The effects of these medications can last for a year after transplant. For allograft (donor) recipients, there is a possibility of graft-versus-host reaction that develops in the salivary glands and can diminish salivary flow and may also affect other oral tissues. This is often an early sign of graft- versus-host disease.
Self-Assessment Quiz Question #3 Which of the following structures is the most the most frequently documented source of sepsis in the immunosuppressed cancer patient? a. Epidermal layer. b. Oral cavity. c. Respiratory tract. d. GI tract.
CONNECTIVE TISSUE DISORDERS
However, each type is distinct and may or may not affect the musculoskeletal system (Arthritis Foundation, 2020). Arthritis alone affects roughly 54 million Americans, and it has been estimated that close to 78 million Americans may be affected by the year 2090 (Arthritis Foundation, 2020). Among infants and children, almost 300,000 are living with arthritis or a rheumatic condition (Arthritis Foundation, 2020). Systemic lupus erythematosus is estimated to affect 1.5 million people in the U.S., and scleroderma is variably estimated to affect 300,000 people in the U.S. (Arthritis Foundation, 2020; Lupus Foundation of America, 2023). Although there are many types of connective tissue disorders, they are related by causality such as genetics, infection, or injury. Library of Medicine, 2018). Oral signs include a high palatal vault, severe dental crowding, and a retrognathic (underdeveloped) mandible. Because of severe laxity of the temporomandibular joint, the mandible can be easily dislocated during dental treatment (Cervino et al., 2020). Ehlers-Danlos syndrome is closely related to Marfan syndrome. There are 11 different types of Ehlers-Danlos syndrome, all of which are based on disruption in collagen formation. These patients present with joint hypermobility and skin hyperextensibility (easily stretchable skin). They tend to bruise and scar very easily. Oral manifestations include small tooth morphology, malformed roots, and premature calcification of the pulp (Lepperdinger et al., 2021). Oral providers should be aware of some common symptoms of many of the connective tissue disorders. Patients tend to be easily fatigued, present with muscle pain and stiffness, demonstrate swelling of the joints, and have poor peripheral circulation. No cures exist for connective tissue disorders, but many drug regimens are employed to control the symptoms. The drugs include salicylates, traditional nonsteroidal anti-inflammatories (NSAIDs), tumor necrosis factor-alpha inhibitors, injectable glucocorticoids, systemic glucocorticoids, and several other tissue-specific medications. As noted above, patients with systemic lupus erythematosus may present with oral lesions, sometimes resembling lichen planus, that can be extremely painful. Palliative treatment is important for patient management and may include a prescription for Gelclair or Zilactin, again with caution. Patients with Ehlers-Danlos syndrome may present with small tooth morphology, malformed roots, and premature calcification of the pulp (Lepperdinger et al., 2021). Being mindful of the classic symptoms may help to minimize the patient’s discomfort and potentially aid in diagnosing certain connective tissue disorders.
More than 200 entities are classified as connective tissue disorders. The affected tissues of the body include collagen-type structures that are responsible for cushioning, protecting, supporting, insulating, and strengthening the body. The connective tissues include cartilage, ligaments, tendons, and skin. Many of the diseases in this category are autoimmune in nature and include rheumatoid arthritis, polymyositis, scleroderma, systemic lupus erythematosus, Marfan syndrome, and Ehlers-Danlos syndrome. Juvenile arthritis (JA) is an umbrella term to describe many autoimmune and inflammatory conditions or pediatric rheumatic diseases in children ages 16 years and younger. Juvenile arthritis affects nearly 300,000 children in the U.S., and various types of JA share common symptoms such as pain, joint swelling, redness, and warmth. Medical considerations/current medical therapies Steroid medications are typically employed to control the symptoms of connective tissue disorders. Routine premedication with antibiotics is not typically recommended, unless cardiac criteria indicate such a regimen. (See Table 1 for antibiotic prophylaxis.) Some patients with systemic lupus erythematosus present with thrombocytopenia (abnormally low platelet count, discussed in the bleeding disorders section of this course). Therefore, a coagulation profile with platelet count should be requested, along with consultation with the patient’s physician. Symptoms of connective tissue disorders depend on the tissue or organ that is involved. Patients may experience symptoms on occasion or on a chronic basis. In the case of rheumatoid arthritis, joint swelling is the hallmark sign and can be severe and disabling. Patients with systemic lupus erythematosus typically present with a butterfly rash across the nose and cheeks. Oral complications include ulcerative lesions on the lips and mucous membranes. These lesions, sometimes resembling erosive lichen planus, can be extremely painful. The most common connective tissue disorder resulting from genetic inheritance is Marfan syndrome, with a reported incidence of 1 in 5,000 (Du et al., 2021). Patients with this disorder display hyperlaxity of joints, possible scoliosis (curvature of the spine), elongated limbs, and a high incidence of heart valve abnormalities (U.S. National Dental treatment implications Before treating the pediatric patient who presents with a confirmed or suspected connective tissue disorder, it is advisable to consult with the patient’s physician. It is important to establish the type of disorder, the type of drug therapy the patient is on, and the short-term and long-term prognosis. For some disorders, such as Marfan or Ehlers-Danlos syndromes, aortic or cardiac complications may influence dental treatment decisions. The oral health provider should plan for short appointments based on the patient’s abilities. It is important not to hyperextend the mandible because it is easily dislocated in this patient population.
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