#Lofgrens syndrome

[mcatmemoranda]

Reviewing questions:

Blindness is a potentially devastating complication of sarcoidosis. Ocular sarcoid can develop in up to half of all cases and most commonly presents as anterior uveitis or keratoconjunctivitis. The most serious complication is optic neuritis.

A complete ophthalmologic examination at the time of diagnosis is recommended, as asymptomatic disease can result in permanent visual impairment. All patients with sarcoidosis should have a careful history and physical examination to identify other organ involvement before having further testing, including ECG, pulmonary-function testing, and baseline laboratory studies (calcium level, alkaline-phosphatase level, renal-function, and hepatic-function tests) to evaluate the extent of disease.

McConnell's sign is defined as right ventricular free wall akinesis with sparing of the apex. Typically this looks as if the apex of the RV is a trampoline. Echocardiogram shows right ventricular strain and a positive McConnell sign, which is indicative of pulmonary embolism (PE).

All patients who have known or suspected bronchiectasis and who are acutely ill with suspected lower respiratory tract infection should be empirically treated with an antipseudomonal antibiotic, such as cefepime until culture results are available to guide treatment decisions.

Heerfordt’s syndrome (uveoparotid fever) is another collection of clinical findings specific enough to diagnose sarcoidosis without a biopsy. These signs and symptoms include uveitis, parotid gland enlargement, and facial nerve palsy.

Lofgren’s syndrome is a form of acute sarcoidosis. Sarcoidosis is a multisystem disease characterized by the presence of noncaseating granulomas and although classically described in African Americans, it has one of its highest prevalences in the northern European population. Clinical variants exist with certain forms having diagnostic and prognostic value. Lofgren’s syndrome is the combination of erythema nodosum, bilateral hilar adenopathy, migratory polyarthralgias, and fever. This pattern is so consistent that it has 95% diagnostic specificity and allows diagnosis of sarcoidosis without a biopsy. Among European patients, the presence of Lofgren’s syndrome portends to a good prognosis. For mild disease symptomatic treatment with non-steroidal anti-inflammatory agents is reasonable or low dose prednisone may be added.

The optimal management of laryngotracheitis (croup) is determined by the severity of disease. There are numerous scoring systems for croup severity, with the Westley croup score being the most validated and most often used. The Westley croup severity scale includes evaluation for the presence of stridor at rest, retractions, and cyanosis as well as assessment of mental status and air entrance. Regardless of any official scoring system, most physicians would consider the presence of retractions and stridor at rest to be indications of moderate to severe disease that requires evaluation in the emergency department. The presence of cyanosis, confusion, depressed mentation, agitation, severe retractions, or absent breath sounds would indicate severe disease and/or the possibility of impending respiratory failure.

Mild cases of croup are treated on an outpatient basis with cool mist therapy and fluid replacement. Moderate cases may require supplemental oxygen, oral or intramuscular corticosteroids, or racemic epinephrine. Severe cases are best treated with hospitalization and racemic epinephrine.

Bottom Line: The most important step in initial management of laryngotracheitis includes nebulized epinephrine (racemic or L-epinephrine) and dexamethasone along with humidified oxygen, fever reduction, and hydration. Epinephrine acts almost immediately, while steroids have a delayed onset of action.

Pneumothorax is defined as a collection of air in the pleural space. The result is pleural separation of the visceral and parietal pleura. Spontaneous pneumothorax lacks an antecedent event (e.g. trauma). There are two types: primary and secondary. Primary spontaneous pneumothorax (PSP) occurs in patients without recognizable underlying lung disease. Secondary spontaneous pneumothorax (SSP) occurs in patients with visible underlying lung disease (e.g. COPD). The most common etiology of PSP is rupture of a subpleural bleb (which are usually not seen on imaging and are undiagnosed). Many cases initially diagnosed as PSP may eventually be diagnosed as SSP after further workup.

