The vasculitides are a group of diseases characterized by a noninfectious necrotizing vasculitis and resultant ischemia. Considerable overlap in the clinical manifestations of these diseases makes it difficult to develop categories with strict criteria. A practical approach has been to classify them into groups by the size of vessels involved, the specific anatomic sites involved, and the clinical manifestations. Some of the more important ones are discussed here.

Polyarteritis Nodosa

Polyarteritis nodosa has been considered the prototype of the vasculitides. This rare disease has an incidence of less than 1 in 100,000 per year. It affects males and females equally and is seen in all racial groups. Patients are usually in the fifth and sixth decades of life at presentation. The vasculitis involves small and medium-sized arteries and can be the result of hepatitis B infection. Tissues involved include the gastrointestinal tract, hepatobiliary system, kidney, pancreas, skin, testicles, peripheral nerves, and the skeletal muscles. Symptoms at presentation are primarily constitutional (fever, weight loss, and malaise) with peripheral neuropathy (mononeuritis multiplex).

Despite widespread arterial involvement, otolaryngologic manifestations are few, but sudden bilateral sensorineural hearing loss can be seen. Vestibular disturbances also have been reported. The proposed mechanism of cochleovestibular damage is thromboembolic occlusion of the end arteries of the inner ear. Other head and neck manifestations include cranial nerve palsies, in which the seventh nerve is most commonly involved and skin or mucosal lesions.

Churg-Strauss Syndrome

Churg-Strauss syndrome, also called allergic angiitis granulomatosis, is a disease consisting of systemic small-vessel vasculitis, extravascular granulomas, and hyperesosinophilia. It occurs in patients with preexisting asthma and allergic rhinitis. The vasculitis generally presents with peripheral neuropathy or pulmonary infiltrates. Tissue eosinophilia is another feature of this disease.

Hypersensitivity Vasculitides

The hypersensitivity vasculitides are a heterogeneous group of small-vessel vasculitides that universally involve the skin, arteritic involvement of small vessels (particularly postcapillary venules), and leukocytoclasis. Diseases in this group include hypersensitivity angiitis, Henoch-Schönlein purpura, and cryoglobulinemia vasculitis. These syndromes appear immune complex mediated and may be triggered by a foreign antigen, which is often not identified. Therapy is concentrated on identification and elimination of inciting antigens. Glucocorticoids, immunosuppressive drugs, and plasmapheresis are commonly used in treatment.

Wegener Granulomatosis

Wegener granulomatosis is a rare (incidence of less than 1 in 100,000 per year) form of vasculitis characterized by the triad of respiratory tract granulomas, vasculitis, and glomerulonephritis (Wegener’s triad). There is no sex predilection. Most patients are white and present in the fifth decade of life. The typical clinical features include bilateral pneumonitis (95% of patients), chronic sinusitis (90%), mucosal ulceration of nasopharynx (75%), and evidence of renal disease (80%). The nasal symptoms usually occur early in the disease and include crusting, epistaxis, and rhinorrhea. Erosion of the septal cartilage with saddle-nose deformity and nasal stenosis may occur after the vasculitis and granulomata destroy cartilage.

Recurrent sinusitis is the most common problem in patients with Wegener granulomatosis. In some cases, the clinical picture of vasculitis first becomes apparent when the patient has a workup before nasal sinus surgery. In others, it is not until excised sinus mucosa is evaluated histopathologically that the diagnosis of vasculitis is established. Repeated nasal mucosal biopsies are often required as acute inflammation can obscure the subtle changes of vasculitis.

The most common oral cavity findings of Wegener granulomatosis include hyperplasia of the gingiva and gingivitis. Edema and ulceration of the larynx are seen in 25% of patients. Significant subglottic stenosis develops in 8.5% of patients and is a poor prognostic sign. Salivary glands may also be involved. Otologic problems develop in 20% to 25% of patients and include conductive hearing loss secondary to serous otitis media; suppurative otitis media, with or without granulation tissue in the middle ear; sensorineural hearing loss, often profound and bilateral; and pinna changes similar to those seen with polychondritis . Facial nerve palsies also have been documented.

