Sarcoidosis history and symptoms
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Roshan Dinparasti Saleh M.D.
Overview
Sarcoidosis is a multisystem granulomatous disorder of unknown etiology that can involve all organ systems to varying extent and degree.
History and Symptoms
Pulmonary Sarcoidosis
- Sarcoidosis commonly involves patients between 20 and 60 years of age[1]. Lung is the most common organ involved by sarcoidosis, but up to 30% of patients present with extrapulmonary sarcoidosis[2][3]. Pulmonary sarcoidosis commonly presents with cough, dyspnea, chest pain, and infrequently fatigue, malaise, fever, and weight loss[4].
- Young children can present with skin rash, arthritis, uveitis, and erythema nodosum, without a predominant lung involvement[5][6]
Cutaneous Sarcoidosis
In 25% of the patients diagnosed with sarcoidosis, cutaneous involvement is seen[7]. The most common patterns of involvement is: papular, nodular, plaque-like, lupus pernio, erythema nodosum, subcutaneous sarcoidosis. See cutaneous manifestations of sarcoidosis in physical exam for more detail.
Ocular Sarcoidosis
In 25% of the patients diagnosed with sarcoidosis, ocular involvement is seen[8]. Sarcoidosis involves the orbit, anterior segment, posterior segment, conjunctiva, lacrimal glands, and extraocular muscles. the usual symptoms include dry eye, blurred vision, photophobia, red eye, and pain[9][10] .
- Intraocular sarcoidosis: these include anterior, intermediate, and posterior uveitis. Typical symptoms of anterior uveitis are pain and redness perticularly at the junction between cornea and sclera (limbus), while posterior and/or intermediate uveitis causes no symptom or floaters[9].
- extraocular orbital sarcoidosis: involvement of lacrimal glands, conjunctiva, extraocular muscles, optic nerve and palpable mass[11].
Upper Respiratory Tract Disease
Sarcoidosis can involve the larynx, pharynx, nares, and sinuses. The symptoms can include cough, dysphagia, hoarsness, nasal obstruction, nasal crusting, anosmia, epistaxis, and nasal polyposis[12][13].
Cardiac Sarcoidosis
The exact rate of myocardial involvement in sarcoidosis is not clear, but it ranges from 5% in patients with systemic sarcoidosis to 25 and even 70% in autopsy studies. Patients may complain of palpitations, syncope, lightheadedness, and chest pain. sudden cardiac death (SCD) can occur[14][15].
Neurosarcoidosis
Sarcoidosis can involve both Central and Peripheral nervous system in 5% of patients. The common symptoms include:
- Peripheral facial nerve palsy in 25 to 50% of patients with neurosarcoidosis[16][17]
- Polyuria and impairment in sleep, thirst, body temprature and libido due to hypothalamic inflammation[17][18]
- Seizures[19]
Reticuloendothelial System
Involvement of the lymph nodes, liver, and spleen is common in sarcoidosis. The patients may present with neck or axillary mass. liver and spleen involvement is usually asymptomatic[20].
Musculocutaneous
There are 3 common patterns of musculocutaneous involvement in sarcoidosis: 1. Acute sarcoid arthritis or acute polyarthritis 2. Chronic arthritis 3. Myopathy(diffuse granulomatous myositis)[21].
Lofgren Syndrome
The presence of erythema nodosum, hilar lymphadenopathy, migratory polyarthralgia, and fever has a 95% diagnostic specifity for sarcoidosis, allowing a diagnosis to be made without biopsy, and is associated with a good prognosis[22].
Exocrine Glands
In 5% of the patients diagnosed with sarcoidosis, painless swelling of salivary glands and parotid gland occurs. Sicca syndrome and xerostomia may also be seen[23].
Renal & Electrolyte
- Symptoms caused by hypercalcemia (presenting feature in 10 to 20 percent of patients)
- Renal colic caused by nephrolithiasis (presenting feature 4 percent of patients)
- Symptoms caused by uremia due to interstitial nephritis
- Symptoms caused by proteinuria (various types of glumerulonephritis may be seen)[24][25].
References
- ↑ Judson MA, Boan AD, Lackland DT: The clinical course of sarcoidosis: presentation, diagnosis, and treatment in a large white and black cohort in the United States. Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG 2012, 29(2):119-127.
- ↑ Ungprasert P, Carmona EM, Utz JP, Ryu JH, Crowson CS, Matteson EL: Epidemiology of Sarcoidosis 1946-2013: A Population-Based Study. Mayo Clinic proceedings 2016, 91(2):183-188.
