SAEM Clinical Images Series: A Lethal Combination of Skin and Lung Findings

dermatomyositis

A 49-year-old female with a past medical history of recurrent diverticulitis initially presented with one month of shortness of breath and a minor nonproductive cough for which she was started on doxycycline by her primary care provider. She then developed a rash on her chest, upper back, and face. Antibiotics were switched to amoxicillin and azithromycin. She underwent a brief admission of six days for shortness of breath but did not have an oxygen requirement at that time. She was evaluated by pulmonology (evaluated for cocci, unknown results), and then discharged. She then presented again to the ED with two weeks of worsening shortness of breath, intermittent fevers (Tmax 101°F), nausea/vomiting, fatigue, and arthralgias.

Vitals: BP 100/66; HR 128; Temp 37.2 °C (99 °F); Resp 44; SpO2 84%; BMI 28.25 kg/m2; Wt 79.4 kg (175 lb); Ht 1.676 m (5′ 6″)

General: NAD

Cardiovascular: Tachycardia, no m/r/g

Lungs: Coarse breath sounds at bases bilaterally, tachypneic

Abdomen: Soft, non-distended

Skin: Heliotrope rash to face (violaceous, erythematous rash to eyelids and nasolabial fold), shawl sign (erythematous patches to chest and upper back), shallow ulcers to tongue and lower inner lip, tender papules involving palms and lateral fingers bilaterally, and faint erythema of proximal nail fold

White blood cell (WBC) count: No leukocytosis

ESR: Elevated

LDH: Elevated

CK: Within normal limits

CXR: Bilateral infiltrates

CTPE: Negative for PE, but with scattered areas of ground glass and consolidative opacities throughout both lungs.

If emergency medicine physicians consider MDA5 Dermatomyositis (MDA5 DM) with rapidly progressive interstitial lung disease (RP-ILD) on their differential for patients presenting with skin and pulmonary symptoms, this can result in more rapid diagnosis and aggressive treatment.

This patient was admitted requiring 40 L HFNC, then two days later required intubation for severe ARDS and was placed on VV-ECMO the same day. Her hospital course was complicated by tachyarrhythmias requiring cardioversion, and Takostubo physiology. She was found to be MDA-5 antibody positive and ultimately expired while waiting for a lung transplant.

Take-Home Points

  • Critical actions in approaching ED patients with dermatological physical exam findings (even in the absence of known rheumatological history) with progressive pulmonary symptoms should include early consideration of dermatomyositis, serologic testing, early rheumatology and pulmonology consults, and early consideration of ECMO as a bridge to response to immunotherapy or lung transplant
  • Beginning these critical actions with first patient contact in the ED will only help improve patient outcomes throughout hospitalization.
  • Huang K, Levy RD, Avina-Zubieta JA. Successful lung transplant in rapid progressive interstitial lung disease associated with anti-melanoma differentiation associated gene 5. Rheumatology (Oxford). 2020 Aug 1;59(8):2161-2163. doi: 10.1093/rheumatology/keaa032. PMID: 32068868.
  • Koga T, Fujikawa K, Horai Y, Okada A, Kawashiri SY, Iwamoto N, Suzuki T, Nakashima Y, Tamai M, Arima K, Yamasaki S, Nakamura H, Origuchi T, Hamaguchi Y, Fujimoto M, Ishimatsu Y, Mukae H, Kuwana M, Kohno S, Eguchi K, Aoyagi K, Kawakami A. The diagnostic utility of anti-melanoma differentiation-associated gene 5 antibody testing for predicting the prognosis of Japanese patients with DM. Rheumatology (Oxford). 2012 Jul;51(7):1278-84. doi: 10.1093/rheumatology/ker518. Epub 2012 Feb 29. PMID: 22378718.
  • Moghadam-Kia S, Oddis CV, Sato S, Kuwana M, Aggarwal R. Anti-Melanoma Differentiation-Associated Gene 5 Is Associated With Rapidly Progressive Lung Disease and Poor Survival in US Patients With Amyopathic and Myopathic Dermatomyositis. Arthritis Care Res (Hoboken). 2016 May;68(5):689-94. doi: 10.1002/acr.22728. PMID: 26414240; PMCID: PMC4864500.