The diagnosis is based on the clinical, pathologic, and laboratory findings. The hallmark pathologic lesion in Wegener granulomatosis is necrotizing granulomatous vasculitis. The discovery of cytoplasmic staining antineutrophil cytoplasmic antibody (c-ANCA) has revolutionized the diagnosis of Wegener granulomatosis. The sensitivity of c-ANCA for Wegener granulomatosis ranges from 65% to 90%, and the specificity is quite high, although this test may be positive in patients with polyarteritis nodosum and Kawasaki disease. Despite the high specificity, however, tissue diagnosis must be used to confirm the diagnosis.

Left untreated, Wegener granulomatosis is fatal within 2 years in 93% of cases. However, good control is achieved with corticosteroids and low-dose daily cyclophosphamide. Azathioprine or methotrexate may be alternatives to cyclophosphamide. Isolated sinus disease may be treated with low-dose steroids, topical nasal steroids, saline irrigations, and antibiotics when bacterial superinfection is suspected. Airway compromise may be alleviated with systemic steroids, although significant subglottic stenosis may require tracheostomy.

Giant Cell Arteritis

Giant cell (“temporal”) arteritis is characterized by focal granulomatous inflammation of arteries of medium and small size. Giant cell arteritis is the most common of the vasculitides, and its prevalence increases with age to about 20 per 100,000 persons aged 50 years and older. It is a general arteritis of which temporal arteritis is only one local manifestation. Many believe this disease also encompasses polymyalgia rheumatica. Polymyalgia rheumatica is a clinical syndrome of muscular pain and morning stiffness in the proximal muscle groups without inflammatory joint or muscle disease. Patients with polymyalgia rheumatica have histologic changes in the arteries that are like those in temporal arteritis. In addition, polymyalgia rheumatica is seen in 50% of patients with classic giant cell arteritis. Systemic symptoms, such as low-grade fever, weight loss, and malaise, may precede localized symptoms in all manifestations of the disease. Polymyalgia rheumatica alone is usually self-limiting and responds well to low-dose prednisone therapy. The symptoms of classic giant cell arteritis reflect involvement of the cranial blood supply by the vasculitis. Headache is the initial symptom in 47% of patients. It is the most common symptom, of variable character, and occurs in as many as 90% of patients.

Despite the name, the temporal artery is erythematous and tender in only 50% of patients with giant cell arteritis. The scalp, however, is often very tender. Jaw claudication occurs in as many as 50% of these patients, and 25% have lingual claudication. Vertigo and hearing loss also have been reported. Involvement of the ascending pharyngeal artery can lead to dysphagia. Cranial nerve deficits, vertebrobasilar insufficiency, and psychosis reflect intracranial disease. Blindness occurs in a third of untreated patients and may be heralded by field defects or amaurosis fugax.

In giant cell arteritis, as with several of the vasculitides, the erythrocyte sedimentation rate (ESR) is usually more than 50 mm/h. Although cases with normal or slightly elevated ESRs have been reported, those results usually lead the clinician away from the diagnosis. Confirmation of diagnosis of giant cell arteritis is accomplished with a biopsy of the temporal artery on the most symptomatic side. If the initial biopsy is negative, the contralateral side should be biopsied. False-negative results range from 5% to 40%.

The treatment is with corticosteroids. Normalization of the ESR or C-reactive protein and loss of symptoms are the therapeutic guidelines. The purpose of treatment is both the elimination of pain and prevention of blindness and other vascular complications. Therapy may be required for 5 years or longer.

Behçet Disease

Behçet disease is a vasculitis affecting Japanese and Mediterranean populations that usually presents in the third decade with oral and genital ulcers and uveitis or iritis. These aphthous-like ulcers are characteristically punched out, with or without surrounding erythema, and are covered with a pale pseudomembrane. They are frequently the first symptom of the disease. They are painful lesions that occur singly or in clusters on the lips, gingiva, buccal mucosa, and tongue and are seen less often on the palate and in the oropharynx. The genital ulcers are similar but are deeper and larger. Healing occurs in a few days or weeks, and some scarring results. Symptoms may occur simultaneously or months apart. Morbidity is secondary to CNS involvement, arthritis, and large-vessel arteritis. Eye involvement is seen in 43% to 72% of patients and loss of sight occurs in 25%. Local treatment for oral and genital ulcers is primarily through the use of corticosteroid creams. Local treatment for eye involvement includes topical mydriatics and corticosteroid injections. Fever is treated with antipyretics or nonsteroidal antiinflammatories. Colchicine, azathioprine, methotrexate, corticosteroids, and dapsone are used systemically.

