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Sarcoidosis

Sarcoidosis (Besnier-Boeck disease, Besnier-Boeck-Schaumann disease) is a multisystem inflammatory disease of unknown etiology, characterized by non-necrotizing granulomatous inflammation, predominantly in the lungs and intrathoracic lymph nodes.

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Presentation

The presentation of the disease is highly dependent on the extent of damage to the organ. 5% of cases have no outward clinical manifestations making it hard to be diagnosed. There are also cases where the condition can be asymptomatic. However, with the aid of a chest X-ray the condition of the lungs can be clearly seen. The most common complaints are hyperthermia, anorexia and arthralgia; this is accompanied by pulmonary symptoms. A group of symptoms consisting of fever, bilateral hilar lymphadenopathy and polyarthralgia has good prognosis.

Splenomegaly
  • Spleen (causing abdominal discomfort and distension due to splenomegaly). Upper respiratory tract (causing nosebleeds, rhinitis, nasal obstruction/masses or tonsillar involvement).[patient.info]
  • Sarcoid myopathy Sarcoid myopathy was described for the first time in a 17-year old female patient who presented with lupus pernio, splenomegaly, and multiple muscle nodules.[doi.org]
  • […] anomaly in sarcoidosis. [26] Anemia occurs in about 20% of people with sarcoidosis. [26] Leukopenia is less common and occurs in even fewer persons but is rarely severe. [26] Thrombocytopenia and hemolytic anemia are fairly rare. [18] In the absence of splenomegaly[en.wikipedia.org]
  • In the absence of splenomegaly, hematological alterations may be exceptionally due to a granulomatous infiltration of the bone marrow or to an autoimmune process, mainly hemolytic anemia or thrombopenia [ 37 ].[doi.org]
Generalized Lymphadenopathy
  • Praveen Ranganath, Rajendra Kapila, Vivek Vadehra, Qing Wang, Eugenio Capitle and Nasrin Ghesani , Generalized Lymphadenopathy and 18-Fluorine Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography , Sexually Transmitted Diseases , 42 , 2[doi.org]
Fatigue
  • Sarcoidosis-associated fatigue: an often forgotten symptom. Expert Rev. Clin. Immunol. 9(2), 109–110 (2013).[doi.org]
  • Patients are eligible if they have a diagnosis of sarcoidosis, significant fatigue (measured using the Fatigue Assessment Scale) and have stable disease.[ncbi.nlm.nih.gov]
  • Depressive symptoms impact HQ and may influence perception of fatigue. Both fatigue and depression have a negative impact on HQ in sarcoidosis.[ncbi.nlm.nih.gov]
  • Respiratory muscle weakness occurs in sarcoidosis and is related to decreased exercise capacity, greater fatigue, dyspnea, and lower quality of life in sarcoidosis patients.[ncbi.nlm.nih.gov]
Fever
  • Heerfordt syndrome (also called uveoparotid fever) manifests as swelling of the parotid gland (due to sarcoid infiltration), inflammation of the eye ( uveitis ), chronic fever, and less often weakness or paralysis of the facial nerve.[merckmanuals.com]
  • Fever and night sweats may recur throughout the illness. Lungs: The organ most affected by sarcoidosis is the lung.[web.archive.org]
  • Sarcoidosis Patient will present as a 30-year-old African American female with a cough, fever, and generalized body aches.[smartypance.com]
  • Abstract A 53-year-old woman from Southern India presented with weight loss, anorexia, fever and asthenia.[ncbi.nlm.nih.gov]
  • This case should remind readers to consider sarcoidosis as a rare cause of fever of unknown origin or of periosteal and tracheal lesions found on imaging.[doi.org]
Weight Loss
  • We present an interesting case of gastric sarcoidosis in a 39-year-old Caucasian man with symptoms of epigastric pain and profound weight loss.[ncbi.nlm.nih.gov]
  • Night sweats and weight loss are common symptoms of the disease. Common signs and symptoms in children are fatigue, loss of appetite, weight loss, bone and joint pain, and anemia .[web.archive.org]
  • Night sweats and weight loss are common symptoms of the disease. Common signs and symptoms in children are fatigue (tiredness), loss of appetite, weight loss, bone and joint pain, and anemia .[web.archive.org]
  • During the previous months, he had felt more tired and had experienced an unintended weight loss of 6-8 kg. A CT scan revealed an inhomogeneous thyroid gland and enlarged lymph nodes along the cervical vessels and in the mediastinum.[ncbi.nlm.nih.gov]
  • Abstract A 53-year-old woman from Southern India presented with weight loss, anorexia, fever and asthenia.[ncbi.nlm.nih.gov]
