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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]
  • […] 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]
Cervical Lymphadenopathy
  • lymphadenopathy in others Muscle 50–80% Asymptomatic disease with or without enzyme elevations in most patients Sometimes insidious or acute myopathy with muscle weakness Hepatic 40–75% Usually asymptomatic Manifests with mild elevations in liver function[merckmanuals.com]
Cough
  • A 78-year-old woman was referred to our hospital for coughing symptoms. A chest computed tomography (CT) scan revealed bilateral diffuse miliary patterns and right pleural effusion.[ncbi.nlm.nih.gov]
  • We here describe a case of a 35-year-old patient who presented with a history of exertion dyspnoea and coughing for the past 20 years. At the age of 15, she was exposed to smoke emanating from a fire.[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]
  • […] of lung disease, with a personal history of ulcerative colitis (UC), under clinical remission following infliximab therapy in maintenance dose, who was admitted for treatment administration, but also for dyspnea, nocturnal sweating, and nonproductive cough[ncbi.nlm.nih.gov]
  • 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.[orpha.net]
Dyspnea
  • 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]
  • In contrast, SF-36 scores associated with fatigue and dyspnea scores (r 0.72; p 0.001 and r 0.85; p 0.001). These findings imply that sarcoidosis patients are less active compared with healthy subjects.[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]
  • A 60-year-old woman with biopsy-proven cardiac sarcoidosis status post implantable cardioverter defibrillator placement presented with periodic dizziness and dyspnea on exertion.[ncbi.nlm.nih.gov]
  • On a multivariate analysis, there was no association between successful diagnostic yield and the presence of dyspnea, spirometric lung function, radiologic stage of sarcoidosis, presence of floating biopsies, presence of at least 50 alveoli, or crush[ncbi.nlm.nih.gov]
Dry Cough
  • 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.[orpha.net]
  • 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]
  • 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]
  • Like lung cancer, pulmonary sarcoidosis can be completely symptomless, or may include a dry cough, shortness of breath, or mild chest pain; sometimes, these are accompanied by fatigue, weakness, and weight loss.[medstarfranklinsquare.org]
  • The most common symptoms of pulmonary sarcoidosis are shortness of breath, which often gets worse with activity; dry cough that will not go away; chest pain; and wheezing.[cedars-sinai.edu]
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]
  • 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]
  • 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]
  • 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]
  • A chest computed tomography (CT) scan revealed bilateral diffuse miliary patterns and right pleural effusion. Bronchoscopy showed multiple nodules in the carina and the bronchus intermedius.[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]
Fatigue
  • 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]
  • Fatigue Assessment Scale (FAS) was used to define significant fatigue ( 22 points). Polish version of SHQ was prepared by the authors and validated.[ncbi.nlm.nih.gov]
  • The FAS and SF-36 scales seem to be effective tools for assessing the severity of fatigue in sarcoidosis patients.[ncbi.nlm.nih.gov]
  • For the secondary outcomes, significant improvement (p 0.001) was seen for all measured parameters, e.g., dyspnoea (modified Medical Research Council Scale, mMRC), fatigue (Fatigue Assessment Scale [FAS]; SRM -0.71), anxiety and depression (Hospital Anxiety[ncbi.nlm.nih.gov]
Fever
  • A 53-year-old woman from Southern India presented with weight loss, anorexia, fever and asthenia.[ncbi.nlm.nih.gov]
  • Sarcoidosis Patient will present as a 30-year-old African American female with a cough, fever, and generalized body aches.[smartypance.com]
  • Other symptoms include anterior uveitis, fever, ankle periarthritis, arthralgias, and pulmonary involvement.[emedicine.medscape.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.[orpha.net]
  • […] of sarcoidosis: Wheezing, coughing or chest pain Rashes or small bumps on the skin Dry eyes or watering eyes An enlarged liver Many people notice this specific set of signs and symptoms (known as Lofgren’s syndrome) when they first get sarcoidosis: Fever[rush.edu]
Weight Loss
  • 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]
  • A 53-year-old woman from Southern India presented with weight loss, anorexia, fever and asthenia.[ncbi.nlm.nih.gov]
  • 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.[orpha.net]
  • There also can be fatigue, weakness and weight loss.[ccohs.ca]
  • 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]
Weakness
  • 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]
  • 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]
  • There also can be fatigue, weakness and weight loss.[ccohs.ca]
  • Like lung cancer, pulmonary sarcoidosis can be completely symptomless, or may include a dry cough, shortness of breath, or mild chest pain; sometimes, these are accompanied by fatigue, weakness, and weight loss.[medstarfranklinsquare.org]