Management depends on the size of the pneumothorax. The spectrum of management includes watchful waiting, aspiration, chest tube insertion, and thoracoscopy with pleurodesis or lung resection.

Rhinosinusitis can be defined as either viral/bacterial and if bacterial, as complicated/uncomplicated. Complicated rhinosinusitis implies the extension of disease outside the nasal cavity/sinuses into adjacent structures, such as soft tissues, ophthalmologic tissues, and nervous system.

Current criteria for the presumptive (many of these cases may still be viral) diagnosis of acute bacterial rhinosinusitis (ABRS) include persistent symptoms lasting more than 10 days without any evidence of clinical improvement or a biphasic illness pattern which is also called double worsening, meaning the patient was sick, improved, and then became sicker a second time within a short time frame, usually within a 10 day period. The IDSA guidelines from 2012 also recommend the use of a high fever greater than 39C associated with severe symptoms for 3-4 days as being more consistent with bacterial rhinosinusitis. According to the guidelines by the American Academy of Otolaryngology-Head and Neck Surgery published in 2015, fever itself early in the course of illness is not sensitive or specific enough to warrant treatment based on this vital sign alone, and they do not recommend using this as criteria for the diagnosis of ABRS. They agree with the other criteria used by the IDSA and acknowledge that a "severe" presentation may warrant the use of antibiotics as recommended by the IDSA and the American Academy of Pediatrics which considers more than 3 days of high fever and purulent nasal discharge as a severe presentation of ABRS in children.

Summary Criteria for the Diagnosis of ABRS

Signs or symptoms of acute rhinosinusitis present for 10 days or more after symptom onset with no improvement

Acute worsening of improving rhinosinusitis within 4-6 days of symptom onset or "double-sickening."

Severe rhinosinusitis: onset of high fever greater than 39C and severe symptoms within 3-4 days of onset

Tx of ABRS: amoxicillin, amoxicillin-clavulanate, doxycycline if PCN allergic, or respiratory fluoroquinolone in kids who can't take doxycycline. I just had a pt in clinic who had bacterial rhinosinusitis and I gave him amoxicillin-clavulanate.

Bottom Line: In patients with ABRS who require antibiotic therapy, the initial therapy of choice is with amoxicillin with or without clavulanate.

One of the feared complications of acute bacterial rhinosinusitis (ABRS) is orbital cellulitis. This infection commonly develops in the setting of ABRS due to the direct extension of bacteria from the sinus cavity into the orbit. Commonly it can project through the roof of the maxillary sinus. Additionally, there can be extension through the adjacent soft tissues. The diagnosis of this condition is clinical and is confirmed with radiographic imaging (CT scan of the sinuses). The presence of pain/difficulty with eye movement, double vision, eye swelling, and erythema should be concerning for orbital infection. The initial imaging test of choice would be with a contrasted CT study of the face and orbits. An MRI could also be performed and has similar accuracy, but is generally slower and more expensive to obtain. Additionally, patients with suspected orbital cellulitis should be evaluated by ophthalmology in the emergency department and started on IV antibiotics with coverage for MRSA (vancomycin generally).

If the sinuses are the suspected source, ampicillin-sulbactam or piperacillin-tazobactam can be added to vancomycin as long as there is no concern for CNS involvement. If the CNS is involved, it should be noted that both ampicillin-sulbactam and piperacillin-tazobactam have relatively poor CNS penetration, and a 3rd generation cephalosporin (ceftriaxone) should be used with the addition of metronidazole for coverage of anaerobic organisms. General guidelines for sepsis and infection should be followed with the physician obtaining the regular laboratories including a complete blood count, metabolic profile, blood cultures, and lactate.