Weakness
  • Other chest manifestations include adenopathy, pulmonary hypertension, and pulmonary muscle weakness. Fibrotic lung disease can lead to bronchiectasis, which can become infected.[ncbi.nlm.nih.gov]
  • Sarcoidosis patients frequently experience fatigue, exercise intolerance and muscle weakness, resulting in reduced quality of life (QOL).[ncbi.nlm.nih.gov]
  • Respiratory muscle weakness occurs in sarcoidosis and is related to decreased exercise capacity, greater fatigue, dyspnea, and lower quality of life in sarcoidosis patients.[ncbi.nlm.nih.gov]
  • A 64-year-old woman was admitted to our hospital for blurred vision, muscle weakness of extremities, Raynaud's phenomenon, and exertional dyspnea. We diagnosed her as having systemic sclerosis complicated with sarcoidosis.[ncbi.nlm.nih.gov]
  • […] patients (steps per day CONCLUSION: While exercise capacity and quality of life measurements were robust predictors of physical activity in patients with sarcoidosis, associations of objectively measured physical activity with fatigue were surprisingly weak[ncbi.nlm.nih.gov]
Malaise
  • Abstract A 66-year-old woman, who was diagnosed with iritis, visited our hospital due to general malaise. A blood analysis revealed hypercalcemia. Computed tomography revealed mediastinal and hilar lymph node hyperplasia.[ncbi.nlm.nih.gov]
  • Fever, fatigue, vague chest pain, a feeling of illness (malaise), loss of appetite, weight loss, and aching joints may be the first indications of a problem in about one third of people.[merckmanuals.com]
  • Fever, arthralgia, and malaise may occur. EN is more common in European, especially Scandinavian, women of childbearing age than in other people.[emedicine.medscape.com]
  • About one-third of people with sarcoidosis have general symptoms such as: Fever Fatigue, weakness Weight loss or loss of appetite Don’t feel well (malaise) People with sarcoidosis whose lungs are affected by the disease (90 percent of individuals) have[verywell.com]
  • Nervous system Granulomas can appear in the brain, spinal cord, and facial and optic nerves May result in headache, confusion and malaise Facial paralysis Musculoskeletal Arthritis ( inflammation of the joints), periarthritis ( inflammation of surrounding[dermnetnz.org]
Cough
  • Her symptoms started with a dry cough, which became chronic about a year after it first developed. Unable to speak a sentence without coughing, Romaine began taking medicine to treat her sarcoidosis.[web.archive.org]
  • She developed a cough and dyspnea after 6 months of etanercept treatment. The other developed erythema nodosum and a plaque lesion on the right arm after 1 year of etanercept. Imaging showed, in both cases, mediastinal adenopathies.[ncbi.nlm.nih.gov]
  • Symptoms include cough, dyspnea, and chest pain. The entire respiratory tract can be involved. The most common areas of involvement are the airways and interstitium.[ncbi.nlm.nih.gov]
  • Almost all people affected by sarcoidosis have lung or chest symptoms: Chest pain (most often behind the breast bone) Dry cough Shortness of breath Coughing up blood (rare, but serious) Symptoms of general discomfort may include: Fatigue Fever Joint ache[nlm.nih.gov]
Dyspnea
  • Respiratory muscle weakness occurs in sarcoidosis and is related to decreased exercise capacity, greater fatigue, dyspnea, and lower quality of life in sarcoidosis patients.[ncbi.nlm.nih.gov]
  • A score of 36 indicated severe fatigue. 27 Dyspnea The Modified Medical Research Council dyspnea scale was used to evaluate dyspnea severity during activity, graded from 0 (absence of dyspnea during strenuous exercise) to 4 (dyspnea during daily activities[doi.org]
  • The Fatigue Assessment Scale (FAS), World Health Organization Quality of Life-BREF assessment instrument (WHOQOL-BREF),Medical Research Council (MRC) dyspnea scale, Visual Analogue Scale (VAS), six-minute walk test (6MWT), submaximal bicycle test and[ncbi.nlm.nih.gov]
  • Thereafter, her symptoms improved except for exertional dyspnea, and she began to complain of productive cough thirteen months after corticosteroid and immunosuppressant therapy.[ncbi.nlm.nih.gov]
  • A 67-year female patient, who was diagnosed as sarcoidosis previously, was admitted to our hospital with symptoms of dyspnea, chest pain and fatigue. Middle lobe atelectasis and endobronchial lesion were observed in thorax computed tomography (CT).[ncbi.nlm.nih.gov]
Dry Cough
  • Her symptoms started with a dry cough, which became chronic about a year after it first developed. Unable to speak a sentence without coughing, Romaine began taking medicine to treat her sarcoidosis.[web.archive.org]