SAEM Clinical Images Series: More Than Skin Deep

skin

A 57-year-old female college counselor living in the northeastern United States with no PMH presented for evaluation of rash, joint pain, and dyspnea for the past three weeks. The patient first noticed the rash on her upper back, describing it as being itchy. The rash then spread to her face, scalp, and thighs. Two weeks ago, she noticed swelling in her hands and had a gradual onset of dyspnea on exertion. The patient has pain in her hands and when moving her fingers. She denied fever, cough, chills, chest pain, headache, vision changes, focal weakness, abdominal pain, nausea, vomiting, and diarrhea. She denied recent travel, sick contacts, significant time spent outdoors, known tick bites, new medications, and changes in her diet. She has never had a rash like this before.

Vitals: BP 110/70; HR 86 BPM; RR 18 breaths/min; T 37°C; SpO2 96% on RA

Skin: Warm and dry. There is a macular, violaceous rash to the upper back and upper thighs. The patient’s hands are slightly edematous with nontender papules on the palmar aspect of the hands.

CV: Heart sounds are normal. No jugular venous distention or lower extremity edema.

Lungs: There are faint bibasilar rales heard on auscultation of the chest.

Extremities: Full ROM of the joints and there are no bony deformities. The patient does not have muscular tenderness.

Neuro: Within normal limits; muscle strength 5/5 in all four extremities.

CBC w/ differential: Normal

BMP: Na 142, K 3.8, Cl 106, HCO3 24, BUN 16, Cr 0.44, Glu 112, Ca 8.5, Mg 2.2, Phos 3.4

LFT’s: AST 105, ALT 76, ALP 68, Tbili 0.2 Alb 3.7

PT/PTT/INR: 12.0s/32.2/1.04

D-dimer: 347

ESR: 62

CRP: 0.75

Ferritin: 625

CK: 195

LDH: 276

Procalcitonin: undetectable

Amyopathic dermatomyositis – specifically anti-melanoma differentiation-associated gene 5 (anti-MDA5) positive dermatomyositis as determined by subsequent inpatient auto-immunological workup. Compared to other dermatomyositides, anti-MDA5 positive dermatomyositis is characterized by an absence of traditional muscular involvement. Additionally, patients can present with respiratory symptoms related to interstitial lung disease (ILD). One phenotype of this condition is associated with a rapidly progressive ILD, but respiratory involvement may be delayed years after the initial symptoms are noticed. The patient’s clinical images demonstrate a macular, violaceous rash in the “shawl sign” and “holster sign” distribution patterns typical of dermatomyositides. Palmar papules (not to be confused with Gottron’s papules which are found on the dorsal surface of the metacarpophalangeal and interphalangeal joints) are fairly specific for anti-MDA5 positive dermatomyositis

There are no specific guidelines for treating anti-MDA5 positive dermatomyositis. Patients are typically started on a high-dose steroid regimen. A rheumatology consult should be obtained to determine if the patient would benefit from treatment with immunosuppressants. Given her complaints of dyspnea, the patient should undergo a non-contrast CT of the chest to evaluate for evidence of scarring or pulmonary fibrosis.

Take-Home Points

  • Anti-MDA5 positive dermatomyositis is associated with rapidly progressive ILD has a poor prognosis.
  • This rare form of dermatomyositis should be suspected if the patient has respiratory complaints in addition to the hallmark cutaneous findings commonly observed in all types of dermatomyositides. Palmar papules are fairly specific for anti-MDA5 positive dermatomyositis. It often lacks typical historical and physical features of muscular weakness.
  • Treatment involves high-dose corticosteroids and consideration of immunomodulator therapy.