Symptoms of Complicated ABRS:

Proptosis or impaired extraocular movements Painful eye movements Diplopia or impaired vision Periorbital edema or erythema Cranial nerve palsies Altered mental status Neck stiffness/meningeal signs Papilledema

Complications of ABRS:

Meningitis Orbital cellulitis Cavernous sinus thrombosis/thrombophlebitis Preseptal cellulitis

Ruptured eardrum:

Tympanic membrane perforations are commonly caused by trauma or acute otitis media and are usually noted on otoscopic examination. Perforations with marked hearing loss or other concerning neurologic signs such as nystagmus, ataxia, or vomiting should receive a prompt evaluation by an otolaryngologist. Supportive care only is a perfectly reasonable treatment plan for patients with simple perforations with minimal hearing loss and no neurologic signs. Many perforations will heal spontaneously within 4 weeks and require no intervention.

[doctorfoxtor]

YOU WANT A HOT BODY YOU WANT A BUGATTI YOU WANT A MASERATI YOU BETTER WORK BITCH

100 days of productivity

day 42 + 43

CVS/RS

COPD w/ serum eosinophilia = likely to respond to steroids! → grey area between COPD and asthma

other steroid responsive features: ≥20% diurnal variation in PEFR, ≥400 ml variation in FEV1 over time, previous h/o atopy/asthma

also, if need to add a long-acting inhaler and pt is taking SAMA, switch the SAMA to SABA first then add either LABA+LAMA (no steroid-responsive fx) or LABA+ICS (steroid-responsive fx)

place an ICD for parapneumonic effusion if fluid is frankly turbid, or if clear fluid stain/culture is positive, or if pH <7.2

Lofgren syndrome: acute sarcoidosis w/ b/l hilar nodes, fever, erythema nodosum and polyarthralgia

normal rhythm variants in athletes include sinus brady, first degree block, type 1 second degree and even junctional rhythm

Ivabradine Inhibits I-funny Ion channels to help angIna but also causes funny I (eye) problems

multiple rapidly resolving episodes of breathlessness/chest tightness after tx for ACS → did pt get ticagrelor? it can cause temporary accumulation of adenosine which is a bronchoconstrictor

CNS/Ophthal/Psych

AACG vs uveitis: AACG = mid-dilated pupil w/ hazy cornea w/ severe pain and haloes; ant uveitis = fixed miotic pupil w/ ciliary flush ± hypopyon

myasthenia-precipitating drugs: My Partner supports LGBTQ (Macrolides, Procainamide/Penicillamine*, Lithium, Gentamicin, Beta-blockers, Tetracyclines, Quinonlones/Quinidine) (*NOT penicillins!)

blepharitis assoc w/ meibomian gland dysfn, seborrhoeic dermatosis, S aureus, rosacea

Endocrine/Repro

SGLT2is can cause either hyperglycaemic or euglycaemic DKA by causing dehydration from osmotic diuresis; if euglycaemia is present, it is bc of glucose wasting in urine

Hashimoto's is specifically link to thyroid MALToma, characterised by marginal B-cells on histology

toxic multinodular goitre → radioiodine, NOT surgery

Pendred sd → borderline hypothyroidism (often euthyroid w/ small goitre) w/ progressive B/L SNHL

Rheum/Derm/Immuno

achondroplasia: homozygotes do not survive past few months of life, so adults with achondroplasia are obligate heterozygotes

systemic mastocytosis: urticaria pigmentosa (itchy nonbleeding truncal red-brown papules) with Darier sign (palpation of paps makes surrounding skin erythematous and itchy/rubbing produces wheals); urine histamine → skin biopsy if inconclusive

IL-1 mainly produced by macrophages

Raynaud is only seen in type 1 cryo and is associated with monoclonal globulinopathies (myeloma/Waldenström)

flexural/face psoriasis: 1st ONLY steroids 2nd vitamin D, tacro 3rd coal tar

drugs for hereditary angiooedema: exogenous C1-esterase inhibitor, icatibant (bradykinin antag), ecallantide (kallikrein inactivator), FFP, danazol (prophylactic)