  • Symptoms may include: Cough Shortness of breath Wheezing Chest pain Rash Fever Pain or irritation of eyes Fatigue, especially with exertion Muscle weakness Night sweats Loss of appetite Weight loss Swollen lymph nodes Seizures Tremors Difficulty hearing[uvahealth.com]
Malaise
  • 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, 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]
  • Almost 33% of patients present with non-specific symptoms which may include fever, weight loss, weakness, and/or malaise. Organ involvement is one of the most important determiners for presentation.[physio-pedia.com]
Dysphagia
  • 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]
Parotid Swelling
  • swelling (most common) Parotitis with xerostomia Heerfordt syndrome (uveoparotid fever), characterized by uveitis, bilateral parotid swelling, facial palsy, and chronic fever Oral lupus pernio, which may disfigure the hard palate and may involve the[merckmanuals.com]
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]
  • 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]
  • pain Skin symptoms Some people who have sarcoidosis develop skin problems, which may include: A rash of red or reddish-purple bumps, usually located on the shins or ankles, which may be warm and tender to the touch Disfiguring sores (lesions) on the[mayoclinic.org]
  • Like lung cancer, pulmonary sarcoidosis can be completely symptomless, or may include a dry cough, shortness of breath, or mild chest pain; sometimes, these are accompanied by fatigue, weakness, and weight loss.[medstarfranklinsquare.org]
  • These include shortness of breath, ankle swelling, irregular heart beat and chest pain.[dermnetnz.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]
  • 61] In males, sarcoidosis may lead to infertility. [62] 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
  • […] 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]
  • eyes Sensitivity to light Blurred vision Fever RISK FACTORS Sarcoidosis can affect people of any age, race, and gender.[chestnet.org]
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
  • Analysis by specific type of chronic condition revealed a significantly higher cumulative incidence of coronary artery disease, congestive heart failure, arrhythmia, stroke or transient ischemic attack, arthritis, depression, diabetes, and major osteoporotic[ncbi.nlm.nih.gov]
  • […] tissues) or arthralgia (painful joints) may occur Arthritis is most commonly acute (coming on suddenly), resulting in swelling of the lower legs and tenderness of ankles, knees and fingers Chronic (long term) sarcoid arthritis is rare Calcium metabolism[dermnetnz.org]
  • It may also lead to arthritis, fever, joint pain, and fatigue. Although sarcoidosis often goes away on its own, it sometimes requires extensive treatment. NYU Langone doctors manage the condition, helping to prevent any long-term complications.[nyulangone.org]
  • Löfgren's syndrome refers to the combination of erythema nodosum with arthritis (commonly affecting the ankles) and bilateral hilar or paratracheal adenopathy seen on CXR.[patient.info]
  • […] tender reddish bumps, rash or sores eyes – blurred vision, pain, severe redness or sensitivity to light heart – abnormal heartbeat brain – headaches or facial paralysis kidney – increased thirst or formation of kidney stones joints – swelling and pain ( arthritis[healthdirect.gov.au]
Arthralgia
  • Other symptoms include anterior uveitis, fever, ankle periarthritis, arthralgias, and pulmonary involvement.[emedicine.medscape.com]
  • 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]
  • Fever, weight loss, arthralgias, and erythema nodosum (more commonly seen in Europeans) are the usual initial presenting symptoms. Cough and dyspnea may be minimal or absent.[smartypance.com]
  • […] 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: Fever Joint ache or pain (arthralgia[ufhealth.org]
  • 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.[symptoma.com]
Night Sweats
  • 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.[orpha.net]
  • 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]
  • Symptoms may include: Cough Shortness of breath Wheezing Chest pain Rash Fever Pain or irritation of eyes Fatigue, especially with exertion Muscle weakness Night sweats Loss of appetite Weight loss Swollen lymph nodes Seizures Tremors Difficulty hearing[uvahealth.com]
  • However, your overactive immune system may lead to problems with different organs of the body and lead to cough, shortness of breath, night sweats, joint pain and fatigue. One Appointment. One Team.[vcuhealth.org]
  • Non-specific symptoms: Fever Weight loss Night sweats Fatigue Symptoms related to the lung: Shortness of breath Wheezing Chest pain Recurring chest colds Less exercise tolerance Symptoms related to lymph nodes: Enlarged lymph nodes in the neck, under[health.ucsd.edu]
Skin Lesion
  • Accordingly, a 6-month course of multidrug therapy led to a marked improvement in the skin lesions.[ncbi.nlm.nih.gov]
  • Recurrent facial palsy in a patient merits investigation for underlying etiology. 8-year-old boy with erythematous itchy skin lesion and recurrent facial palsy. 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]
  • 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]