  • Allenbach Y, Uzunhan Y, Toquet S, Leroux G, Gallay L, Marquet A, Meyer A, Guillaud C, Limal N, Gagnadoux F, Hervier B, Borie R, Deligny C, Terrier B, Berezne A, Audia S, Champtiaux N, Devilliers H, Voermans N, Diot E, Servettaz A, Marhadour T, Castelain V, Humbert S, Blanchard-Delaunay C, Tieulie N, Charles P, Gerin M, Mekinian A, Priou P, Meurice JC, Tazi A, Cottin V, Miyara M, Grange B, Israël-Biet D, Phin-Huynh S, Bron C, De Saint Martin L, Fabien N, Mariampillai K, Nunes H, Benveniste O; French Myositis Network. Different phenotypes in dermatomyositis associated with anti-MDA5 antibody: Study of 121 cases. Neurology. 2020 Jul 7;95(1):e70-e78. doi: 10.1212/WNL.0000000000009727. Epub 2020 Jun 2. PMID: 32487712; PMCID: PMC7371381.
  • Nombel A, Fabien N, Coutant F. Dermatomyositis With Anti-MDA5 Antibodies: Bioclinical Features, Pathogenesis and Emerging Therapies. Front Immunol. 2021 Oct 20;12:773352. doi: 10.3389/fimmu.2021.773352. PMID: 34745149; PMCID: PMC8564476.

ALiEM AIR Series | Respiratory 2023 Module

ALiEM AIR- respiratory module 2023

Welcome to the AIR Respiratory Module! After carefully reviewing all relevant posts in the past 12 months from the top 50 sites of the Digital Impact Factor [1], the ALiEM AIR Team is proud to present the highest quality online content related to related to respiratory diseases in the Emergency Department. 6 blog posts met our standard of online excellence and were approved for residency training by the AIR Series Board. More specifically, we identified 3 AIR and 3 Honorable Mentions. We recommend programs give 3 hours of III credit for this module.

AIR Stamp of Approval and Honorable Mentions

In an effort to truly emphasize the highest quality posts, we have 2 subsets of recommended resources. The AIR stamp of approval is awarded only to posts scoring above a strict scoring cut-off of ≥30 points (out of 35 total), based on our scoring instrument. The other subset is for “Honorable Mention” posts. These posts have been flagged by and agreed upon by AIR Board members as worthwhile, accurate, unbiased, and appropriately referenced despite an average score.

Take the AIR Respiratory Module at ALiEMU

Interested in taking the AIR quiz for fun or asynchronous (Individualized Interactive Instruction) credit? Please go to the above link. You will need to create a free, 1-time login account.

Highlighted Quality Posts: Respiratory

SiteArticleAuthorDateLabel
EMCritIBCC: Asthma Josh Farkas, MDApril 12, 2023AIR
EMCritIBCC: Severe Community Acquired PneumoniaJosh Farkas, MDOctober 11, 2022AIR
EM DocsEmpyema: ED Presentation, Evaluation, and ManagementHeath Garner, MDApril 11, 2022AIR
Rebel EMPigtail Catheter vs Large Bore Chest Tube for PneumothoraxJessica DiPeri, MDDecember 1, 2022HM
The Skeptics Guide to EMHey Ho! High Flow vs Standard O2 therapy for hospitalized children with respiratory failureDennis Ren, MDApril 22, 2023HM
PEM BlogWhy we do what we do: Treatments for severe asthmaBrad Sobolewski, MDAugust 23, 2022HM

(AIR = Approved Instructional Resource; HM = Honorable Mention)

If you have any questions or comments on the AIR series, or this AIR module, please contact us!

Reference

  1. Lin M, Phipps M, Chan TM, et al. Digital Impact Factor: A Quality Index for Educational Blogs and Podcasts in Emergency Medicine and Critical Care. Ann Emerg Med. 2023;82(1):55-65. doi:10.1016/j.annemergmed.2023.02.011, PMID 36967275

 


 

SAEM Clinical Images Series: ‘Tis Not the Season to be Wheezing

wheezing

A 2-year-old male with a history of solitary kidney presented with greater than one month of daily coughing, wheezing, and decreased appetite. The patient was previously seen by his primary care physician after three weeks of symptoms where he was prescribed albuterol as needed for viral bronchospasm. The patient’s wheezing did not improve after two weeks of albuterol treatment so a chest x-ray was ordered. The patient’s mother denied any fevers, vomiting, diarrhea, weight changes, or night sweats.