GIT/Renal

dyspepsia causing drugs: acid producing/ulcerative - NSAIDs, steroids; GERD - bisphosphonates, Ca channel blockers, nitrates, methylxanthines

lactulose can worsen bloating ssx in IBS—to avoid in IBS

idiopathic membranous gnitis → anti-phospholipase A2 Abs

duloxetine can actually be used for stress incontinence if pelvic floor exercise don't work out

Onc/Haem

citrate is also used in stored plasma, so hypocalcaemia is just as much a complication in plasma exchange

factor V Leiden makes factor V resistant to protein C

lymphohistiocytic variant of RS cell is seen in lymphocyte predominant HD

ID

Lyme: ELISA is screening, immunoblot is confirmatory

Japanese B encephalitis: fever, AMS, seizures, and characteristically parkinsonism; serology; supportive care

Pharm/Toxo

only antiepileptics not safe in breastfeeding are barbiturates

IFN-alpha uses: hep B, hep C, Kaposi, metastatic RCC, hairy cell leukaemia

Genetics

Friedrich's ataxia: AR adolescent ataxiGAA, cerebellar/spinocerebellar tract dysfunction, optic atrophy, absent deep reflexes but extensor plantars, HOCM, diabetes, arched palate

Ataxia-telangiectasia: AR toddler ataxia, double strand break repair, cerebellar dysfunction, IgA deficiency, lymphomas/leukaemias, AV malformations (including telangiectasias)

if an AFAB person develops X-linked disorders, also rule out Turner syndrome

[thedigitalhuman]

Corrupted Blood started with a band of high-level players who took down an end-game boss with the ability to cast a blood-draining spell. While the players made sure they were free of the spell’s effect after the battle, they allegedly forgot to treat one of the pets. That pet was then able to carry the pathogen out of the dungeon as a result of a programming oversight, culminating in a full-blown pandemic.

Corrupted Blood quickly spread from city to city. Game developer Blizzard tried to quarantine some areas of the game in response, but to no avail. After a week, Blizzard only managed to put the kibosh on the mayhem with hard resets and patches.

Epidemiologists later found players exhibited some interesting behavior during this time. Some people volunteered to help, but wound up falling sick. Others “went to work” as usual (making money by dealing weapons) and infected others. And some got themselves infected out of curiosity.

“Some people tried to uphold law and order, some tried their best to help, others maliciously disseminated the virus,” one Weibo user wrote, recalling the Corrupted Blood incident. “In a game with anonymity, good and evil appear even more real than in real life.”

Epidemiologists have also compared the Corrupted Blood incident to China’s outbreak of Sars (severe acute respiratory syndrome). Ran Balicer, Eric Lofgren and Nina Fefferman wrote several papers on the event, citing similarities to the previous coronavirus outbreak that also originated in animals.

Balicer pointed out that both the virtual and real epidemic faced failed attempts to quarantine infected people, and both cases demonstrated a high potential for rapid spread around the world. While the disease in WoW was spread by characters teleporting from city to city, Sars found its way to other countries by way of air travel.

“This plague bug in a video game really carries a lot of real-life meaning!” a Weibo influencer wrote.

Digital Human, Series 19 Episode 3 - Lab Rats

[hs4lifeblog-blog]

Aspirations

If you had aspirations and goals for yourself even before the HS kicked in, keep having them. Keep those aspirations intact because it will give you the motivation you need to keep going. At least it is working for me. I have never considered myself as a quitter and I certainly don’t give up easily. This isn’t a terminal disease so don’t treat it as if it is. It is part of who you are and who i am, it doesn’t make you any different, if anything it give you uniqueness. I still believe I can be the business woman that I’ve always wanted to be but I have had to accept that it won’t happen now. I have always dreamt of living in Spain and continuing my life there. I absolutely love that country ever since I went the first time at 15 years old. I’ve always felt like I belonged there and live among their breath-taking views. I hold on to that possibility that some day it will happen and it will. 

