Gross pathology image showing sarcoidosis with honeycombing: Prominent honeycombing is present in the lower lobes accompanied by fibrosis and some honeycombing in the upper lungs. Honeycombing consists of cystically dilated airways separated by scar tissue resembling the honeycomb of bees. It is a nonspecific end stage of many types of interstitial lung disease.
Monday, February 2, 2015
The Prognosis of Sarcoidosis
The disease can remit spontaneously or become chronic, with exacerbations and remissions. In some persons, it can progress to pulmonary fibrosis and death. About half of cases resolve without treatment or can be cured within 12–36 months, and most within five years. Some cases, however, may persist several decades.[6] Two-thirds of people with the condition achieve a remission within 10 years of the diagnosis.[103] When the heart is involved, the prognosis is generally less favourable, although, corticosteroids appear effective in improving AV conduction.[104][105] The prognosis tends to be less favourable in African Americans, compared to white Americans.[4] Persons with sarcoidosis appear to be at significantly increased risk for cancer, in particular lung cancer, lymphomas,[106] and cancer in other organs known to be affected in sarcoidosis.[107][108] In sarcoidosis-lymphoma syndrome, sarcoidosis is followed by the development of a lymphoproliferative disorder such as non-Hodgkin lymphoma.[109] This may be attributed to the underlying immunological abnormalities that occur during the sarcoidosis disease process.[110] Sarcoidosis can also follow cancer[111] or occur concurrently with cancer.[112][113] There have been reports of hairy cell leukemia,[114] acute myeloid leukemia,[115] and acute myeloblastic leukemia[116] associated with sarcoidosis.
Gross pathology image showing sarcoidosis with honeycombing: Prominent honeycombing is present in the lower lobes accompanied by fibrosis and some honeycombing in the upper lungs. Honeycombing consists of cystically dilated airways separated by scar tissue resembling the honeycomb of bees. It is a nonspecific end stage of many types of interstitial lung disease.
Gross pathology image showing sarcoidosis with honeycombing: Prominent honeycombing is present in the lower lobes accompanied by fibrosis and some honeycombing in the upper lungs. Honeycombing consists of cystically dilated airways separated by scar tissue resembling the honeycomb of bees. It is a nonspecific end stage of many types of interstitial lung disease.
Sarcoidosis Affect All Races
Sarcoidosis most commonly affects young adults of both sexes, although studies have reported more cases in females. Incidence is highest for individuals younger than 40 and peaks in the age-group from 20 to 29 years; a second peak is observed for women over 50.[6][104]
Sarcoidosis occurs throughout the world in all races with an average incidence of 16.5 per 100,000 in men and 19 per 100,000 in women. The disease is most common in Northern European countries and the highest annual incidence of 60 per 100,000 is found in Sweden and Iceland. In the United Kingdom the prevalence is 16 in 100,000.[117] In the United States, sarcoidosis is more common in people of African descent than Caucasians, with annual incidence reported as 35.5 and 10.9 per 100,000, respectively.[118] Sarcoidosis is less commonly reported in South America, Spain, India, Canada, and the Philippines. There may be a higher susceptibility to sarcoidosis in those with celiac disease. An association between the two disorders has been suggested.[119]
There also has been a seasonal clustering observed in sarcoidosis-affected individuals.[120] In Greece about 70% of diagnoses occur between March and May every year, in Spain about 50% of diagnoses occur between April and June, and in Japan it is mostly diagnosed during June and July.[120]
The differing incidence across the world may be at least partially attributable to the lack of screening programs in certain regions of the world, and the overshadowing presence of other granulomatous diseases, such as tuberculosis, that may interfere with the diagnosis of sarcoidosis where they are prevalent.[104] There may also be differences in the severity of the disease between people of different ethnicities. Several studies suggest the presentation in people of African origin may be more severe and disseminated than for Caucasians, who are more likely to have asymptomatic disease.[49] Manifestation appears to be slightly different according to race and sex. Erythema nodosum is far more common in men than in women and in Caucasians than in other races. In Japanese persons, ophthalmologic and cardiac involvement are more common than in other races.[6]