Cogan Syndrome

Cogan syndrome is a rare disease of young adults (median age 22) characterized by Ménière-like audiovestibular dysfunction, interstitial keratitis, and nonreactive tests for syphilis. The symptoms frequently begin after an upper respiratory infection. Audiovestibular manifestations are usually bilateral and can include fluctuating hearing loss, vertigo, tinnitus, and aural pressure. These symptoms may resolve spontaneously and reappear months later. The hearing loss is progressive and severe and usually associated with decreased or absent vestibular responses on caloric testing. Bilateral deafness results in 65% of cases. The ear symptoms may precede or follow the ocular disease by several years. Often, Cogan syndrome is accompanied by a large- or medium-vessel systemic vasculitis. Pathologic features suggest end-organ specific autoimmune mechanisms rather than vasculitis damage to the inner ear. Hearing outcomes are improved with early initiation of systemic corticosteroid treatment so early diagnosis is critical. Permanent visual loss is rarely seen.

Kawasaki Disease

Kawasaki disease, also known as mucocutaneous lymph node syndrome, is a disease of the pediatric age group and the most common cause of acquired heart disease in children in the developing world. The cause is unknown, but epidemiologic data suggest an infectious cause. Clinical characteristics include fever, conjunctivitis, red and dry lips, erythema of the oral mucosa, polymorphous truncal rash, desquamation of the fingers and toes, and cervical lymphadenopathy. Although cervical adenopathy is the least common feature, seen in 50% to 75% of patients, this disease must be considered in febrile children with cervical lymphadenopathy unresponsive to antibiotics without an alternative diagnosis. Twenty percent to 25% of untreated children develop coronary artery dilation or aneurysms, and a small percentage die from myocardial infarction due to thrombosis or rupture of coronary aneurisms, usually from 2 to 12 weeks after onset of disease. Treatment with intravenous d-globulin and aspirin in the first 10 days of illness reduce the incidence of coronary abnormalities 10-fold.

The term mixed connective tissue disease was coined in 1972 to describe a distinct entity with coexisting features of SLE, systemic sclerosis, polymyositis, and dermatomyositis. This entity is characterized by high titers of anti-U1 RNP, a ribonucleoprotein antibody. The prevalence is unknown, and no consensus diagnostic criteria have been developed. However, 80% of patients are women, and onset usually occurs between the ages of 30 and 60. Death results primarily from pulmonary fibrosis and hypertension.

Head and neck manifestations are a combination of the features seen in the other connective tissue disorders. Mucocutaneous changes include malar rash, discoid lupus, sclerodermatous skin thickening, oral mucosal ulceration, and nasal septal perforation. Sicca complex has been described. Esophageal dysfunction is present in most cases, resulting in abnormal peristalsis, heartburn, and dysphagia in 60%, 48%, and 38% of patients, respectively. As with most other connective tissue diseases, corticosteroid and immunosuppressive agents are the mainstays of treatment.

Relapsing polychondritis is characterized by episodic recurring inflammation of cartilaginous structures that are eventually replaced by granulation tissue and fibrosis. Women (3:1) are more commonly affected than men, and the average age at onset is 47. There appears to be a racial predilection for whites. Defined features of the disease include recurrent chondritis of the auricles, nonerosive inflammatory polyarthritis, chondritis of the nasal cartilages, ocular inflammation, chondritis of laryngeal and/or tracheal cartilages, and cochlear or vestibular damage. Diagnosis requires three of these features without histologic confirmation, two of these features with response to steroids or dapsone, or any one feature with histologic confirmation.