If I have learned anything from having all these limiting conditions is that urge to go out and live more. You take the little things for granted and once you can’t do or have what you want it makes you want it even more. Now I want everything I used to want 10 times more. And not just that, even little things like riding a bike, which I never found it fun, but now I see a bike and I want to ride it all over Boston. 

Put there is one thing I want to focus on, and that is what HS gave me now: 

Patience: it takes time and it is essential to not let the frustration get in your head. The fact that you know that thing might get worse and there is no cure then patience comes into place. It is best to just breathe and not think the worst. 

Strength: I have heard so many people tell me how strong I am and at first I just thought that’s what I needed to be. I am a rational person and often a realist. If I wasn’t strong then it is basically giving in to the condition. I refuse to let it get to my head all the cons to this condition and add on to the depressive moods. Strength is necessary at this point. 

Hope: I am a strong believer that pain is only temporary. I can be going through this but I know that there will be good days. There is always a period where everything calms down and that is what I constantly hope for. You never know when those good days are coming, that’s why you need to keep hoping. 

These three thing that HS gave me are the things that are going to get me to my goals. Once I get through this rough part I will continue to strive towards my aspirations. Patience, strength and hope will get me to follow what I believe in and get what I ultimately want. Things like HS and Lofgren’s syndrome are bumps in the road, rough bumps but nothing one can’t get through. 

[nickyschneiderus]

Google CEO tells Congress why searching ‘idiot’ results in Trump images

Google CEO Sundar Pichai had to explain to Congress Tuesday why looking up the term “idiot” on his company’s search engine produced pictures of President Donald Trump.

Appearing before the House Judiciary Committee, Pichai was questioned by Rep. Zoe Lofgren (D-Calif.) on the issue in an attempt to refute allegations that the result was due to a political bias from Google.

“Right now, if you Google the word ‘idiot’ under images, a picture of Donald Trump comes up. I just did that,” Lofgren said. “How would that happen? How does search work so that would occur?”

Pichai replied by stating that such results are based on “things like relevance, freshness, popularity” and what internet users are associating with a particular term as opposed to a specific decision by Google.

“We provide search today for any time you type in a keyword,” Pichai said.”Wwe have gone out and stored copies of billions of pages in our index, and we take the keyword and match it against the pages and rank them based on over 200 signals.”

“So it’s not some little man sitting behind the curtain figuring out what we’re going to show the user?” Lofgren asked in response. “It’s basically a compilation of what users are generating.”

While Pichai did not confirm how the president came to be linked to the term “idiot,” a story last July published by the Guardian noted how anti-Trump activists were able to manipulate Google’s algorithm by “upvoting a post containing a photo of him and the word ‘idiot’ on Reddit.”

As noted by the Verge, a similar tactic was used in the mid-2000s so that searches for the term “miserable failure” returned images of then-President George W. Bush.

READ MORE:

What exactly is ‘Trump Derangement Syndrome?’

What is Spygate, Trump’s favorite new conspiracy theory?

The best Donald Trump memes on the internet

H/T the Verge

from Ricky Schneiderus Curation https://www.dailydot.com/debug/congress-google-idiot-trump/

[hcsmca]

Lofgren syndrome in acute sarcoidosis [Practice]

Read more from CMAJ

[mcatmemoranda]

Reviewing Questions:

The flow-volume loop for obstructive lung disease shows a "scooped-out" pattern because of smaller expiratory flow rate. The Y-axis is flow in Liters/second and the X-axis is lung volume in Liters. In COPD, the pt needs more time to exhale. So when the pt exhales rapidly, there's more air than normal still left in the lungs, which decreases the ridal volume. This is called "dynamic hyperinflation." Pts with COPD have increased residual volume because they can't exhale fully; increased residual volume also means increased total lung capacity.

Massive PE is when the pt has PE and hemodynamic instability (hypotension). This can lead to sudden cardiac death because the cardiac output decreases. The thrombus can also occlude the pulmonary arteries and the ischemia that causes can cause right ventricular strain, which can lead to arrhythmias.