It is more common in certain occupations, namely firefighters, educators, military personnel, persons who work in industries where pesticides are used, law enforcement, and healthcare personnel.[121] In the year after the September 11 attacks, the rate of sarcoidosis incidence went up four-fold (to 86 cases per 100,000).[19][121]
Sarcoidosis occurs throughout the world in all races with an average incidence of 16.5 per 100,000 in men and 19 per 100,000 in women. The disease is most common in Northern European countries and the highest annual incidence of 60 per 100,000 is found in Sweden and Iceland. In the United Kingdom the prevalence is 16 in 100,000.[117] In the United States, sarcoidosis is more common in people of African descent than Caucasians, with annual incidence reported as 35.5 and 10.9 per 100,000, respectively.[118] Sarcoidosis is less commonly reported in South America, Spain, India, Canada, and the Philippines. There may be a higher susceptibility to sarcoidosis in those with celiac disease. An association between the two disorders has been suggested.[119]
There also has been a seasonal clustering observed in sarcoidosis-affected individuals.[120] In Greece about 70% of diagnoses occur between March and May every year, in Spain about 50% of diagnoses occur between April and June, and in Japan it is mostly diagnosed during June and July.[120]
The differing incidence across the world may be at least partially attributable to the lack of screening programs in certain regions of the world, and the overshadowing presence of other granulomatous diseases, such as tuberculosis, that may interfere with the diagnosis of sarcoidosis where they are prevalent.[104] There may also be differences in the severity of the disease between people of different ethnicities. Several studies suggest the presentation in people of African origin may be more severe and disseminated than for Caucasians, who are more likely to have asymptomatic disease.[49] Manifestation appears to be slightly different according to race and sex. Erythema nodosum is far more common in men than in women and in Caucasians than in other races. In Japanese persons, ophthalmologic and cardiac involvement are more common than in other races.[6]
It is more common in certain occupations, namely firefighters, educators, military personnel, persons who work in industries where pesticides are used, law enforcement, and healthcare personnel.[121] In the year after the September 11 attacks, the rate of sarcoidosis incidence went up four-fold (to 86 cases per 100,000).[19][121]
Sarcoidosis also known as The Besnier-Boeck-Schaumann Disease
It was first described in 1877 by Dr. Jonathan Hutchinson, a dermatologist as a condition causing red, raised rashes on the face, arms, and hands.[9] In 1888 the term Lupus pernio was coined by Dr. Ernest Besnier, another dermatologist.[122] Later in 1892 lupus pernio's histology was defined.[122] In 1902 bone involvement was first described by a group of three doctors.[122] Between 1909 and 1910 uveitis in sarcoidosis was first described, and later in 1915 it was emphasised, by Dr. Schaumann, that it was a systemic condition.[122] This same year lung involvement was also described.[122] In 1937 uveoparotid fever was first described and likewise in 1941 Löfgren syndrome was first described.[122] In 1958 the first international conference on sarcoidosis was called in London, likewise the first USA sarcoidosis conference occurred in Washington, DC in the year 1961.[122] It has also been called Besnier-Boeck disease or Besnier-Boeck-Schaumann disease.[123]
Sarcoidosis can cause Glaucoma, Hypertension and Lesions
Skin[edit]
Main article: Cutaneous manifestations of sarcoidosis
Sarcoidosis involves the skin in between 9 and 37% of persons and is more common in African Americans than in their white counterparts.[2] The skin is the second most commonly affected organ, after the lungs.[21] The most common lesions are erythema nodosum, plaques, maculopapular eruptions, subcutaneous nodules, and lupus pernio.[21] Treatment is not required, since the lesions usually resolve spontaneously in two to four weeks. Although it may be disfiguring, cutaneous sarcoidosis rarely causes major problems.[2][22][23] Sarcoidosis of the scalp presents with diffuse or patchy hair loss.[24][25]Heart[edit]
