illness, such as chronic infections (eg tuberculosis, osteomyelitis) or inflammatory diseases (eg rheumatoid arthritis, ankylosing spondylitis). Hereditary amyloidosis or ATTR is a rare form of amyloidosis, inherited as an autosomal dominant. The amyloid deposits are composed of the protein transthyretin, or TTR, which is made in the liver. Therefore, the offspring of a person with the condition has a 50% chance of inheriting it. Symptoms of amyloidosis result from abnormal functioning of the particular organs involved. The heart, kidneys, liver, bowels, skin, nerves, joints, and lungs can be affected. As a result, symptoms are vague and can include fatigue, shortness of breath, weight loss and loss of appetite, numbness, tingling, and weakness. Amyloidosis affecting the kidney leads to nephrotic syndrome which is characterised by severe loss of protein in the urine and swelling of the extremities. The most frequent cause of death in systemic amyloidosis is kidney failure. The most common oral manifestation of amyloidosis is macroglossia, which occurs in 20% of patients. The enlarged tongue demonstrates lateral ridging due to teeth indentation. Although pain is not usually present, enlargement, firmness, and loss of mobility are common. Submandibular swelling occurs subsequent to tongue enlargement and can lead to respiratory obstruction. Interference with taste has also been reported in some patients, and hyposalivation may result from amyloid deposition in the salivary glands.
Aetiology
It is caused by extracellular deposition of insoluble protein called amyloid.
Diagnosis


  • It is made by detecting the characteristic amyloid protein in a biopsy specimen of involved tissue (such as mouth, rectum, kidney, heart or liver).

  • A needle aspiration biopsy of fat, just under the skin of the belly (fat pad aspiration), offers a simple and less invasive method to diagnose systemic amyloidosis.

  • The detection of amyloid deposits in a patient warrants further evaluation for possible multiple myeloma which yields a poor prognosis for the patient.

Treatment




  • Currently, amyloidosis has no cure and treatment seeks to limit further production of the amyloid protein.

  • Combination aggressive treatment in AL using melphalan chemotherapy medication, in conjunction with bone marrow stem cells transplantation, has been promising.

  • More aggressive treatment options may be employed in AA to directly target the underlying disease responsible for amyloidosis.

  • ATTR can now be cured with liver transplantation.



Sarcoidosis
Sarcoidosis is a disease in which noncaseating epithelioid granulomas form nodules that may affect any organ system. It usually occurs between the ages of 20-40 years. Women are slightly more likely to develop the disease than men. Normally the onset is gradual and it may be asymptomatic or chronic. Granulomas most often appear in the lungs or the lymph nodes, but virtually any organ can be affected. Common symptoms are vague, such as fatigue, fever, swollen lymph nodes, weight loss, aches and pains, arthritis, dry eyes, blurred vision, severe redness of the eyes and sensitivity to light. Almost everyone who has sarcoidosis eventually experiences lung problems, which may include persistent dry cough, shortness of breath (dyspnea), wheezing, and chest pain. About 5-10% of patients develop serious disability, and lung scarring or infection may lead to respiratory failure and death. Up to 25% of the individuals who have sarcoidosis develop skin problems, which vary and range from rashes and nodules to erythema nodosum or lupus pernio. Oral involvement in sarcoidosis is rare and usually manifests after systemic symptoms develop. The symptoms may include lip swelling and multiple nodular painless ulcerations of the gingiva, buccal/labial mucosa, and palate. Although less common, salivary gland involvement is a possibility, leading to tumor-like swellings. Sarcoidosis can be difficult to diagnose, partly because the disease produces few signs and symptoms in its early stages; and when symptoms do occur, they vary and can mimic those of other disorders.
Aetiology
The cause of the disease is still unknown.
Diagnosis


  • This is commonly a diagnosis of exclusion of other granulomatous diseases such as Wegener granulomatosis, Crohn disease, syphilis or tuberculosis.

  • Blood test may show a high level of calcium, and liver and kidney function tests should be carried out to determine the extent of the disease.

  • Chest x-ray, CT scan, and pulmonary function tests are needed.

  • Microscopic examination of specimens of lung tissue obtained with a bronchoscope, or of other tissues can provide the ultimate diagnosis.

  • Kveim reaction is a diagnostic test for sarcoidosis, involving intradermal injection of antigen derived from a lymph node known to be sarcoid. If a lump appears on the skin at the test site in 4-8 weeks, the reaction is said to be positive and that the patient has sarcoidosis.

Treatment




  • Between 30-70% of patients do not require therapy because the disease commonly improves or clears up spontaneously.

  • Corticosteroids (prednisolone) have been the standard treatment for many years and their use is generally limited to severe, progressive or organ-threatening disease.

  • Multiple organ and progressive pulmonary involvement indicate a poor prognosis.



