Sarcoidosis Remission and Aden Protocol
Sarcoidosis is a chronic, idiopathic, granulomatous disease with a strong predilection for African American and Puerto Rican women in their third or fourth decades of life. Patients most often present with pulmonary symptoms, including shortness of breath, hemoptysis, nonproductive cough, and dyspnea on exertion however, the spectrum of patient presentation can range from asymptomatic to involvement of any organ system. When neurologic involvement is present, the term neurosarcoidosis has been used. Neurologic involvement can be central or peripheral. Diabetes insipidus, basal leptomeningitis, and peripheral neuropathy are frequent manifestations (76). An estimated 12 of all patients with sarcoidosis and 50 of patients with neurosarcoi-dosis have facial paralysis, which remains the most frequent neurological presentation of the disorder. The paralysis can be either unilateral or bilateral in up to 33 of patients. Any case of bilateral facial paralysis deserves consideration of...
Sarcoidosis is a chronic systemic granulomatous disease of unknown etiology, characterized by the presence of noncaseating granulomas in any organ system of the body. Involvement of the head and neck is noted in 10 to 15 of patients (17). Please see Chapter 6 for a detailed discussion of sarcoidosis. Epidemiology. Sarcoid has a slight female preponderance and occurs in all age groups, although more commonly in young adults. Pathogenesis. An etiology for sarcoid has not been established. Hallmark pathologic findings for the disorder include noncaseating granulomata consisting of modules of epithelioid histiocytes surrounded by a mixed inflammatory infiltrate (Fig. 7). Intracytoplasmic inclusions, asteroid and Schaumann bodies, can be seen. Special stains for a multiplicity of microorganisms are noted to be negative. Clinical Manifestations. Any organ system may be affected by sarcoid, but pulmonary and cutaneous involvement are typically noted. Otolaryngologic signs and symptoms...
Diagnosis of SS also requires exclusion of other conditions that can mimic it. These include previous radiation therapy to the head and neck, amyloidosis, sarcoidosis, lymphoma, graft versus host disease, hepatitis C virus infection, HIV-diffuse infiltrative lymphocytosis syndrome, medication-induced dryness, and uncontrolled diabetes mellitus.
While sarcoidosis frequently pursues an unpredictable clinical course, its prognosis may correlate with specific types of disease onset and patterns of clinical manifestations. For example, acute onset of erythema nodosum with symptomatic bilateral hilar adenopathy usually has a self-limited course, while insidious onset of disease and extrapulmonary lesions are often followed by inexorable progression of pulmonary fibrosis (30). In the head and neck, complications of sarcoidosis include hearing loss, vestibular dysfunction, chronic sinusitis, infection, decreased visual acuity and blindness, hoarseness, upper respiratory obstruction, stridor, cranial nerve palsies, and pituitary dysfunction. The complications of the persistent ocular inflammation are described above, but it should be emphasized that sarcoidosis is a significant cause of blindness in the United States.
Transthoracic FNAC can be used with confidence to select treatment modalities and avoid unnecessary surgeries in patients with lung malignancies. When the diagnosis of small cell carcinoma is reached in a patient with a lung mass, a surgical treatment approach is no longer considered and chemotherapy becomes the treatment of choice 18 . As a result of EUS-FNA, thoracotomy thoracoscopy was avoided in 49 and mediastinoscopy was avoided in 68 in a study by Larsen et al 122 . According to Yasufuku et al 24 , cytologically diagnosed all the 5 cases of small cell lung cancer in their study were referred for chemotherapy. In the report by Larsen et al 122 , the direct result of the cytological diagnosis obtained by EUS-FNA was that a final diagnosis of small cell lung cancer was made in eight patients resulting in referral for chemotherapy, and in another three patients with benign disease specific treatment could be initiated (sarcoidosis, mediastinal abscess, and leiomyoma of esophagus).
The objectives of cervical mediastinoscopy are to establish a histologic diagnosis of level 2, 4, and 7 lymph nodes and to assess the operability of central lung cancers. The indication for the operation is middle mediastinal adenopathy suspected from clinical history, chest X-ray, or CT scan. The more common etiologies of middle mediastinal adenopathy include primary malignancies, e.g., primary bronchogenic carcinoma granuloma-producing diseases of infectious etiology, such as Mycobacterium tuberculosis and Histoplasma capsulatum and granuloma-producing diseases of uncertain etiology, such as sarcoidosis.