Obstructive Sleep Apnea (OSA) can lead to hypetension, pulmonary hypertension, right heart failure, and cardiac arrhythmias. Pts may complain of morning headaches, non-refreshing sleep, falling asleep during the day.

A loud pulmonic component of S2 is heard when there's high pressure in the pulmonary artery because blood is backing up in pulmonary hypertension. Hypoxic vasoconstriction leads to increased resistance in the pulmonary vasculature, so blood will back up into the right side of the heart. Right ventricular hypertrophy can cause left parasternal lift. The pressure that the right ventricle has to pump against can increase until you end up with right heart failure, which causes JVD and edema of the legs.

When you give a lot of O2 to someone with COPD, you undo the hypoxic vasoconstriction that the pt normally has; this causes increased perfusion to less well-ventilated alveoli, which actually increases the dead space. So pts end up with oxygen-induced hypercapnia. I still don't really get this because I'm thinking that oxygen is being increased in all the alveoli when you give supplemental O2, so wouldn't the blood that goes there be oxygenated? I don't know, but I got the question right because I answered one like it before. I guess what happens is even though you're giving O2, you're still moving blood away from well-ventilated alveoli to less well-ventilated alveoli. Yes, the bottom line is that giving too much oxygen to COPD pts causes increased ventilation-perfusion mismatch. Moving blood away from better-ventilated alveoli to less well-ventilated alveoli increases physiologic dead space because the better-ventilated alveoli get less perfusion.

In sarcoidosis, CD4+ T cells release interferon gamma and TNF-alpha, which stimulate macrophages to create non-caseating granulomas. So bronchoalveolar lavage fluid in pts with sarcoidosis will show lots of lymphocytes (T cells) with high CD4+/CD8+ ratio (greater than 2 to 1). Pts may have Lofgren syndrome (erythema nodosum, arthritis, bilateral hilar lymphadenopathy). The non-caseating granulomas are made from T cells and activated macrophages. The macrophages release ACE and 1-aplha hydroxylase. The 1-alpha hydroxylase causes increased levels of activated vitamin D (1,25-dihydroxycholecalciferol), which causes elevated calcium levels. This is why sarcoidosis pts can have high ACE levels and hypercalcemia. Pts will have dyspnea and cough because of interstitial lung disease. Eyes can also be affected (anterior uveitis). The CD4+/CD8+ ratio can differentiate sarcoidosis from other ILDs (Interstitial Lung Diseases) that look similar. If the CD4+/CD8+ ratio is greater than 2 to 1, it's sarcoidosis.

Neutrophils, macrophages, and CD8+ T cells release proteases, which destroy alveoli in cigarette-smoke induced COPD. Macrophages are stimulated by cigarette smoke, causing them to release cytokines that activate CD8+ T cells, which then destroy alveoli. Stimulated macrophages also stimulate neutrophils to release proteases. Stimulated macrophages themselves also release proteases, which destroy alveoli and cause mucus hypersecretion in the bronchi. Cigarette smoke also causes epithelial cell dysfunction, so the mucocilliary escalator doesn't work as well.

Pancoast tumor = superior sulcus tumor = tumors in the lung apex. They can cause radiculopathy (numbness/tingling) down the pt's arm/fingers (dermatomes T1, T2, and T8). Pts with superior sulcus tumors have smoking history, pain in the shoulder/scapula/axilla. Horner syndrome (ptosis, miosis, anhydrosis = drooping eyelids, constricted pupils, lack of sweating) can occur is cervical sympathetic ganglia are affected.

CTA is the test of choice to diagnose PE, but it should not be used in pts with CKD because the contrast dye can cause kidney injury. So you can do a ventilation/perfusion scan instead in those pts. In a V/Q scan, a radio tracer is inhaled and then the lungs are scanned to see ventilation. Then a radio tracer is injected and the scan is done again to see perfusion. If there is good ventilation, but perfusion defect, the pt has a PE.