Auricular chondritis and nonerosive arthritis are the most common presenting symptoms of relapsing polychondritis. Auricular chondritis is characterized by the sudden onset of erythema and pain, sparing the lobule, which lacks cartilage. It is the feature presentation in 33% of patients and will develop in 90% of those with the disease. Resolution occurs in 5 to 10 days with or without treatment. Patients may develop conductive hearing loss secondary to collapse of the external auditory canal, and 40% suffer cochlear or vestibular dysfunction, possibly due to vasculitis of the internal auditory artery.

Chondritis of the nasal cartilages develops in 75% of patients and often does not coincide with the auricular involvement. The nasal cartilage chondritis also has a sudden onset and a resolution, which resolves in several days with or without treatment. After the cartilaginous inflammation subsides, deformities result from the loss of cartilage. These are disfiguring in the ear, and often cause a classic saddle deformity of the nose.

Laryngeal involvement presents early with a nonproductive cough, which progresses to hoarseness and stridor. Fifty-three percent of patients with relapsing polychondritis will have respiratory tract involvement during the course of their disease. Diagnostic endoscopy is dangerous in these patients due to the risk of tracheal collapse. With extensive airway involvement, management is difficult even with tracheotomy. In most cases, corticosteroids are the main form of treatment. The antileprosy sulfone dapsone also has helped in some cases, and methotrexate is playing an increased role.

The inflammatory myopathies are a group of disorders characterized by proximal muscle weakness and nonsuppurative inflammation of skeletal muscle. Polymyositis and dermatomyositis are subsets of this group. The incidence of these disorders is estimated to be 5 new cases per million persons annually. There is a 2:1 female preponderance. The age at onset is between 40 and 60 years, and a pediatric variant affects children between 5 and 15 years of age. The diagnostic criteria for polymyositis and dermatomyositis are complex and evolving.

The inflammatory myopathies may be isolated or associated with other abnormalities. Polymyositis and dermatomyositis may be associated with other connective tissue disorders, including systemic sclerosis, SLE, and RA. In about 20% of patients, the myopathy is associated with a malignancy, particularly ovarian cancer, but cancer of the lung, prostate, breast, and colon have been implicated. Peng et al. reported an increased incidence of nasopharyngeal carcinoma in patients with dermatomyositis living in an endemic area for this neoplasm.

Head and Neck Manifestations

Head and neck manifestations of polymyositis reflect proximal muscle involvement. Half of patients report weakness of the neck muscles. Difficulty in phonation and deglutition occurs because of diseased tongue muscles. Nasal regurgitation is common because of palatal and pharyngeal muscle involvement. Thirty percent of patients with these diseases have dysphagia secondary to involvement of the upper esophagus, cricopharyngeus, pharynx, and superior constrictors. Dysfunction of these muscle groups also results in aspiration and secondary pneumonia. The skin lesions of dermatomyositis vary, but there is a predilection for sun-exposed areas.

Treatment

Steroids are used to treat symptomatic patients. Methotrexate and other immunosuppressive agents are used in patients who do not tolerate or respond to steroids. Upper gastrointestinal symptoms related to esophageal dysfunction may require omeprazole, cisapride, and referral to a speech therapist.

Scleroderma, also called systemic sclerosis, is characterized by sclerotic skin changes that are often accompanied by multisystem disease. Progressive fibrosis of involved organs is the pathologic hallmark of the disease. Scleroderma may be limited to relatively benign cutaneous involvement (extremities distal to the elbows and knees and the face and neck) or may exist as an aggressive systemic disease. The incidence is 3 to 12 new cases per million per year. Scleroderma has a 3:1 female to male preponderance, with the median age at onset between 40 and 50 years. Initial presentation includes Raynaud’s phenomenon, edema of the fingers and hands, and skin thickening. The American College of Rheumatology criteria for scleroderma include one major criteria (sclerodermatous skin changes proximal to the metacarpal-phalangeal joints) and two of three minor criteria: sclerodactyly, digital pitting scars, and bibasilar pulmonary fibrosis on chest radiograph. Visceral and often fatal manifestations are seen in the gastrointestinal tract, lung, heart, and kidneys. Arthralgias and muscle weakness are common musculoskeletal complaints, and Raynaud’s phenomenon is almost universal.