- ↑ Baughman RP, Teirstein AS, Judson MA, Rossman MD, Yeager H, Jr., Bresnitz EA, DePalo L, Hunninghake G, Iannuzzi MC, Johns CJ et al: Clinical characteristics of patients in a case control study of sarcoidosis. American journal of respiratory and critical care medicine 2001, 164(10 Pt 1):1885-1889.
- ↑ Sharma OP: Fatigue and sarcoidosis. The European respiratory journal 1999, 13(4):713-714.
- ↑ Pattishall EN, Kendig EL, Jr.: Sarcoidosis in children. Pediatric pulmonology 1996, 22(3):195-203.
- ↑ Milman N, Hoffmann AL: Childhood sarcoidosis: long-term follow-up. The European respiratory journal 2008, 31(3):592-598.
- ↑ Roberts SD, Mirowski GW, Wilkes D, Kwo PY, Knox KS: Sarcoidosis. Part II: extrapulmonary and systemic manifestations. Journal of the American Academy of Dermatology 2004, 51(4):628-630.
- ↑ Jamilloux Y, Kodjikian L, Broussolle C, Seve P: Sarcoidosis and uveitis. Autoimmunity reviews 2014, 13(8):840-849.
- ↑ 9.0 9.1 Evans M, Sharma O, LaBree L, Smith RE, Rao NA: Differences in clinical findings between Caucasians and African Americans with biopsy-proven sarcoidosis. Ophthalmology 2007, 114(2):325-333
- ↑ Birnbaum AD, French DD, Mirsaeidi M, Wehrli S: Sarcoidosis in the national veteran population: association of ocular inflammation and mortality. Ophthalmology 2015, 122(5):934-938.
- ↑ Mavrikakis I, Rootman J: Diverse clinical presentations of orbital sarcoid. American journal of ophthalmology 2007, 144(5):769-775.
- ↑ Baughman RP, Lower EE, Tami T: Upper airway. 4: Sarcoidosis of the upper respiratory tract (SURT). Thorax 2010, 65(2):181-186.
- ↑ Reed J, deShazo RD, Houle TT, Stringer S, Wright L, Moak JS, 3rd: Clinical features of sarcoid rhinosinusitis. The American journal of medicine 2010, 123(9):856-862.
- ↑ Kron J, Ellenbogen KA: Cardiac sarcoidosis: contemporary review. Journal of cardiovascular electrophysiology 2015, 26(1):104-109.
- ↑ Darlington P, Gabrielsen A, Sorensson P, Cederlund K, Eklund A, Grunewald J: Cardiac involvement in Caucasian patients with pulmonary sarcoidosis. Respiratory research 2014, 15:15
- ↑ Stern BJ, Krumholz A, Johns C, Scott P, Nissim J: Sarcoidosis and its neurological manifestations. Archives of neurology 1985, 42(9):909-917.
- ↑ 17.0 17.1 Joseph FG, Scolding NJ: Neurosarcoidosis: a study of 30 new cases. Journal of neurology, neurosurgery, and psychiatry 2009, 80(3):297-304.
- ↑ Bihan H, Christozova V, Dumas JL, Jomaa R, Valeyre D, Tazi A, Reach G, Krivitzky A, Cohen R: Sarcoidosis: clinical, hormonal, and magnetic resonance imaging (MRI) manifestations of hypothalamic-pituitary disease in 9 patients and review of the literature. Medicine 2007, 86(5):259-268.
- ↑ Pawate S, Moses H, Sriram S: Presentations and outcomes of neurosarcoidosis: a study of 54 cases. QJM : monthly journal of the Association of Physicians 2009, 102(7):449-460.
- ↑ Warshauer DM, Lee JK: Imaging manifestations of abdominal sarcoidosis. AJR American journal of roentgenology 2004, 182(1):15-28.
- ↑ Kellner H, Spathling S, Herzer P: Ultrasound findings in Lofgren's syndrome: is ankle swelling caused by arthritis, tenosynovitis or periarthritis? The Journal of rheumatology 1992, 19(1):38-41.
- ↑ O'Regan A, Berman JS: Sarcoidosis. Annals of internal medicine 2012, 156(9):ITC5-1, ITC5-2, ITC5-3, ITC5-4, ITC5-5, ITC5-6, ITC5-7, ITC5-8, ITC5-9, ITC5-10, ITC15-11, ITC15-12, ITC15-13, ITC15-14, ITC15-15; quiz ITC15-16.
- ↑ James DG, Sharma OP: Parotid gland sarcoidosis. Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG 2000, 17(1):27-32.
- ↑ Rizzato G, Colombo P: Nephrolithiasis as a presenting feature of chronic sarcoidosis: a prospective study. Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG 1996, 13(2):167-172.
- ↑ presentation of sarcoidosis: a frequent occurrence but a challenge to an early diagnosis. A 3-year-long prospective observational study. Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG 2004, 21(2):119-126