The frequency of cardiac involvement varies and is significantly influenced by race; in Japan over 25% of persons with sarcoidosis experience symptomatic cardiac involvement, whereas in the US and Europe only about 5% of cases present with cardiac involvement.[2] Autopsy studies in the US have revealed a frequency of cardiac involvement of about 20–30%, whereas autopsy studies in Japan have shown a frequency of 60%.[13] The presentation of cardiac sarcoidosis can range from asymptomatic conduction abnormalities to fatal ventricular arrhythmia.[26] Conduction abnormalities are the most common cardiac manifestations of sarcoidosis among persons and can include complete heart block.[27] Second to conduction abnormalities, in frequency, are ventricular arrhythmias and occurs in about 23% of persons with cardiac involvement.[27] Sudden cardiac death, either due to ventricular arrhythmias or complete heart block is a rare complication of cardiac sarcoidosis.[28][29] Cardiac sarcoidosis can cause fibrosis, granuloma formation or the accumulation of fluid in the interstitium of the heart or a combination of the former two.[30]Eye[edit]
Eye involvement occurs in about 10–90% of cases.[13] Manifestations in the eye include uveitis, uveoparotitis, and retinal inflammation, which may result in loss of visual acuity or blindness.[31] The most common ophthalmologic manifestation of sarcoidosis is uveitis.[13][32] The combination of anterior uveitis, parotitis, VII cranial nerve paralysis and fever is called uveoparotid fever or Heerfordt syndrome (D86.8). Development of scleral nodule associated with sarcoidosis have been observed.[33]Sarcoidosis - Respiratory Tract
Localization to the lungs is by far the most common manifestation of sarcoidosis.[16] At least 90% of affected persons experience lung involvement.[2] Overall, about 50% develop permanent pulmonary abnormalities, and 5 to 15% have progressive fibrosis of the lung parenchyma. Sarcoidosis of the lung is primarily an interstitial lung disease in which the inflammatory process involves the alveoli, small bronchi and small blood vessels.[17] In acute and subacute cases, physical examination usually reveals dry rales.[2] At least 5% of persons will suffer pulmonary arterial hypertension.[2][18] Less commonly, the upper respiratory tract (including the larynx, pharynx, and sinuses) may be affected, which occurs in between 5 and 10% of cases.[19]
Sarcoidosis of the lungs can be divided into four stages. Stage 0 — No intrathoracic involvement. Stage I — Bilateral hilar adenopathy. Stage II — Pulmonary parenchyma involved. Stage III — Pulmonary infiltrates with fibrosis.[2] Stage 4 is end-stage lung disease with pulmonary fibrosis and honeycombing.[20]
Sarcoidosis of the lungs can be divided into four stages. Stage 0 — No intrathoracic involvement. Stage I — Bilateral hilar adenopathy. Stage II — Pulmonary parenchyma involved. Stage III — Pulmonary infiltrates with fibrosis.[2] Stage 4 is end-stage lung disease with pulmonary fibrosis and honeycombing.[20]
Sarcoidosis - Origin of the Name - Common Signs and Symptoms
Sarcoidosis was first described in 1877 by an English doctor named Dr. Jonathan Hutchinson as a skin disease causing red, raised lesions on the arms, face, and hands.[9]
The word "sarcoidosis" comes from Greek [σάρκο-] sarcο- meaning "flesh", the suffix -(e)ido (from the Greek εἶδος -eidos [usually omitting the initial e in English as the diphthong epsilon-iota in Classic Greek stands for a long "i" = English ee]) meaning "type", " resembles" or "like", and -sis, a common suffix in Greek meaning "condition". Thus the whole word means "a condition that resembles crude flesh".
Sarcoidosis is a systemic inflammatory disease that can affect any organ, although it can be asymptomatic and is discovered by accident in about 5% of cases.[11] Common symptoms, which tend to be vague, include fatigue (unrelieved by sleep; occurs in 66% of cases), lack of energy, weight loss, joint aches and pains (which occur in about 70% of cases),[6] arthritis (14–38% of persons), dry eyes, swelling of the knees, blurry vision, shortness of breath, a dry, hacking cough, or skin lesions.[1][12][13][14] Less commonly, people may cough up blood.[1] The cutaneous symptoms vary, and range from rashes and noduli (small bumps) to erythema nodosum, granuloma annulare, or lupus pernio. Sarcoidosis and cancer may mimic one another, making the distinction difficult.[15]
The combination of erythema nodosum, bilateral hilar lymphadenopathy, and joint pain is called Löfgren syndrome which has a relatively good prognosis.[1] This form of the disease occurs significantly more commonly in Scandinavian patients, than in those of non-Scandinavian origin.[4]
The word "sarcoidosis" comes from Greek [σάρκο-] sarcο- meaning "flesh", the suffix -(e)ido (from the Greek εἶδος -eidos [usually omitting the initial e in English as the diphthong epsilon-iota in Classic Greek stands for a long "i" = English ee]) meaning "type", " resembles" or "like", and -sis, a common suffix in Greek meaning "condition". Thus the whole word means "a condition that resembles crude flesh".