  • Skin lesions and bone cysts are characteristically present in the chronic form of the disease. Sarcoidosis may cause no symptoms, or an attack may begin with the appearance of tender red nodules on the front of the legs and with joint pain.[britannica.com]
Alopecia
  • 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]
  • "Sarcoidosis-induced alopecia" . Dermatology Online Journal . 18 (8): 4. PMID 22948054 . Archived from the original on 2 March 2014. Birnie, David (July 2014).[en.wikipedia.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]
  • 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
  • It can cause problems with the: lungs – cough , chest pain or feeling short of breath skin – tender reddish bumps, rash or sores eyes – blurred vision, pain, severe redness or sensitivity to light heart – abnormal heartbeat brain – headaches or facial[healthdirect.gov.au]
  • Symptoms related to the eyes: Burning Itchiness Redness Pain Dryness Sensitivity to light Seeing black spots (floaters) Blurry vision Symptoms related to the heart: Shortness of breath Palpitations Leg swelling Symptoms related to the nervous system: Headache[health.ucsd.edu]
  • Headache, seizure, ataxia, cognitive impairment, etc.[casa.gov.au]
  • Lung symptoms can include: a dry cough shortness of breath wheezing chest pain around your breastbone Skin symptoms can include: skin rashes skin sores hair loss raised scars Nervous system symptoms can include: seizures hearing loss headaches Eye symptoms[healthline.com]
  • […] include: Fever Joint ache or pain (arthralgia) Skin symptoms may include: 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[ufhealth.org]
Peripheral Neuropathy
  • Nerves/central nervous system (CNS) Check the function of the VIIth and other cranial nerves and peripheral sensory/motor nerve function to detect peripheral neuropathy. Formal neurological examination is appropriate if there are relevant symptoms.[patient.info]
  • Other complications of sarcoidosis include: High blood calcium levels Liver disease Peripheral neuropathy, a change in the sensitivity of the peripheral nerves (usually those in your hands and feet) Meningitis, an inflammation of the tissues surrounding[chestnet.org]
  • neuropathy (common) May affect any cranial nerve CNS involvement, with nodular lesions or diffuse meningeal inflammation typically in the cerebellum and brain stem Hypothalamic diabetes insipidus, polyphagia and obesity, and thermoregulatory and libidinal[merckmanuals.com]
  • "Nerve granulomas and vasculitis in sarcoid peripheral neuropathy". Brain . 125 (Pt 2): 264–75. doi : 10.1093/brain/awf027 . PMID 11844727 . Tavee J, Culver D; Culver (2011). "Sarcoidosis and small-fiber neuropathy".[en.wikipedia.org]
Cranial Nerve Involvement
  • 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
  • […] 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]
  • 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]
  • PPD skin testing should be done early in the assessment along with anergy controls.[merckmanuals.com]
  • Originally, scientists thought that sarcoidosis was caused by an acquired state of immunological inertness (anergy).[medicinenet.com]
Pulmonary Infiltrate
  • Chest radiography is abnormal in about 90% of cases and shows lymphadenopathy and/or pulmonary infiltrates (without or with fibrosis), defining sarcoidosis stages from I to IV.[orpha.net]
  • The main tools your doctor will use to diagnose sarcoidosis include: Chest X-rays to look for cloudiness (pulmonary infiltrates) or swollen lymph nodes ( lymphadenopathy ).[webmd.com]
  • In the absence of symptoms, deterioration of lung function such as a reduction of total lung capacity, forced vital capacity, or diffusing capacity, or radiographic progression such as new pulmonary infiltrates or reticular fibrotic changes, are indications[clinicaladvisor.com]
  • infiltrates) Stage III Pulmonary involvement without enlarged lymph nodes Stage IV Advanced, irreversible pulmonary fibrosis Bihilary lymphadenopathy In cases of unclear or not quite specific radiological findings but with typical clinical signs present[lecturio.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
  • 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 is the most common abnormality. If sarcoidosis is suspected, a chest x-ray should be the first test if it has not already been done.[merckmanuals.com]
Nephrolithiasis
  • 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]
  • Nephrolithiasis and nephrocalcinosis may occur, sometimes leading to chronic kidney disease .[merckmanuals.com]
Pericardial Effusion
  • AV block formation, left ventricular insufficiency, and pericardial effusion have been reported. Bones Sarcoidosis may attack the bones as well causing, among others, cystic changes in the phalanges of fingers.[lecturio.com]
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]
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]
  • A chest computed tomography (CT) scan revealed bilateral diffuse miliary patterns and right pleural effusion. Bronchoscopy showed multiple nodules in the carina and the bronchus intermedius.[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

  1. Davies CW, Tasker AD, Padley SP, Davies RJ, Gleeson FV. Air trapping in sarcoidosis on computed tomography: correlation with lung function. Clin Radiol. Mar 2000;55(3):217-21.
  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