Cystic fibrosis
Cystic fibrosis (CF) is an inherited common multisystemic disease which affects the entire body causing progressive disability and often early death. The name CF refers to the characteristic scarring (fibrosis) and cyst formation. Cystic fibrosis affects the cells that produce sweat, mucus, and digestive juices. Normally, these secretions are thin and slippery, but in CF a defective gene causes the secretions to become thick and sticky. Instead of acting as a lubricant, the secretions plug up tubes, ducts and passageways, especially in the lungs and pancreas. Cystic fibrosis is the most common fatal disorder among caucasians of European descent and one in 25 carry one gene for CF. In the past, most people with CF died in their teens. Improved screening and treatment allow many people with CF to live into their 50s or even longer. The symptoms and severity of CF vary widely. Some people have serious problems from birth. Others have a milder version of the disease that doesn't show up until they are teens or young adults. One of the first signs of CF is an excessive salt in the sweat. Most of the other signs and symptoms affect the respiratory or the digestive system. Difficulty in breathing is the most serious symptom and results from frequent lung infections, which is the cause of death in CF patients. Other symptoms include sinus infections, poor growth, diarrhea, and infertility. Lip swelling, gingivitis, and dryness are the more frequent oral findings.
Aetiology


  • Children who inherit a faulty cystic fibrosis transmembrane conductance regulator (CFTR) gene from each parent will have CF.

  • Those who inherit a faulty gene from one parent and a normal CFTR gene from the other parent will be CF carriers. CF carriers usually have no symptoms and live normal lives; however, they can pass the faulty CFTR gene onto their children.

Diagnosis




  • Newborn screening for CF can be achieved by using genetic testing and blood test. DNA samples from blood or saliva can be checked for specific mutations on the gene responsible for CF, and blood tests help measure the health of liver and pancreas.

  • In early childhood, sweat test that measures the amount of salt in sweat, is the most useful test for diagnosing CF.

  • Chest x-ray, CT scan or MRI are recommended to examine the lungs and internal organs.

  • In case of lung infection, sputum test is needed to identify the pathogen and choose the antibiotic.

Treatment




  • There is no cure for CF.

  • Medications such as antibiotics, mucus thinning drugs, and bronchodilators are used to treat and prevent lung infections.

  • Ultimately lung transplantation is often necessary as CF worsens.


Wegener’s granulomatosis
Wegener's granulomatosis (WG) is an uncommon necrotising vasculitis of small arteries and veins. It classically involves inflammation of the arteries that supply blood to the tissues of the lungs, the nasal passages (sinuses), and the kidneys. When both lungs and kidneys are affected, the condition is referred to as generalised WG. When only the lungs are involved, the condition is referred to as limited WG. It usually affects young or middle-aged adults. Symptoms of WG include fatigue, weight loss and fever, shortness of breath, bloody sputum, joint pains, and sinusitis. Nasal ulcerations, and even bloody nasal discharge, can occur. Other areas of the body that can also become inflamed include eyes, nerves (neuropathy), middle ear (otitis media), and skin, resulting in skin nodules or ulcers. Oral involvement in WG is common, and autopsy studies of patients with the disease show this site is affected in nearly all cases. Oral lesions include ulcerations and gingival enlargement. Initially, bright red to purple friable diffuse papules originate on the labial interdental papillae. The gingivae take on a characteristic swollen, reddened, and granular appearance. The characteristic gingival appearance is a pathognomonic finding termed strawberry gingivitis, although it is less common than other findings. Involvement may eventually include the lingual and palatal mucosa. Tooth and alveolar bone loss are common. Oral manifestations may correlate with disease progression, thereby providing prognostic value.

Aetiology

The cause of WG is unknown.

Diagnosis




  • Early diagnosis of the disease is essential.

  • Blood tests include ESR, CRP, and urine tests to detect protein and RBC.

  • A more specific blood test used to diagnose and monitor WG is the antineutrophil cytoplasmic antibodies (ANCAs), which is elevated when the disease is active.

  • Biopsy findings of the gingival papillomatous lesion confirm the diagnosis.

  • X-ray tests of the chest and sinuses are recommended to detect abnormalities of WG.

  • Open lung or kidney biopsies are also commonly used in making a diagnosis.

Treatment


  • Treatment is usually with oral corticosteroids and cyclophosphamide.

  • Prompt treatment is important to prevent further damage to the lungs and kidneys.