  • In most cases, sarcoidosis is revealed by persistent dry cough, eye or skin manifestations, peripheral lymph nodes, fatigue, weight loss, fever or night sweats, and erythema nodosum.[ncbi.nlm.nih.gov]
  • For example, patients suffering from pulmonary sarcoidosis may have a persistent dry cough, shortness of breath, wheezing, and chest pain. There also can be fatigue, weakness and weight loss.[ccohs.ca]
Persistent Cough
  • Sarcoidosis Symptoms and Facts Symptoms of sarcoidosis vary widely depending on the organs involved: In the lungs: persistent cough, chest pain, and shortness of breath (these are the most common symptoms of the disease) In the skin: rashes, skin discoloration[uofmhealth.org]
  • When it happens, depending on the extent of the fibrosis, the patient usually suffers a persistent cough, may experience shortness of breath, and may not be able to tolerate exercise.[healthcommunities.com]
  • Fatigue and persistent cough are usually improved with steroid treatment. If steroids are prescribed, you should see your doctor at regular intervals so that he or she can monitor the disease and the side effects of treatment.[webmd.com]
  • The symptoms of sarcoidosis depend on which organs are affected, but typically include: tender, red bumps on the skin shortness of breath a persistent cough For many people with sarcoidosis, symptoms often improve without treatment within a few months[nhs.uk]
  • For pulmonary patients, increased tissue fibrosis, decreased airflow, persistent cough, and a possibly of bronchiectasis. Of the stages, remission periods vary greatly.[physio-pedia.com]
Pleural Effusion
  • To the best of our knowledge, this is the first described case of sarcoidosis presenting as large transudative pleural effusion.[ncbi.nlm.nih.gov]
  • KEYWORDS: endobronchial nodules; miliary opacities; pancreatic sarcoidosis; pleural effusion; sarcoidosis[ncbi.nlm.nih.gov]
  • Note that CXR may also demonstrate pleural involvement, such as a pneumothorax or pleural effusion. Stage 0 - normal findings on chest radiograph. Stage I - bilateral hilar lymphadenopathy (which may be accompanied by paratracheal adenopathy).[patient.info]
Dysphagia
  • Because he had persistent dysphagia, he underwent total thyroidectomy with resolution of dysphagia. Histopathological examination of the thyroid revealed non necrotizing granulomas consistent with sarcoidosis.[ncbi.nlm.nih.gov]
  • The following symptoms are encountered relatively commonly as a result of neurological involvement: Facial numbness, dysphagia, hoarseness, headache, visual field defects, polydipsia, hearing impairment, lesions of cranial nerves VII, VIII, IX and X,[patient.info]
Chest Pain
  • A 67-year female patient, who was diagnosed as sarcoidosis previously, was admitted to our hospital with symptoms of dyspnea, chest pain and fatigue. Middle lobe atelectasis and endobronchial lesion were observed in thorax computed tomography (CT).[ncbi.nlm.nih.gov]
  • Symptoms include cough, dyspnea, and chest pain. The entire respiratory tract can be involved. The most common areas of involvement are the airways and interstitium.[ncbi.nlm.nih.gov]
  • For example, patients suffering from pulmonary sarcoidosis may have a persistent dry cough, shortness of breath, wheezing, and chest pain. There also can be fatigue, weakness and weight loss.[ccohs.ca]
  • If granulomas (inflamed lumps) form in your lungs, you may wheeze, cough, feel short of breath, or have chest pain. Or, you may have no symptoms at all. Some people who have sarcoidosis feel very fatigued (tired), uneasy, or depressed.[web.archive.org]
  • Heart: Granulomas that form in the heart may cause chest pain (angina) or heart failure. Granulomas that form near the heart's electrical conducting system can trigger potentially fatal irregularities in the heartbeat.[web.archive.org]
Hepatomegaly
  • […] vision, watery eyes and photophobia (dislike of light) iris nodules retinochoroiditis conjunctivitis lacrimal gland involvement optic nerves proptosis (protruding eyeball) Uncommonly, cataracts, glaucoma, and blindness can result Liver Up to 1/3 have hepatomegaly[dermnetnz.org]
  • Licence: CC BY-SA 3.0 Liver: 80 % Elevated liver enzymes and in some cases hepatomegaly may be found. Clinically, there are usually no symptoms. Joints: 40 % Joint pain and swollen joints occur frequently.[lecturio.com]
  • Abdomen Check for hepatomegaly and splenomegaly. Cardiorespiratory Chest signs of sarcoidosis are usually not detected unless advanced interstitial lung disease is present, when there may be scattered crackles.[patient.info]