Head and Neck Manifestations

Eighty percent of patients with systemic sclerosis have signs and symptoms involving the head and neck, and in 30% of these patients the head and neck symptoms were part of the presenting complaints. The typical facies associated with scleroderma consists of tight skin, thin lips, and vertical perioral furrows. The skin changes are secondary to the underlying dermal and subcutaneous inflammatory process. Dysphagia is the most common initial complaint. Abnormal radiographic findings are observed for the distal two thirds of the esophagus in 80% of patients with systemic sclerosis, and 50% of these patients are symptomatic. Decreased or absent peristalsis with mild to moderate dilation is reported, and hiatal hernia is common.

In the skin, edema precedes epidermal atrophy and loss of appendages. Eventually, 35% of these patients develop facial tightness. A decreased ability to open the mouth is the initial complaint in 19% of patients, a manifestation that is secondary to skin changes and not to temporomandibular joint dysfunction. Additional skin manifestations include telangiectasias (19%), calcinosis (3%), and linear scleroderma, which usually affects scalp and limbs, involving the cheeks and chin.

Gingivitis and periodontal membrane thickening are common. Oral tissues demonstrate edema followed by atrophy and induration of mucosal and muscular tissues. About 25% of patients report xerostomia, xerophthalmia, or both. Involvement of the larynx occurs, and almost half of patients with systemic sclerosis complain of voice change. Raynaud’s phenomenon of the tongue, an unusual complication, may present as mucosal blanching associated with dysarthria. Trigeminal neuralgia and facial nerve palsy are infrequent manifestations.

Treatment

Randomized controlled trials in scleroderma have failed to reveal effective disease-modifying therapies. The treatment of systemic sclerosis is symptomatic. Calcium channel blockers can be useful in Raynaud’s phenomenon. Omeprazole is the drug of choice for reflux esophagitis. Angiotensin-converting enzyme inhibitors are used in the treatment of renal disease.

Sjogren syndrome is a chronic disorder characterized by immune-mediated destruction of exocrine glands predominantly but not exclusively the lacrimal and salivary glands. Sjogren syndrome occurs in primary and secondary forms. The primary form is a diagnosis of exclusion. The secondary form refers to the sicca complex accompanying any of the connective tissue diseases. Secondary Sjogren syndrome is primarily seen with RA but is also commonly associated with SLE, scleroderma, and primary biliary sclerosis. Sjogren syndrome occurs in 1% of the general population and in 10% to 15% of patients with RA. There is a 9:1 female preponderance; onset occurs between 40 and 60 years of age. In one third of patients with primary Sjogren syndrome, the disorder is systemic with involvement of extraglandular sites. Sjogren syndrome is associated with an increased (33 to 44 times) risk of lymphoma.

The common clinical manifestations of Sjogren syndrome include xerophthalmia with secondary keratoconjunctivitis and xerostomia, with or without salivary gland enlargement. These manifestations are called sicca complex or sicca syndrome. Minor salivary gland biopsy usually demonstrates heavy lymphocyte infiltration, although parotid biopsy may be more sensitive and specific. Rheumatoid factor and antinuclear antibodies are high in most Sjogren syndrome patients. Antibodies directed toward Sjogren syndrome A (Ro/SS-A) and Sjogren syndrome B (La/SS-B) antigens are noted in 60% and 30% of patients, respectively. These antibody tests lack specificity because they are found commonly in patients with SLE, RA, and polymyositis. Many anti-Ro-positive Sjogren syndrome patients will go on to develop SLE . Salivary gland biopsy is the best single criterion for Sjogren syndrome (specificity 83%, sensitivity 81%). There is often confusion between the designations Sjogren syndrome and Mikulicz disease. The latter is swelling of the salivary glands accompanying nonconnective tissue diseases, such as hyperlipoproteinemia, malnutrition, diabetes, cirrhosis, tuberculosis, and sarcoidosis. A nonspecific lymphocytic infiltration is seen on biopsy of the salivary glands of patients with Mikulicz disease. Sjogren syndrome is not related to the divergent group of systemic diseases associated with Mikulicz disease.