Sarcoidosis is an autoimmune disorder of unknown etiology. It affects multiple organ systems and is characterized by an accumulation of T-lymphocytes and mononuclear phagocytes causing noncaseating epithelioid granulomas. Its appearance in the oropharynx mimics that of infectious adenotonsillitis. Sarcoid frequently involves
Painless enlargement of the salivary glands occurs in 4 to 6 of patients with sarcoidosis. The parotid glands are most commonly enlarged, but any of the salivary glands can be affected. Sarcoidosis may mimic Sjogren's syndrome by causing symptoms and signs of dry eyes and mouth. The constellation of parotid enlargement with facial palsy, fever, and anterior uveitis is called Heerfordt's syndrome (30,33). Sarcoidosis targets the upper respiratory tract in up to 18 of patients and is probably more frequently seen in the nose than the sinuses (34). Sinonasal disease may occur in isolation or may accompany manifestations in other organ systems. Nasal obstruction, rhinorrhea, nasal crusts, epistaxis, anosmia, pain in maxillary teeth, facial pain, headache, and intermittent dysphagia are the most common symptoms (34,35). Examination most frequently reveals erythematous, edematous, friable, hypertrophied mucosa and nasal crusts with studding by small pale or erythematous nodules. Also seen...
Their role in diagnosis is limited because of moderate sensitivity and specificity. The antiglia-din antibodies are found in intestinal secretions as well as in serum of patients with untreated celiac disease. However, these antibodies are also found in a variety of autoimmune disorders including rheumatoid arthritis, Sjogren's syndrome, sarcoidosis, inflammatory bowel disease, and cows' milk protein intolerance. IgA antigliadin antibodies have sensitivity of 75-90 and specificity of 82-95 . The IgG antigliadin antibodies range in sensitivity from 69 to 85 and have specificity of 73-90 they are useful in the diagnosis of celiac patients with IgA deficiency. Other than this use gliadin antibodies have fallen from favor as a screening test for celiac disease (National Institute of Health consensus panel).
(Chapter 9), dermatomyositis (Chapter 14), polyarteritis nodosa (inflammation of the arteries), sarcoidosis (a rheumatic disease that may cause elevated calcium levels), and mixed connective tissue disease (Chapter 10) often have polyarticular arthritis in addition to their other symptoms. There are a variety of less common diseases that also may begin with polyarticular arthritis and should be excluded by history, physical, and laboratory evaluation. Children with chronic pains in their hands or feet without obvious synovitis should be evaluated for the possibility of other conditions such as Fabry disease or mucopolysaccharidosis.
Uveitis occurs in a wide variety of systemic diseases including those described herein and in other chapters of this book. Several associated diseases are of primary clinical interest, although individually they are relatively uncommon or even rare in the usual practice of a head and neck specialist. Sarcoidosis is a common cause of anterior, intermediate, and posterior uveitis, which is covered in more detail later in this chapter. Other diseases causing uveitis are described in other chapters of this book and include Behcet's disease, relapsing polychondritis, syphilis, Lyme disease, cat-scratch disease, tuberculosis, fungal infection, and infection with the human immunodeficiency virus, cytomegalovirus, and herpes viruses.
Sarcoid Sarcoid is a multisystem disorder characterized by non-caseating granuloma. There seems to be two overlapping patterns of clinical presentation in the juvenile population with skin, joint and eye disease in younger children, and lymphadenopathy, weight loss, fever, pulmonary involvement, but no arthritis, in older children and adolescents (42). Sarcoid is more common in the black population and females. Treatment is with steroids and a steroid sparing agent such as methotrexate or azathioprine.
In most cases of pulmonary disease occurring in patients who have SS, however, a tissue biopsy, either open or closed, must be obtained, as the differential diagnosis in these patients is extensive (eg, rheumatoid nodules, sarcoidosis, lymphoma, and even amyloidosis). It should be remembered that even though sarcoidosis is an exclusionary criterion listed in the American European Consensus Criteria,1 there have been several reports of SS and sarcoidosis coexisting in the same patient.98,99
The diagnosis of tularemia is based on paired antibody agglutination titers. Biopsy specimens of affected lymph nodes will show abscess formation with caseating necrosis. In the latter stages of the disease, the histologic picture will demonstrate granulomatous inflammation similar to mycobacterial infection or sarcoid.