  • ] In males, sarcoidosis may lead to infertility. [60] Around 70% of people have granulomas in their livers, although only in about 20–30% of cases, liver function test anomalies reflecting this fact are seen. [19] [26] About 5–15% of persons exhibit hepatomegaly[en.wikipedia.org]
Red Eye
  • eyes Sensitivity to light Blurred vision Note that this is not an all-encompassing list.[stopsarcoidosis.org]
  • […] persistent cough, chest pain, and shortness of breath (these are the most common symptoms of the disease) In the skin: rashes, skin discoloration In the brain: Bell’s palsy, numbness, or tingling in the arm or leg In the eye: blurry vision, painful red[uofmhealth.org]
  • Some people with sarcoidosis complain of dry, sore, and red eyes, probably because sluggish tear glands that have been affected by the disorder no longer produce enough tears to keep the eyes lubricated.[web.archive.org]
  • Some people with sarcoidosis complain of dry, sore, and red eyes, probably because tear glands that have been affected by the disorder no longer produce enough tears to keep the eyes lubricated.[merckmanuals.com]
Conjunctival Nodule
  • If present, biopsy of skin lesions, conjunctival nodules, enlarged peripheral lymph nodes, or an enlarged lacrimal or parotid gland is preferable to an intra-thoracic biopsy due to the less invasive nature of the procedure.[clinicaladvisor.com]
Arthritis
  • Semin Arthritis Rheum 34(5 Suppl1):34–38 PubMed CrossRef Google Scholar 3.[doi.org]
  • KEYWORDS Sarcoidosis - rheumatic diseases - arthritis - myopathy[dx.doi.org]
  • Why: sarcoidosis can result in an acute arthritis commonly affecting the ankles and knees and less commonly the proximal interphalangeal joints, wrists, and elbows. The acute arthritis is symmetric and lasts for a few weeks.[icd9data.com]
  • We then provide a detailed description of the rheumatologic manifestations of sarcoidosis and present a treatment algorithm based on current clinical evidence for patients with sarcoid arthritis.[ncbi.nlm.nih.gov]
Arthralgia
  • Other symptoms include anterior uveitis, fever, ankle periarthritis, arthralgias, and pulmonary involvement.[emedicine.medscape.com]
  • The most common form, the acute form, may be the first sign of sarcoidosis and present with arthralgia, arthritis, or periarthritis.[ncbi.nlm.nih.gov]
  • The acute course is marked by the presence of fever, arthralgia (painful joints), night sweats, acute arthritis, erythema nodosum (inflammation under the skin), dry cough, dyspnea (laboured breathing) when under stress, and swollen lymph nodes between[ims.uniklinik-freiburg.de]
  • […] people affected by sarcoidosis have lung or chest symptoms: Chest pain (most often behind the breast bone) Dry cough Shortness of breath Coughing up blood (rare, but serious) Symptoms of general discomfort may include: Fatigue Fever Joint ache or pain (arthralgia[nlm.nih.gov]
Skin Lesion
  • Accordingly, a 6-month course of multidrug therapy led to a marked improvement in the skin lesions.[ncbi.nlm.nih.gov]
  • CASE CHARACTERISTICS: 8-year-old boy with erythematous itchy skin lesion and recurrent facial palsy. OBSERVATION: He had a past history of aseptic meningitis and nephrocalcinosis.[ncbi.nlm.nih.gov]
  • If you have a specific question or concern about a skin lesion or disease, please consult a dermatologist.[aocd.org]
  • For patients with more than 1 skin lesion, the lesion with the highest combined score of induration, erythema, desquamation, and area of involvement was designated the index lesion.[doi.org]
  • Several patients with sarcoidosis-specific skin lesions in venous puncture sites have been reported. However, in these patients the pathogenesis of the cutaneous lesions is not clear because the presence of foreign bodies is not to be expected.[ncbi.nlm.nih.gov]
Night Sweats
  • If you have symptoms, they may include cough shortness of breath weight loss night sweats fatigue tests to diagnose sarcoidosis include chest x-rays, lung function tests, and a biopsy. Not everyone who has the disease needs treatment.[icd9data.com]
  • In most cases, sarcoidosis is revealed by persistent dry cough, eye or skin manifestations, peripheral lymph nodes, fatigue, weight loss, fever or night sweats, and erythema nodosum.[ncbi.nlm.nih.gov]
  • If you have symptoms, they may include Cough Shortness of breath Weight loss Night sweats Fatigue Tests to diagnose sarcoidosis include chest x-rays, lung function tests, and a biopsy. Not everyone who has the disease needs treatment.[medlineplus.gov]