Sarcoidosis is a systemic inflammatory disease that can affect any organ, although it can be asymptomatic and is discovered by accident in about 5% of cases.[11] Common symptoms, which tend to be vague, include fatigue (unrelieved by sleep; occurs in 66% of cases), lack of energy, weight loss, joint aches and pains (which occur in about 70% of cases),[6] arthritis (14–38% of persons), dry eyes, swelling of the knees, blurry vision, shortness of breath, a dry, hacking cough, or skin lesions.[1][12][13][14] Less commonly, people may cough up blood.[1] The cutaneous symptoms vary, and range from rashes and noduli (small bumps) to erythema nodosum, granuloma annulare, or lupus pernio. Sarcoidosis and cancer may mimic one another, making the distinction difficult.[15]
The combination of erythema nodosum, bilateral hilar lymphadenopathy, and joint pain is called Löfgren syndrome which has a relatively good prognosis.[1] This form of the disease occurs significantly more commonly in Scandinavian patients, than in those of non-Scandinavian origin.[4]
"Sarcoidosis" is Represented By The Purple Ribbon
Sarcoidosis, also called sarcoid, is a disease involving abnormal collections of inflammatory cells (granulomas) that can form as nodules in multiple organs.[1] The granulomas are most often located in the lungs or its associated lymph nodes, but any organ can be affected.[2] Sarcoidosis seems to be caused by an immune reaction to an infection or some other trigger (called an antigen, which may be from one's environment) that continues even after the initial infection or other antigen is cleared from the body.[3] In most cases it clears up by itself without any medical intervention, but some cases go on to affect the person long-term or become life-threatening and require medical intervention, most often with medications.[2] The average mortality rate is less than 5% in untreated cases.[4]
Treatment is usually designed to help relieve the symptoms and thus does not directly alter the course of the disease.[5] This treatment usually consists of drugs like ibuprofen or aspirin.[5] In cases where the condition develops to the point that it has a progressive and/or life-threatening course, the treatment is most often steroid treatment with prednisone or prednisolone.[5] Alternatively, drugs that are most commonly used to treat cancer and suppress the immune system, such as methotrexate, azathioprine and leflunomide, may be used.[5][6][7]
In the United States it most commonly affects people of Northern European (especially Scandinavian or Icelandic) or African/African American ancestry between the ages of 20 and 29, although any race or age group can be affected.[4] Japan has a lower rate of sarcoidosis than the United States, although in these people the disease is usually more aggressive in its course with the heart often affected.[4] Japanese individuals also have a different peak age for sarcoidosis, 25–40 years of age.[8] It occurs about twice as often in women, where it usually takes a more aggressive course.[4] In developing countries it often goes misdiagnosed as tuberculosis (TB) as its symptoms often resemble those of TB.[4]
Treatment is usually designed to help relieve the symptoms and thus does not directly alter the course of the disease.[5] This treatment usually consists of drugs like ibuprofen or aspirin.[5] In cases where the condition develops to the point that it has a progressive and/or life-threatening course, the treatment is most often steroid treatment with prednisone or prednisolone.[5] Alternatively, drugs that are most commonly used to treat cancer and suppress the immune system, such as methotrexate, azathioprine and leflunomide, may be used.[5][6][7]
In the United States it most commonly affects people of Northern European (especially Scandinavian or Icelandic) or African/African American ancestry between the ages of 20 and 29, although any race or age group can be affected.[4] Japan has a lower rate of sarcoidosis than the United States, although in these people the disease is usually more aggressive in its course with the heart often affected.[4] Japanese individuals also have a different peak age for sarcoidosis, 25–40 years of age.[8] It occurs about twice as often in women, where it usually takes a more aggressive course.[4] In developing countries it often goes misdiagnosed as tuberculosis (TB) as its symptoms often resemble those of TB.[4]
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