Graft versus host disease
Graft versus host disease (GVHD) is a common complication that occurs in the bone marrow involving a donor and a recipient. Since only identical twins have identical tissue types, a donor's bone marrow is normally a close, but not perfect match to the recipient's tissues. Histocompatibility antigen test Bone marrow transplantation is frequently used to treat cancer, mainly leukaemias. Clinically, GVHD is divided into acute and chronic forms. The acute form of the disease is normally observed within the first 3 months after transplant, and is a major challenge to transplants owing to associated morbidity and mortality. The chronic form of GVHD usually starts more than 3 months after transplant, and can last a lifetime. Rates of GVHD vary from between 30-40% among related donors and recipients to 60-80% between unrelated donors and recipients. The greater the mismatch between the donor and recipient, the greater the risk of GVHD. After a transplant, the recipient usually takes drugs that suppress the immune system; this helps reduce the chances or severity of GVHD. Symptoms in both acute and chronic GVHD range from mild to severe. The acute form is characterised by selective damage to the liver, skin and mucosa, and the GIT. Chronic GVHD also attacks the above organs, but over its long-term course it can also cause damage to the connective tissue and exocrine glands. In both acute and chronic GVHD, the patient is very vulnerable to infections. The oral manifestations of acute GVHD have been described as painful ulcerations together with cheilitis, striae, white plaque-like patches, xerostomia and erythema. Minor erythema of the oral mucosa suggests chronic GVHD, whereas a normal oral cavity denotes absence of the disease. Additional contributing causes of oral complications are thought to arise from the side effects of chemotherapy and radiation therapy.
Aetiology

Graft versus host disease is a complication that can occur after a bone marrow transplant in which the newly transplanted material attacks the transplant recipient's body.

Diagnosis


  • It is by oral biopsy if white lesion (lichenoid reaction), minor salivary gland biopsy if xerostomia (Sjogren’s syndrome), and skin biopsySkin biopsy if scleroderma-like lesions.

  • Chest x-ray and GI endoscopyGastrointestinal endoscopy with or without a biopsybiopsy.

  • Liver function testsLiver function tests (ALP, AST, and bilirubin levels will be increased), and liver biopsyLiver biopsy if the patient only has liver symptoms.

Treatment


  • The goal is to suppress the immune response without damaging the new cells.

  • High dose corticosteroids are the most effective treatment for acute GVHD.

  • Treatment of chronic GVHD includes corticosteroids with or without cyclosporine.

  • Antibodies to T-cells and other medicines are given to patients who do not respond to steroids.



Rheumatoid arthritis
Rheumatoid arthritis (RA) is a common, autoimmune systemic disease that causes chronic inflammation of the joints. The disease is 3 times more common in women than in men. It can begin at any age, but it most often starts between 40-60 years. While RA is a chronic illness, meaning it can last for years, patients may experience long periods without symptoms. However, RA is typically a progressive illness that has the potential to cause joint destruction and functional disability; and is often accompanied by Sjogren’s syndrome. When the disease is active, symptoms can include fatigue, loss of energy, lack of appetite, low grade fever, muscle and joint aches, and stiffness. The joint inflammation of RA causes swelling, pain, stiffness, and redness. Moreover, studies have shown that the progressive damage to the joints does not necessarily correlate with the degree of pain, stiffness, or swelling present in the joints. The TMJ is affected in more than 17% of adults and children with RA, but it is usually among the last joints involved. Pain, swelling, and limited movement are the most common findings. In children, destruction of the condyle results in mandibular growth disturbance and facial deformity, followed by ankylosis. In early stages, x-rays of the TMJ are usually negative but later show bone destruction, which may result in an anterior open-bite deformity. In most patients with RA, the condition will necessitate few or no changes in routine dental care. However, considerations include the patient’s ability to maintain adequate oral hygiene, xerostomia and its related complications, the patient’s susceptibility to infections, impaired haemostasis, and untoward drug actions and interactions. Oral ulcerations and lichenoid reaction may appear as a consequence of the use of NSAIDs or other anti-rheumatics.

Aetiology



  • It is an autoimmune diseas.

  • In some families, multiple members can be affected, suggesting a genetic basis for the disorder.

Diagnosis

  • Blood tests for CBC, CRP, and RF (found in 80% of patients).

  • Anticitrulline antibody (anti-CCP) and ANA is present in most patients with RA.

  • Imaging techniques to visualise the structural integrity of the jaw joint, such as x-ray, CT scan, MRI or arthroscopy.

  • Arthrocentesis, the removal and analysis of fluid in the joint using a syringe, can be helpful to determine if an infection is present.

Treatment




  • Applying moist heat packs to the painful jaw joint, eating a soft diet, and using night guard is often helpful.

  • NSAIDs may be given, and jaw function should be restricted.

  • Injection of steroids directly into the painful joint may provide pain relief. This can only be done a limited number of times, as repeated use can harm the joint.

  • Arthrocentesis can be helpful in relieving joint swelling and pain.

  • Antibiotic prophylaxis may be needed before dental treatment if patient is taking oral corticosteroid due to immunosuppression.

  • The enzyme geranylgeranyltransferase-I (GGTase-I) inhibitor has the potential to treat RA, as it protects against inflammation and joint destruction.





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