  • Night sweats and weight loss are common symptoms of the disease. Common signs and symptoms in children are fatigue, loss of appetite, weight loss, bone and joint pain, and anemia .[web.archive.org]
Alopecia
  • alopecia [ 19 ].[web.archive.org]
  • Mohammad-Ali Yazdani Abyaneh, Preethi Raghu, Kenneth Kircher, Heinz Kutzner, Kortz Alison and John Andrew Carlson , Circumscribed cicatricial alopecia due to localized sarcoidal granulomas and single-organ granulomatous arteritis: a case report and systematic[doi.org]
  • Lesions can heal with scarring, and, if plaques involve the scalp, they may lead to alopecia. Patients with plaque lesions usually have more severe systemic involvement. Annular sarcoidosis.[emedicine.medscape.com]
  • CUTE ISTIOCITOSI SARCOIDOSI (MALATTIA DI BESNIER-BOECK-SCHAUMANN) TUMORI MESENCHIMALI E LESIONI PSEUDOTUMORALI NEI O NEVI MELANOMA CUTANEO ALTERAZIONI DELLA PIGMENTAZIONE CUTANEA ITTIOSI E DERMATOSI ITTIOSIFORMI MALATTIE E ALTERAZIONI DEGLI ANNESSI Alopecia[medicalinformation.it]
  • Alopecia and discoid lupus erythematous-like lesions may occur in patients with sarcoidosis. Early sarcoidosis may mimic juvenile rheumatoid arthritis with the findings of rash, arthritis, and uveitis.[doi.org]
Cutaneous Manifestation
  • We present a rare case of a 37-year-old woman with bihilar, mediastinal, and abdominal lymphadenopathy in conjunction with a histologically proven cutaneous manifestation of sarcoidosis in a tattoo of the lower back exhibiting an increased uptake of FDG[ncbi.nlm.nih.gov]
  • Physicians commonly have difficulty in differentiating tuberculoid form of leprosy (TL) from sarcoidosis' cutaneous manifestation.[ncbi.nlm.nih.gov]
  • Sarcoidosis presenting as a scarring alopecia: report of a rare cutaneous manifestation of systemic sarcoidosis. Dermatology . 1996;193(2):144-6. [ PubMed ] 11. Bleehen SS. Systemic sarcoidosis with scalp involvement.[web.archive.org]
  • manifestations The remainder of this article pertains to a general discussion of sarcoidosis.[radiopaedia.org]
Skin Plaque
  • Laser surgery has been used in treating disfiguring skin plaques and lupus pernio. Success in individual cases has been reported with allopurinol, isotretinoin, leflunomide, pentoxifylline, infliximab and thalidomide.[dermnetnz.org]
Headache
  • This can cause a number of symptoms, such as abnormal heartbeats, shortness of breath, headaches, and vision problems. If sarcoidosis affects the heart or brain, serious complications can occur.[web.archive.org]
  • This can cause many symptoms, such as abnormal heartbeats, shortness of breath, headaches, and vision problems. If sarcoidosis affects the heart or brain, serious complications can occur.[web.archive.org]
  • […] or pain (arthralgia) Weight loss Skin symptoms may include: Hair loss Raised, red, firm skin sores ( erythema nodosum ), almost always on the front part of the lower legs Rash Scars that become raised or inflamed Nervous system symptoms may include: Headache[nlm.nih.gov]
  • Other Symptoms: Because sarcoidosis can affect any organ in the body, a wide variety of symptoms can be seen, including: Fatigue Unexplained weight loss Night sweats Overall feeling of sickness Irregular heart beat Swollen legs Headaches Visual problems[stopsarcoidosis.org]
Peripheral Neuropathy
  • EFNS Guidelines on the use of skin biopsy in the diagnosis of peripheral neuropathy. Eur J Neurol. 2005;12:1–12. CrossRef Google Scholar 23. Shy ME, Frohman EM, So YT, Arezzo JC, Cornblatj DR, et al.[doi.org]
  • Peripheral neuropathy is a rare, yet treatable manifestation of sarcoidosis, a multisystem disorder characterized by the presence of non-caseating granulomas that are seldom found in nerve biopsy specimens.[ncbi.nlm.nih.gov]
  • New symptoms of length-dependent peripheral neuropathy occurred in two of our subjects, on average 4 months after starting the medication. Peripheral neuropathy has been reported as a complication of leflunomide [ 25 ].[doi.org]
  • Sarcoid peripheral neuropathy. Neurology 1991 ; 41 : 1558 –61. 2,103 Views 103 Citations Related articles in PubMed[dx.doi.org]
Cranial Nerve Involvement
  • No actual sensory deficit Ankle reflexes abolished Cranial nerve involvement No No Facial diplegia No No No No No No No No CSF cells/ml n.d. n.d. 36 lymph. 3 lymph. n.d. 1 lymph. 5 lymph. 2 lymph. n.d.[dx.doi.org]
  • Peripheral nerve or cranial nerve involvement with sarcoidosis often does not need to be treated, or it will respond to a short course of steroids.[clinicaladvisor.com]