Head and Neck Manifestations

Exocrine gland pathology dominates the head and neck manifestations of Sjogren syndrome. About 80% of these patients complain of xerostomia, the most prominent symptom of this disease. These patients report difficulty chewing, dysphagia, changes in taste, fissures of the tongue and lips, and an increased number of dental caries. Oral candidiasis and angular cheilitis are frequent complications of dry mouth. Diminished secretion of tears leads to keratoconjunctivitis sicca and eye complaints including dryness, burning, itching, and foreign body sensation. Loss of glandular secretions in the nasal passages cause crusting and secondary epistaxis in 50% of patients and hyposmia in 40%. Chronic sinusitis may result from inspissated secretions, and there may be occlusion of the nasolacrimal ducts. Other manifestations of primary Sjogren syndrome include intermittent unilateral parotid swelling in 30% of patients and sensorineural hearing loss.

Treatment

Symptomatic treatment is the primary approach to patients with Sjogren syndrome because no treatment alters the course of the disease. Patients and their primary care physicians must be made aware of the deleterious effects of decongestants, antihistamines, diuretics, and specific drugs used for treatment of cardiovascular and psychiatric problems that are known to produce a dry mouth. In addition to increasing oral fluid intake, the use of saliva and tear substitutes and pilocarpine may be helpful. Nonsteroidal and steroidal antiinflammatories fail to improve salivary flow. Antifungals are used to treat oral candidiasis. Close supervision by a dentist and preservative dental treatments are essential .

Head and Neck Manifestations

Articular involvement predominates in the diverse head and neck manifestations of RA, affecting the ossicles, temporomandibular joints, cricoarytenoid joints, and the cervical spine. Temporomandibular joint dysfunction may be prominent. Most patients with RA have temporomandibular joint complaints. Pain or tenderness at the joint or in the masseter or temporalis muscles, crepitus, limited mobility, or deviation are commonly reported. Radiographic evidence of joint erosion is often present. Temporomandibular joint dysfunction in patients with RA may be severe and cause contractures of the muscles of mastication, producing an anterior open bite deformity.

Cricoarytenoid joint involvement in RA is the most frequent cause of arthritis in these joints. Histologic abnormalities of the cricoarytenoid joints are present in 86% of patients with RA. Clinically, however, only 30% of the patients with RA are hoarse. Cricoarytenoid arthritis may present with dyspnea on exertion, anterior neck or ear pain, fullness in the throat, dysphagia, and aspiration. Hoarseness in RA is usually the result of cricoarytenoid joint involvement but may be caused by rheumatoid nodules within the cords and ischemic recurrent nerve paresis or paralysis. The sudden onset of stridor and dyspnea in a patient with RA is an emergency requiring systemic steroids and possibly tracheostomy. The oral cavity usually is not involved with abnormalities related to RA unless there is associated Sjögren syndrome. In an uncommon variant of rheumatoid vasculitis, there are oral ulcers similar to those seen in polyarteritis nodosa.

The middle ear may be involved in severe cases of RA if synovitis develops in the ossicular joints, but this rarely result in a conductive hearing loss except during an acute RA flair. Stiffness in the incudomalleolar and incudostapedial joints does not impair sound conduction but results in stiffness abnormalities detected on tympanometric testing. Autoimmune inner ear disease has been related to RA, but no definitive mechanism has been proven.

Treatment

Salicylates, nonsteroidal antiinflammatory agents, gold salts, penicillamine, hydroxychloroquine, and immunosuppressive agents have been used to suppress the inflammation in RA. Additional treatment goals include maintaining joint function and prevention of joint deformities.

Systemic lupus erythematosus (SLE) is a common connective tissue disease. The incidence is 1 in 1,000, with nine women affected for every man. It has a highly variable course and affects primarily women of childbearing age. Protean systemic manifestations include photosensitive skin eruptions, serositis, pneumonitis, myocarditis, nephritis, hypercoagulability, and central nervous system (CNS) involvement. Criteria for the diagnosis of SLE are outlined in Table 14.2 (2). Death results most often from active SLE, infections, and atherosclerotic vascular disease, although the survival rate of this disease is 80% at 10 years and 65% at 20 years.