FIGURE 1 (A) Granulomatous anterior uveitis Mutton-fat keratic precipitates (large) on the backside of the cornea and synechiae in a patient with sarcoidosis. (B) Intermediate uveitis Snow bank and snow ball found at the periphery of the cornea in a patient with intermediate uveitis. (C) Recurrent toxoplasmosis old and inactive pigmented retinal scars with cream-colored reactivation lesion at the edge. (D) Band keratopathy corneal calcification in a patient with JRA. (E) Cystoid macular edema CME imaged with OCT. Note cystic area in macula with thickened retina surrounding the macula. (F) Iris nodules multiple iris nodules in a patient with sarcoidosis. Abbreviations JRA, juvenile rheumatoid arthritis CME, cystoid macular edema OCT, ocular computerized tomography. Source Courtesy of Dr. Glenn Jaffe, Duke University Eye Center. FIGURE 1 (A) Granulomatous anterior uveitis Mutton-fat keratic precipitates (large) on the backside of the cornea and synechiae in a patient with sarcoidosis....
Serum calcium measures the level of calcium in the blood. It may be abnormal in children with disease of the parathyroid gland, which secretes a hormone to regulate calcium levels. Sarcoidosis is a rheumatic disease that may cause elevated calcium levels. The level may also be abnormal in children with kidney disease, excessive vitamin D, or metabolic abnormalities. These problems may result in bone and joint abnormalities or pain if they persist for an extended period. Low calcium may be associated with severe muscle cramps. Rickets may cause problems in children who do not get enough vitamin D and is associated with a low serum calcium level and joint pains however, rickets is extremely rare in the United States because most dairy products contain supplemental vitamin D.
Inflammatory diseases include chronic granulomatous disease such as eosinophilic granuloma or histiocytosis, Wegener's granulomatosis, sarcoidosis, amyloidosis, cholesterol granuloma, and invasive cholesteatoma. Characteristic lesions within the middle ear or temporal bone on examination or imaging are aided with serologic testing (i.e., sedimentation rate, anti-neutrophile cytoplasmic antibody (c-ANCA)) and tissue biopsy for diagnosis. In cases of suspected systemic involvement, pulmonary, renal, and CNS function tests and imaging may be useful.
Patients who present with painful, asymmetric neuropathy, especially if the onset is acute or subacute, should be considered for nerve biopsy. Histopathology of nerve biopsy tissue can be particularly helpful in confirming a clinical diagnosis of vasculitis, as well as sarcoidosis, leprosy, and amyloid neuropathy. Complications include infection, poor wound healing, and painful dysesthesias. In patients with SS who are selected for nerve biopsy, the histopathologic findings include necrotizing vasculitis, lymphocytic vasculitis, and nonspecific perivascular inflammatory infiltrates. A recent retrospective study of 40 patients meeting criteria for pSS suggested that vasculitis was predictive of a clinical response to immunosuppressive therapy.54
When they are evaluated, some of the children with Sjogren's syndrome clearly have SLE these children are said to have secondary Sjogren's syndrome. Sjogren's syndrome is also a known complication of rheumatoid arthritis in adults, but it rarely occurs in children with juvenile arthritis. I have seen children with sarcoidosis who had Sjogren's syndrome and arthritis. Children who have dry eyes and dry mouth but lack other findings suggestive of connective tissue disease have primary Sjogren's syndrome. However, over time some children with what was thought to be primary Sjogren's syndrome ultimately develop more findings and are diagnosed with SLE. The only way to deal with this is to treat the Sjogren's appropriately (usually with hydroxychloroquine and low-dose steroids) and to monitor them carefully.
A complete discussion of the clinical features of sarcoidosis is beyond the scope of this chapter. Briefly, sarcoidosis most commonly affects the lungs, where it may be asymptomatic or cause symptoms of dyspnea, dry cough, wheezing, and chest pain. Lung rales are heard in less than 20 of patients. The diagnosis may be aided by chest radiograph and highresolution chest CT scan showing bilateral, symmetrical lymphadenopathy with or without pulmonary interstitial opacities. Sarcoidosis may also affect other organs (Table 4). Laboratory findings include anemia, leucopenia, thrombocytopenia, hypercalcemia, elevated erythrocyte sedimentation rate, hypergammaglobulinemia, and a positive test for rheumatoid factor (due to chronic immune stimulation). The serum levels of ACE are elevated in 75 of untreated patients with sarcoidosis, but a positive test is not diagnostically specific, and its value in monitoring disease activity is controversial (30,33). TABLE 4 Other Organ Systems in...
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