Workup

Diagnosing sarcoidosis is not only done with one test. Since the disease is quite complex, it requires multiple combination laboratory examinations to come up with a concrete diagnosis. The signs and symptoms of sarcoidosis are very general as well, so the disease needs thorough medical examination. The following are the major laboratory examinations conducted to diagnose the disease.

Imaging

  • Chest X-ray – a primary imaging study done in a person suspected to have sarcoidosis. This is done to visualize infiltrates present within the lugs and even swollen lymph nodes.
  • Bronchoscopy – examines the patency of the bronchial pathways as well as checks for granulomas. It is also done for the purpose of extracting a tissue for biopsy.
  • CT scan – done to acquire a clearer view of the lungs as well as the lymph nodes.
  • Pulmonary function test – measures lung components and functioning [1]

Laboratory tests

  • Blood test – ensures overall health and identifies abnormal blood finding
  • Serum markers – serum amyloid A (SAA), soluble interleukin-2 receptor (sIL-2R), lysozyme, angiotensin-converting enzyme (ACE) and glycoprotein are checked for normal levels. These three are known markers of sarcoidosis [2].

These are just a few of the most important workups done to identify sarcoidosis. The first step in diagnosing sarcoidosis is to rule out other pulmonary conditions such as tuberculosis or COPD among others. As mentioned, the signs and symptoms are very general, they can occur and mimic that of other diseases; the reason why it’s important to set aside other diseases.

Anergy
  • Compared with the asymptomatic group, symptomatic patients had impaired peripheral responses to TLR2, TLR4, and TLR9 ligands (anergy) and reduced peripheral populations of CD4 1 FoxP3 1 regulatory T cells (Tregs).[ncbi.nlm.nih.gov]
  • […] environmental agents Increased risk with some HLA genotypes Increase in pulmonary T cell CD4:CD8 ratio Increase in T cell derived cytokines IL-2 and IFN-gamma Increase in other cytokines (IL-8, TNF, others) in microenvironment Clinical features Commonly anergy[pathologyoutlines.com]
  • Anergy is believed to be responsible for the increased risk of sarcoidosis patients to aquire opportunistic infections and cancer.[doi.org]
  • This paradoxic state of simultaneous hyper- and hypoactivity is suggestive of a state of anergy. The anergy may also be responsible for the increased risk of infections and cancer.[en.wikipedia.org]
  • The quantinterferon test may be more specific and sensitive in detecting latent tuberculosis, and may be helpful in the patient with known anergy (a common occurrence in sarcoidosis) [ 135 ].[doi.org]
Pulmonary Infiltrate
  • Sarcoidosis can affect all individuals with any race, sex, or age but commonly affects young- and middle-aged adults and usually presents with bilateral hilar lymphadenopathy, pulmonary infiltration, skin and ocular lesions.[ncbi.nlm.nih.gov]
  • Sarcoidosis is a granulomatous disease that most frequently affects the lungs with pulmonary infiltrates and/or bilateral hilar and mediastinal lymphadenopathy.[ncbi.nlm.nih.gov]
  • Abstract Sarcoidosis is a granulomatous disease that most frequently affects the lungs with pulmonary infiltrates and/or bilateral hilar and mediastinal lymphadenopathy.[doi.org]
  • This disease most commonly presents with bilateral hilar lymphadenopathy, pulmonary infiltrations, and skin and eye lesions. Locomotor system involvement is observed at a range of 15% and 25%.[ncbi.nlm.nih.gov]
  • Radiographic Stages of Pulmonary Sarcoidosis: STAGE TAB DESCRIPTION 0 TAB Normal Chest Radiograph I TAB Bilateral Hilar Lymphadenopathy II TAB Pulmonary Infiltration and Bilateral Hilar Lymphadenopathy III TAB Pulmonary Infiltration alone Steroid-requiring[clinicaltrials.gov]
Nephrolithiasis
  • Increased serum calcium with no other cause Increased urine calcium Nephrolithiasis, no stone analysis Nephrolithiasis analysis showing calcium Nephrolithiasis with negative family history for stones Neurologic Positive MRI with uptake in meninges or[doi.org]
  • Can cause nephrolithiasis, neuropsychiatric disturbance, abdominal pain and bone pain.[patient.info]
  • Kidney stones (nephrolithiasis) may result from the abnormal calcium metabolism Sarcoidosis How is sarcoidosis diagnosed? There is no single or specific diagnostic test for sarcoidosis.[dermnetnz.org]
  • The renal disease is commonly related to elevated calcium levels with resultant nephrolithiasis and nephrocalcinosis.[doi.org]
  • Abnormal calcium metabolism in sarcoidosis can lead to pancreatitis, nephrocalcinosis, nephrolithiasis, impaired renal function, renal failure and death, 11 highlighting the importance of diagnosing hypercalcaemia and hypercalciuria in these patients[dx.doi.org]
Bilateral Hilar Adenopathy
  • Twenty-two patients admitted over a 2-year period to our department with bilateral hilar adenopathy and a variety of symptoms compatible with sarcoidosis were studied prospectively.[ncbi.nlm.nih.gov]
  • Whole-body gallium scans can be used to show useful sites for biopsy and, in some cases, to follow disease progression Serial pulmonary function tests are important for assessing disease progression and guiding treatment Bilateral hilar adenopathy in[smartypance.com]
  • Chest radiograph findings in sarcoidosis are classically divided into 5 stages: Stage 0: no adenopathy or infiltrates Stage I: bilateral hilar adenopathy alone Stage II: bilateral hilar adenopathy and diffuse reticulonodular opacities Stage III: reticulonodular[clinicaladvisor.com]
  • Stage II - bilateral hilar adenopathy with pulmonary infiltrates (parenchymal involvement or reticular opacities). Stage III - parenchymal infiltrates without hilar adenopathy.[patient.info]
  • Bilateral hilar adenopathy Isolated reduced D l CO c. Diffuse infiltrates d.[doi.org]
Pericardial Effusion
  • AV block formation left ventricular insufficiency, and pericardial effusion has been reported. Bones Sarcoidosis may attack the bones as well causing, among others, cystic changes in the phalanges of fingers.[lecturio.com]
  • effusion ( 43 ).[doi.org]
Bilateral Pulmonary Infiltrate
  • pulmonary infiltrates fibrocystic sarcoidosis typically with upward hilar retraction, cystic and bullous changes Although people with stage 1 radiographs tend to have the acute or subacute, reversible form of the disease, those with stages 2 and 3 often[en.wikipedia.org]
Granulomatous Tissue
  • A search for mycobacterial DNA in granulomatous tissues from patients with sarcoidosis using the polymerase chain reaction. Am Rev Respir Dis 1992 ; 145 : 1142 –1148. Gerdes J, Richter E, Rusch-Gerdes S, et al.[doi.org]
  • tissue from patients with sarcoidosis. 6 The important targets of 1,25(OH) 2 -D 3 are the intestinal epithelium and bone, where the hormone acts to increase intestinal calcium and phosphate absorption, increase osteoclastic recruitment and bone resorption[dx.doi.org]
Pleural Effusion
  • To the best of our knowledge, this is the first described case of sarcoidosis presenting as large transudative pleural effusion.[ncbi.nlm.nih.gov]
  • KEYWORDS: endobronchial nodules; miliary opacities; pancreatic sarcoidosis; pleural effusion; sarcoidosis[ncbi.nlm.nih.gov]
  • Note that CXR may also demonstrate pleural involvement, such as a pneumothorax or pleural effusion. Stage 0 - normal findings on chest radiograph. Stage I - bilateral hilar lymphadenopathy (which may be accompanied by paratracheal adenopathy).[patient.info]