Head and Neck Manifestations

The head and neck manifestations of SLE are dominated by skin and mucosal lesions. A malar or “butterfly” rash is the first sign of the disease in 50% of patients. Oral ulcerations are painful and show a pronounced hyperemia and edema with a tendency for superficial ulcerations and bleeding. Localized telangiectasis may produce a red halo effect around affected mucosa. Secondary moniliasis and xerostomia are common. Histologically, mucosal lesions in SLE demonstrate orthokeratosis and parakeratosis alternating with areas of epithelial atrophy. Keratotic plugging, acanthosis, and pseudoepitheliomatous hyperplasia are common findings. Superficial, perivascular, and deep lymphocytic infiltration can be seen throughout the mucosa.

Numerous other head and neck problems may be associated with SLE. In 3% to 5% of well-established cases, there is ulceration or perforation of the nasal septum. Inflammatory changes of the larynx and trachea have been described, including true vocal fold thickening or paralysis, cricoarytenoid arthritis, and subglottic stenosis. Up to 25% of SLE patients report dysphagia. Acute enlargement of the parotid gland occurs in 10% of SLE patients and may be unilateral, tender, and confused with acute parotitis. Xerostomia may become a chronic problem.

Neuropathy is also a major characteristic of SLE, and the cranial nerves are affected in 15% of patients. This may involve the motor supply to the extraocular muscles, the sensory divisions of the trigeminal nerve, the motor divisions of the facial nerve, the vestibular portion of the vestibulocochlear nerve, or the optic nerve. Sudden hearing loss has been described with SLE, although a definitive link has not been established. Such hearing loss may be due to thrombosis or vasculitis, although temporal bone studies have been inconclusive. A definitive link to SLE has been considered but not established. There is also a nonspecific lymphadenopathy associated with SLE that in some cases appears to be related to skin or mucosal lesions.

Discoid lupus erythematosus is a subtype of SLE in which cutaneous lesions result in significant scarring, but there is no visceral involvement. These lesions are well-demarcated, erythematous, edematous papules that depigment and scar on resolution. The face is involved in 85% of cases, the scalp in 60%, and the ear in 44%. There also may be associated leukoplakia of the tongue and oral mucosa.

Treatment

Management of this severe and complex disease requires a rheumatologist. Because many SLE patients are highly photosensitive, avoidance of sun exposure and liberal use of sunscreens are recommended. The general treatment of SLE includes nonsteroidal antiinflammatory drugs, topical and low-dose systemic steroids, and antimalarials. Low-dose methotrexate may be an alternative to systemic steroids. The use of high-dose systemic steroids and immunosuppressive agents, such as azathioprine and cyclophosphamide, is restricted to cases with visceral involvement that might lead to organ (heart, kidney, CNS) damage.

Symptomatic treatment of the head and neck manifestations of SLE, as with all the connective tissue diseases, is required when the systemic steroids become ineffective. Many patients complain about the loss of salivary flow and the development of oral and pharyngeal lesions. Saliva substitutes are helpful. Steroid-based topical solutions for the treatment of mouth ulcers are effective if delivered efficiently. Ointments and creams designed for intraoral use are not effective. Mouthwashes are useful when used frequently and held in contact with the diseased tissues for a few minutes. One such preparation is Klack’s solution, which consists of tetracycline, cortisone, diphenhydramine, and nystatin. The oral lesions also may be helped with postprandial cleansing using mouthwashes with stock hydrogen peroxide solution diluted with equal parts of warm tap water.

The connective tissue diseases have a pattern of organ involvement that overlaps and often makes specific diagnosis difficult. When first recognized as a distinct histopathologic characteristic, the perivascular collagen deposition prompted the name collagen vascular disease. Their association with immunologic reactions to body proteins subsequently caused the name change to autoimmune diseases. These entities are, however, more correctly called connective tissue diseases. Biochemical and ultrastructure studies indicate that collagen derangement and evidence of autoimmune processes may not be the primary disease.

The exact cause of connective tissue diseases remains obscure. Specific autoantibodies are associated with some of these entities, supporting the presumption of an autoimmune mechanism. Definite evidence demonstrating loss of self-tolerance or specific antigens is lacking.

