Oral Lupus: Etiology, Epidemiology, and Clinical Decisions

Oral Manifestations of Lupus Erythematosus

Oral Manifestations of Lupus

Oral Manifestations of Lupus Erythematosis

Systemic lupus erythematosus (SLE), or lupus for short, is a chronic and generally progressive autoimmune disease affecting many tissues in the body (Lupus Foundation of America, 2015). The U.S. Centers for Disease Control and Prevention (CDC, 2015) discriminates between SLE and lupus affecting the skin only (discoid/cutaneous lupus erythematosis) or that caused by medications (temporary). SLE and cutaneous lupus erythematosus (CLE) are the only two types of lupus relevant to this article, since temporary lupus can be treated effectively by changing medication. Prevalence estimates vary considerably, but in the United States an estimated 161,000 individuals have a definitive diagnosis of SLE. Information about incidence rates is equally poor, but the most recent study found that 5.6 out of 100,000 Whites in Rochester, Minnesota developed SLE between 1980 and 1992. What follows is a detailed examination of lupus erythematosus (LE), oral manifestations of this disease, and a discussion of the recommended treatment approaches.

BRIEF 1:

Lupus is caused by a person's immune system malfunctioning and the generation and expansion of auto-antibodies recognizing healthy tissue (Lupus Foundation of America, 2015). The targeted tissues in turn suffer from chronic and often progressively painful inflammation and localized tissue damage. Although the disease can manifest at any point in person's lifespan, minority women between the ages of 15 and 40 represent the demographic with the highest incidence rates (CDC, 2015). The cause of the disease is unknown, but infections or other environmental factors may trigger disease onset in genetically susceptible individuals (Mayo Clinic Staff, 2014a). Only a small number individuals, however, who have a family history of autoimmune disease will develop LE, so it is hard to predict which family members will develop the disease. The signs and symptoms associated with SLE include arthritis, skin rashes, photosensitivity, pleuritis, pericarditis, blood cell abnormalities, kidney problems, seizures, and psychosis, but most relevant to this discussion is the manifestation of oral ulcers. The primary treatment approaches rely on the administration of immunosuppressants, which are effective is slowing disease progression and minimizing flares. Flares can be life-threatening and hospitalizations are not uncommon among this patient population. In the absence of a diagnosis and effective treatment, the risk of serious complications and even death is significant.

BRIEF 2:

The oral manifestations of SLE include discoid lesions, honeycomb plaques, raised keratotic plaques (horny growth similar to warts), erythema (capillary dilation), purpura (small hemorrhage), petechiae (sub-epidermal hemorrhage), cheilitis (inflammation and cracking of lips), and ulcers with an irregular shape (Chi, Neville, Krayer, & Gonsalves, 2010). Lesions will develop in patients suffering from CLE, but only in the presence of skin lesions. Researchers examined the oral lesions of 46 patients with a confirmed diagnosis of SLE or CLE and found the most common oral lesion to be lichenoid mucositis (Lourenco et al., 2007). The lichenoid mucositis lesions were associated with a perivascular inflammatory infiltrate and localized epithelial thickenings or atrophy. Spongiosis, to varying degrees, was also observed in most patients. Histological examination of biopsy samples revealed IgG, IgM, and/or C3 (complement factor 3) deposits in the basement membrane in all samples and immune infiltrates consisting primarily of CD4+ T cells. CD20+ B cells and macrophages were also present, but in far fewer numbers compared to T cells. Importantly, oral lesions were most common in adult women suffering from CLE (73.9%), rather than SLE (26.1%); although, the reverse has been found by other researchers sampling different populations (e.g., see Chi et al., 2010). Most patients in the study were women and the median age of those with oral lesions was 41.8 years.

BRIEF 3:

Diagnosis of SLE or CLE (LE) with oral manifestations, in the absence of a formal diagnosis of LE, can be obtained through histopathology and direct immune-fluorescence (DIF) of oral biopsy samples (Lourenco et al., 2007). The most common histopathological findings will be ". . . epithelial hyperkeratosis with atrophy of the rete pegs, superficial and deep mononuclear inflammatory infiltrate, edema in the lamina propria, liquefative degeneration of basal epithelial cells and predominantly patchy PAS-positive subepithelial deposits" (p. 561). DIF results will reveal primarily IgM deposits in the basement membrane and perivascular T cell infiltrates. A differential diagnosis against several other conditions must be performed to rule out other causes of the oral lesions.

Once a diagnosis of LE has been confirmed the next objective is to treat the oral lesions using appropriate medications and care plan. Oral lesions in patients undergoing systemic treatment for LE will often resolve without additional interventions; however, topical treatments are often prescribed for patients (Brennan, Valerin, Napenas, & Lockhart, 2005). Other considerations include renal involvement, neuropsychiatric symptoms that may impair treatment compliance, cardiovascular disease, infections, anemia, leucopenia, autoimmune thrombocytopenia, antiphospolipid syndrome, and chronic candidiasis. Once the disease has progressed to the point of involving the kidneys and cardiovascular system the prognosis is generally poor, which may be an important consideration before undertaking prolonged and painful treatments for oral lesions.

BRIEF 4:

Care should be coordinated with a rheumatologist since a number of other conditions must be ruled out before a diagnosis of SLE or CLE can be given (Mayo Clinic Staff, 2014b). Care coordination with a rheumatologist should continue once LE has been diagnosed because systemic treatment may be the optimum treatment approach for resolving oral lesions and consideration must be given to kidney, cardiovascular, and brain involvement (Chi et al., 2010). Pretreatment planning will require gathering together information by the patient and family caregivers that can address the following questions (Mayo Clinic Staff, 2014b):

When symptoms began and whether flare-ups occur?

What triggers, if any, worsen symptoms?

Family history of autoimmune disease?

Current medications, herbal remedies, and supplements?

Patients may also want to create a list of questions to ask their provider, such how to obtain more information about their condition, treatment options, and recommended lifestyle changes.

BRIEF 5:

Patients may be diagnosed as having acute CLE, subacute CLE, or chronic CLE, but patients with acute CLE are either suffering from SLE or will eventually develop SLE (Brennan et al., 2005, p. 129). Mucosal involvement of chronic or subacute CLE may manifest alone or as part of SLE. CLE patients with oral lesions may be treated systemically, but if topical treatments are chosen then corticosteroids, anti-mycotic agents, or intralesional steroid injections are typically used. Basic recommendations for all CLE patients are: (1) to avoid sunlight exposure using proper clothing or sun block with an SPF rating of 15 or higher, (2) use topical or systemic corticosteroids, and (3) use anti-malarial drugs for systemic involvement (p. 134-135). Patients with complications, like renal nephritis or cardiovascular involvement, will need to be treated simultaneously for these conditions as well.