Treatment

Most patients only require symptomatic treatment with non-steroidal anti-inflammatory drugs (NSAIDs). For extrapulmonary sarcoidosis, corticosteroid therapy is often indicated, although steroid-sparing agents (e.g. methotrexate, azathioprine) are often used as alternatives [3] [4] [5] [6].

Prognosis

When sarcoidosis is detected early, the prognosis can be very excellent. However, there are many cases of misdiagnosis because of the nature of its clinical manifestations which is very general. Although the disease can disappear over time, there are cases where it becomes chronic or remissions and relapse may occur. According to studies, half of sarcoidosis cases can be cured within 12 to 36 months while the other half remains chronic or can come and go for years.

Etiology

The exact etiology of this disease is unknown. However, many physicians suggest that it is actually caused by a mix of environmental factors and genetics. There is also a study suggesting that it is caused by an alteration in the immune system’s normal response to external pathogens. However, what initiates the abnormal response is not known. There are a lot of probable causes mentioned but all these are just speculations that need further investigation.

Although the cause of sarcoidosis is unknown there are several risk factors which can trigger the disease. Age and gender are major considerations. The disease is most common in women and the targeted age group is mostly between the ages of 20 and 40. With regards to race, African Americans are more prone of having this disease. It can also be severe to this specific race than that of others. People who have family members that have sarcoidosis are likely to acquire the disease as well.

Epidemiology

The cases of sarcoidosis are widely distributed all over the world. It can happen to anyone regardless of race or ethnicity. People under the age of 50 are more vulnerable with developing the disease; specifically its peak is highly noticeable at the age between 20 to 39 years old. The occurrence of sarcoidosis differs from one place or country to another. Environmental exposures, genetic considerations and predispositions are just a few examples of factors that affect the occurrence of the disease. According to research, the highest number of cases recorded each year came from Northern European countries with an average of 5 to 40 people having sarcoidosis in a sample of 100,000. In Asia, Japan has had 1 to 2 cases with the same sample population.

In the United States, black Americans have the tendency to develop sarcoidosis more than white Americans. Doctors observe that the disease occurs two to three times more in black Americans. Aside from this, the disease is more severe in this group of people; it may even lead to death. Based on gender, sarcoidosis is more prevalent in females. This data is consistent among all races. While there are many articles saying that sarcoidosis is influenced by socioeconomic status, this is actually a myth. The risk factors for this disease are the same in both the rich and the poor.

Sex distribution
Age distribution

Pathophysiology

The development of sarcoidosis starts when an outside pathogen is inhaled. With a foreign body inside the lungs this will then trigger the initiation of the inflammatory process. Immune mediating cells such as giant cells, compose the core, surrounded by an outer rim of T lymphocytes will start migrating to the area in order to help in the normal healing process.