The prevailing histopathologic feature of these diseases is a varying amount of connective tissue and blood vessel inflammation with abundant fibrinoid deposits. It is the tissue distribution of the inflammatory response and the pattern of organ involvement that differentiate one connective tissue disease from another. The clinical patterns resulting from this unique tissue response give the members of this family of diseases their individual names.

The syndromes of thyroiditis include five disorders. These disorders must be differentiated from each other and from other diseases such as lymphoma, Graves disease, and neoplasia.

Hashimoto Thyroiditis

Hashimoto thyroiditis, the most common form, is goiterous thyroiditis associated with elevated levels of antithyroid antibodies. It often presents in middle-aged women. A family history of thyroiditis is common. The disease also is associated with other autoimmune diseases, including Graves disease.

The presentation is a slowly progressive painless enlargement that is rarely limited to one area of the thyroid. Microscopically, lymphocytic infiltration, with fibrosis and Hürthle cell change of the follicular cells, predominates. As more of the gland is involved in the disease process, there is glandular enlargement caused by compensation of the uninvolved gland. Rarely, overcompensation with resultant hyperthyroidism ensues. Typically, the disease progresses to hypothyroidism.

Antimicrosomal antibody is the most specific test for diagnosing Hashimoto thyroiditis. About 90% of patients have positive titers for this antibody. Titers are also positive in low levels in Graves disease and subacute thyroiditis. Thyroid function tests may show hypothyroidism, compensated hypothyroidism with elevated thyroid-stimulating hormone, or hyperthyroidism or they may be normal. The erythrocyte sedimentation rate may be elevated, although not to the levels seen in subacute thyroiditis.

Treatment consists of thyroid hormone replacement to decrease the size of the goiter and to treat the hypothyroidism. If the goiter fails to regress with suppression, the physician must consider lymphoma or neoplasm, both of which are associated with Hashimoto thyroiditis. Fine-needle aspiration is useful if there is a dominant “cold” nodule on the radionucleotide scanning or suspicion of malignancy. Surgery is indicated if medical treatment fails to relieve obstructive symptoms, if the diagnosis is in doubt, or for cosmetic reasons.

Subacute Thyroiditis

Subacute thyroiditis consists of two separate entities: granulomatous and lymphocytic thyroiditis. Both are rare compared with Hashimoto thyroiditis.

Subacute granulomatous thyroiditis is now thought to be caused by a viral infection. It is characterized by infiltration of inflammatory cells, especially the characteristic giant cells, which leads to fairly rapid thyroid swelling and pain that often is associated with a transient hyperthyroidism as the glandular stores are released. After depletion, a period of transient hypothyroidism follows, usually lasting a few weeks. Complete recovery occurs in most cases, with 10% having a persistent goiter.

Subacute lymphocytic thyroiditis is also called painless thyroiditis because of the characteristic painless development of thyroiditis. This and the presence of a lymphocytic rather than a giant cell infiltrate differentiate these two diseases. Otherwise, the two diseases are clinically similar. The lack of fibrosis, Hürthle cell change, and the different clinical course differentiate it from Hashimoto thyroiditis. Permanent goiter and hypothyroidism more commonly are seen in subacute lymphocytic thyroiditis than in subacute granulomatous thyroiditis. Painless thyroiditis most commonly is seen in postpartum women (i.e., postpartum thyroiditis). One episode usually implies repeated episodes with each subsequent pregnancy.

Acute Suppurative Thyroiditis

This exceedingly rare form of thyroiditis is characterized by bacterial or fungal (i.e., in immunocompromised patients) infections. The typical pathogen is Staphylococcus aureus. The clinical picture of acute suppurative thyroiditis is rapid development of pain, redness, and swelling of the gland. Treatment consists of antibiotics and surgical drainage if there is abscess formation.

Reidel Thyroiditis

Reidel thyroiditis is an extremely rare fibrosing thyroiditis of unknown cause. These patients typically present with gradual onset of local pressure. On examination, the gland is hard. Pain and tenderness are unusual. The fibrosis may progress to involve the entire gland or merely produce hypothyroidism. Treatment is thyroid hormone replacement or surgical release of obstruction (i.e., isthmusectomy), if required.

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