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...
This is an idiopathic chronic granulomatous disease that occasionally affects the larynx. Peak onset is from the age of 20 to 40 years. There is a higher incidence in African Americans, Puerto Ricans, and Scandinavians, and it is sometimes familial. The pathogenesis of sarcoidosis is unknown. One theory is that it results from a mycobacterial infection. It is usually systemic, most commonly including the lungs and hilar lymph nodes. Isolated local involvement is rare, so head and neck manifestations are virtually always in the context of disease in other sites. A review of 2319 patients seen at the Mayo Clinic for sarcoidosis found head and neck involvement in 9 of cases. Of these, the eyes and lacrimal system were involved in 40 , the nose in 13 , and the larynx in 6 (10). Symptoms of laryngeal involvement include hoarseness, dysphagia, stridor, and dyspnea. On physical examination, supraglottic involvement appears as a pale and diffusely enlarged, sometimes nodular epiglottis. Vocal...
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.
The detection of abnormal lymphoid tissue is an important aspect of chest imaging. This is true especially for the mediastinum, the central portion of the chest that contains the heart, major blood vessels, and other structures. Abnormally enlarged lymph nodes, or lymphadenopathy, in the mediastinum can be caused by primary malignancy such as lymphoma, metastatic disease that results from the spread of breast or lung cancer through the lymphatics, tuberculosis, or non-infectious inflammatory diseases such as sarcoidosis. Typically radiologists can easily locate lymph nodes in a chest scan. The detection task is therefore to determine which of the located lymph nodes are enlarged.
Hypersplenism refers to a condition in which the blood cells are culled excessively within the spleen. It usually occurs when the haemopoietic cells are intrinsically abnormal - as in idiopathic thrombocytopenic purpura (ITP), congenital spherocytosis and acquired haemolytic anaemias (due to various leukaemias, Hodgkin's lymphoma, sarcoidosis, lupus erythematosus, etc.).
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
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.
Differential Diagnosis Idiopathic pericarditis, infectious pericarditis (viral, bacterial, mycoplasmal, mycobacterial), Lyme disease, uremia, neoplasm, connective tissue disease, lupus, rheumatic fever, polymyositis, myxedema, sarcoidosis, post myocardial infarction pericarditis (Dressler's syndrome), drugs (penicillin, isoniazid, procainamide, hydralazine).
Intrahepatic Causes of Jaundice Viral hepatitis, medication-related hepatitis, acute fatty liver of pregnancy, alcoholic hepatitis, cirrhosis, primary biliary cirrhosis, autoimmune hepatitis, Wilson's disease, right heart failure, total parenteral nutrition Dubin Johnson syndrome, Rotor's syndrome (direct hyperbilirubinemia) Gilbert's syndrome, Crigler-Niger syndrome (indirect) sclerosing cholangitis, sarcoidosis, amyloidosis, tumor.
High Resolution (thin section) CT scan (HRCT) - slices are 1-3 mm thick. HRCT is of value for the investigation of sarcoidosis, lymphoma, cryptogenic and extrinsic alveolitis, occupational lung disease, bronchiectasis and lymphangitis carcinomatosa. It is also used to distinguish emphysema from interstitial lung disease or pulmonary vascular disease. Biochemistry - alpha-1-antitrypsin levels, autoantibodies, aspergillus antibodies, IgE to specific allergens. Hypercalcaemia occurs in 1 in 5 patients with sarcoidosis, as a paraneoplastic syndrome notably in association with squamous cell carcinoma and secondary to bony metastases. Hyponatraemia is seen in association with small cell carcinoma. Transthoracic needle aspiration and biopsy (TTNA and TTNB) percutaneous needle aspiration and biopsy - are performed under fluoroscopic or CT guidance. They successfully diagnose lung cancer with at least 85-90 accuracy. The most common indication for aspiration is to evaluate a solitary...
Myocarditis viral, bacterial or fungal. In developed countries viral infections predominate. Parasitic aetiologies include toxoplasmosis and the protozoon Trypanosoma cruzi. Granuloma-tous myocarditis can occur due to TB or sarcoidosis. Myocarditis occurs secondary to collagen vascular disease, especially rheumatic fever, and may also be drug or radiation induced.
Sarcoidosis of the heart. Clin Cardiol. 1993 16 462-72. 5. Sekiguchi M, Yazaki Y, Isobe M, Hiroe M. Cardiac sarcoidosis diagnostic, prognostic, and therapeutic considerations. Cardiovasc Drugs Ther. 1996 10 495-510. Figure 63. Sarcoidosis, histologic section. Non-necrotizing granuloma (Hematoxylin and Eosin, 20X. Courtesy of Dr. Sui Zee, Albert Einstein College of Medicine). Figure 63. Sarcoidosis, histologic section. Non-necrotizing granuloma (Hematoxylin and Eosin, 20X. Courtesy of Dr. Sui Zee, Albert Einstein College of Medicine).
Differentiating HS from Crohn's disease merits attention. At times these diseases can be clinically indistinguishable and authors have emphasized that although foreign body granulomas are a common finding in HS, the presence of discrete epithelioid granulomas in the dermis away from the site of active inflammation is unusual and should alert the pathologist to the possibility of systemic granulomatous disease such as Crohn's or sarcoidosis 22 . Several authors have reported the co-morbidity of HS and Crohn's 23 (see Fig. 4.10).
Addison's disease chronic adrenal cortical insufficiency is most commonly of autoimmune aetiology but other causes include tuberculosis, malignant infiltration, fungal infection and sarcoidosis. Treatment is aimed at the underlying cause, in addition to steroid replacement. Surgical resection is seldom indicated.
He did have findings that were strongly suggestive of heart failure despite maintaining normal blood pressure and pulse rate. His complaints of chest pain were associated with shortness of breath, consistent with pulmonary congestion secondary to left heart failure. The chest X-ray demonstrated an enlarged heart, which may be secondary to pre-existing cardiomegaly, or to an increase of the cardiac silhouette due to dilatation. However, it also showed increased vascular congestion and interstitial edema, which is one of the earliest signs of elevated left atrial pressure, presumably due to increased end-diastolic pressure in a failing left ventricle. Moreover, he had an S4 gallop rhythm, which is a pre-systolic extra sound due to atrial contraction with blood forced into a less compliant left ventricle. Myocardial infarction decreases the compliance of the left ventricle due to the stiffness of non-contracting myocytes, and the presence of interstitial edema. Although not specific for...
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.
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...