Non-necrotizing granulomas will start to develop and a lesion is formed inside the lungs – this is now called a sarcoid inflammation. A granuloma is a solid formed cell that blocks external pathogens. The core of the latter is composed of epithelioid histiocytes and multinucleated giant cells while the external wall consist of T lymphocytes. T cells play a major role in the occurrence of sarcoidosis because they elicit an exaggerated immune response.

Lymphocytes move tightly together during electrophoresis, they also have limited T-cell receptors and a steady antigen-driven process. The inflammatory process of sarcoidosis is reliant on a consistent initiation of CD4+ T cells. A difference in genetic make-up and T cell receptors which regulates the affinity of antigens is the reason for the development of the disease.
Several studies have been conducted to determine the specific cause of sarcoidosis.

It is still unclear whether it is caused by a single factor or by an alteration in the normal response of the immune system. At present, only identifiable risk factors are known.

Classification

The classification for sarcoidosis is identified according to the results of a chest X-ray as well as the body part or organ affected. The following are classified stages according to chest X-ray results.

  • Stage 0 : normal chest radiograph
  • Stage I : hilar or mediastinal nodal enlargement only
  • Stage II : nodal enlargement and parenchymal disease
  • Stage III : parenchymal disease only
  • Stage IV : end-stage lung (pulmonary fibrosis)

As mentioned, Sarcoidosis can also be classified according to the affected organ. These are just some of the most common ones.

  • Erythrodermic sarcoidosis
  • Subcutaneous sarcoidosis
  • Mucosal sarcoidosis
  • Neurosarcoidosis
  • Annular sarcoidosis
  • Mucosal sarcoidosis

Prevention

There are no guidelines for prevention of sarcoidosis.

Summary

Sarcoidosis is one of the many diseases that are idiopathic in nature. Considered an inflammatory disease, sarcoidosis is characterized by the presence of granulomas which are mainly composed of accumulated inflammatory cells such as T-lymphocytes. Sarcoidosis can occur at any part and organ in the body. However, it is most common in the lungs and the lymph nodes. This disease may cause a disruption in the physiological function of the organ or affect its overall structure.

An exact cure for the treatment of sarcoidosis has yet to be discovered. Although there is no cure at present, patients of the disease respond well with adjunct therapies.

The occurrence of sarcoidosis is quite unpredictable; it can occur at any time without any trigger and disappear on its own. Its symptoms may also become dormant and resurface after several years or the person can carry it throughout his lifetime. While the prognosis for sarcoidosis is generally good, there are severe cases of the disease where it can lead to organ damage and eventually failure.

Though uncommon, sarcoidosis is not a new disease. It was discovered over 100 years ago by two well-known dermatologists, Dr. Jonathan Hutchinson from England and Dr. Caesar Boeck from Norway. Before the term Sarcoidosis was coined the disease was called Hutchinson's disease or Boeck's disease. It was later changed to sarcoidosis which means flesh-like. The latter is a term used to describe breaks in the skin which are highly evident in people with sarcoidosis.

Patient Information

Once you are diagnosed with sarcoidosis, you have to immediately consult a specialist for your condition. Scheduled check-ups, follow-ups and daily monitoring must be done to observe the progression of the disease. It is also best to religiously undergo laboratory studies as to monitor whether you are getting well or not.

Following treatment regimens and listing unusual symptoms will also help in the developing a care plan. It is also important to remember that once you have sarcoidosis you have to get an annual shot of influenza immunization. Since sarcoidosis is a lung disorder, if smoking is a habit you have to find ways to slowly stop it. Consult an expert and seek for advice, immediately stopping might cause withdrawal symptoms which may be very harmful.

References

Article

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  2. Miyoshi S, Hamada H, Kadowaki T, et al. Comparative evaluation of serum markers in pulmonary sarcoidosis. Chest. Jun 2010;137(6):1391-7.
  3. Baughman RP, Judson MA, Teirstein AS, Moller DR, Lower EE. Thalidomide for chronic sarcoidosis. Chest. Jul 2002;122(1):227-32.
  4. Fazzi P, Manni E, Cristofani R, et al. Thalidomide for improving cutaneous and pulmonary sarcoidosis in patients resistant or with contraindications to corticosteroids. Biomed Pharmacother. Jun 2012;66(4):300-7.
  5. McKinzie BP, Bullington WM, Mazur JE, Judson MA. Efficacy of short-course, low-dose corticosteroid therapy for acute pulmonary sarcoidosis exacerbations. Am J Med Sci. Jan 2010;339(1):1-4.

  6. Pietinalho A, Tukiainen P, Haahtela T, Persson T, Selroos O. Early treatment of stage II sarcoidosis improves 5-year pulmonary function. Chest. Jan 2002;121(1):24-31.

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Last updated: 2017-08-22 15:19