Walking pneumonia = atypical PNA due to mycoplasma pneumoniae. The CXR looks worse than what the pt looks like on clinical exam. You need to supplement the culture with cholesterol in order for mycoplasma pneumoniae to grow. Mycoplasma pneumoniae does not have a cell wall, capsule, or envelope.

The normal Alveolar-arterial gradient is 4 to 15 mmHg. Pts with OHS can have hypoxemia (PaO2 less than 75 mmHg), hypercapnia (PaCO2 greater than 45 mmHg) on ABG, and normal Aa gradient.

Bronchiectasis causes chronic airway inflammation, which leads to hypertrophy of bronchial arteries. Those arteries can rupture, causing hemoptysis. Pts with CF get bronchiectasis and therefore can have bronchial arterial hypertrophy.

The dual blood supply of the lungs is the pulmonary circulation and the bronchial circulation. The pulmonary circulation is the pulmonary arteries, which bring deoxygenated blood from the right ventricle to the lungs to be oxygenated; the blood is specifically brought to the respiratory bronchioles and then the alveoli. The bronchial circulation is the bronchial arteries, which come from the aorta, bringing oxygenated blood to the bronchi and terminal bronchioles. Pts with bronchiectasis can experience life-threatening hemorrhage from hyperterophied bronchial arteries that rupture with coughing.

[mcatmemoranda]

Löfgren syndrome is a type of acute sarcoidosis,[1] an inflammatory disorder characterized by swollen lymph nodes in the chest, tender red nodules on the shins, fever and arthritis.[2] It is more common in women than men, and is more frequent in those of Scandinavian, Irish, African and Puerto Rican heritage. It was described in 1953[3] by Sven Halvar Löfgren, a Swedish clinician.[4] Some have considered the condition to be imprecisely defined.

[mcatmemoranda]

I got a question about sarcoidosis. I didn't know that 5 to 10% of pts can have Bell palsy...

Restrictive lung disease and bilateral hilar lymphadenopathy are almost pathognomonic for sarcoidosis. Peripheral cranial nerve VII palsy (Bell palsy), which can present with unilateral drooping of eyelid and mouth, is the most common cranial nerve lesion of sarcoidosis, seen in up to 50% of the 5–10% of sarcoidosis patients who develop neurological symptoms. In addition to restrictive lung disease, erythema nodosum and Lofgren syndrome (bilateral hilar lymphadenopathy, erythema nodosum, and arthritis) may also be present.

Approximately 75% of untreated sarcoidosis patients will have elevated ACE levels. This is due to the increased production of ACE by the non-caseating granulomatous cells found in the various organ systems. However, elevated ACE levels have a poor sensitivity and low specificity and are not used as a diagnostic test or for disease monitoring. Diagnosis requires high clinical suspicion with compatible imaging findings, exclusion of alternative diagnoses, and confirmation with biopsy, which would demonstrate non-caseating granulomas.

Most patients do not require treatment, as they are non-symptomatic, nonprogressive, or will experience spontaneous remission. For those with more severe disease, treatment largely depends on glucocorticoids and other immunosuppressive agents.

Bottom Line: Sarcoidosis is a granulomatous disease with unclear etiology. The presence of restrictive lung disease and bilateral hilar lymphadenopathy is pathognomonic. When cranial nerves are involved, cranial nerve VII palsy (facial nerve palsy) is most common.

COMBANK Insight: The ABCDE of the sarcoid mnemonic is helpful. It stands for ACE (elevated ACE levels), Bilateral hilar lymphadenopathy, Calcium (elevated calcium levels), vitamin D (elevated vitamin D levels), and Erythema nodosum.

[mcatmemoranda]

Löfgren’s syndrome = erythema nodosum, hilar lymphadenopathy, arthritis