Vitals: BP 131/60; Pulse 148; Temp 36.7 °C (98.1 °F) (Axillary); Resp 28; Wt 15.7 kg (34 lb 9.8 oz); SpO2 95%

General: Alert; well appearing

HEENT: Pupils equally reactive to light; moist mucous membranes; nares with normal mucosa without discharge

Cardiovascular: Regular rate; regular rhythm; normal S1, S2; no murmur noted; distal pulses 2+

Pulmonary: Good aeration throughout all lung fields; clear breath sounds bilaterally; prolonged expiratory phase; stridor with agitation

Abdomen: Soft; non-tender; non-distended

White blood cell (WBC) count: 56.1/uL (Blasts 58%)

Platelets: 288/uL

Uric acid: 8.3 mg/dL

LDH: 2231 iU/LD

D-Dimer: 3.22 ug/mL

Fibrinogen: 463 mg/dL

Bronchospasm, bronchiolitis, viral infection, pneumonia, foreign body aspiration, space-occupying lesion, vocal cord dysfunction, cardiac dysfunction, and acute chest in patients with sickle cell disease.

The radiograph shown demonstrates a mediastinal mass. This patient was ultimately diagnosed with T-cell acute lymphoblastic leukemia. T-ALL can present with fatigue, fevers, weight loss, easy bleeding/bruising, paleness, or a mediastinal mass. Mediastinal masses found on chest x-ray require further evaluation to determine the diagnosis, location, and treatment. If malignancy is suspected, an oncology referral and bone marrow sample will be necessary.

Take-Home Points

  • In patients with first-time wheezing that does not improve with bronchodilator therapy, consider alternative diagnoses and further evaluation.
  • A mediastinal mass is found at the time of diagnosis in 10% to 15% of children with acute lymphoblastic leukemia.

  • Steuber, P (2021). Overview of common presenting signs and symptoms of childhood cancer.UpToDate. Retrieved January 2, 2021.2.
  • Juanpere, S., Cañete, N., Ortuño, P., Martínez, S., Sanchez, G., & Bernado, L. (2013). A diagnostic approach to the mediastinal masses. Insights into imaging, 4(1), 29–52.https://doi.org/10.1007/s13244-012-0201-0

SAEM Clinical Images Series: Found Down

found down

A 67-year-old caucasian male experiencing homelessness was “found down” in a parking lot. EMS reported that he had a GCS of 6 with a systolic blood pressure in the 80’s, finger stick glucose of 100, and no response to intranasal naloxone. He was intubated in the field and arrived to the emergency department unresponsive with a BP of 95/60, HR 125, T 38°C, and O2 Sat 100%. Hemodynamic stabilization was achieved with central venous access, and laboratory and imaging studies for the evaluation of altered mental status ensued.

General: Disheveled male

HEENT: Normocephalic; PERRLA 3-2 mm; dried blood in nares

Skin: Warm; dry; no visible signs of trauma

Cardiovascular: Tachycardic with no murmurs, rubs, or gallops

Respiratory: Bilateral breath sounds on ventilator; diffuse rales

Gastrointestinal: Soft; non-distended; bowel sounds present

Musculoskeletal: No deformities

Neurologic: Unresponsive; GCS 3

COVID-19 rapid antigen: Detected

Complete Blood Count (CBC): WBC 17 k; Hemoglobin 15; Platelets 185

Comprehensive Metabolic Panel (CMP): Na 133; K 4.6; Cl 91; CO2 21; BUN 18; Cr 2.2; Ca 8.4; Alb 2.1; Tbili 0.4; Alk phos 112; AST 242; ALT 68

ABG on FiO2 100%: 6.99/>95/405/23/100%

Lactate: 16.4

Ammonia: 90

CK total: 716

Trop I HS: 809

PT: 14

INR: 1.05

PTT: 45

Urinalysis: Unremarkable

EtOH, Acetaminophen, Salicylate: Negative

UDS: Negative

Chest Radiograph: Diffuse ground-glass opacities

Air embolism to the right ventricle and pulmonary artery. As little as 20 mL or less of air rapidly infused may cause obstruction, ischemia, and hemodynamic collapse.

Risk factors include central venous catheterization, lung trauma, ventilator usage, hemodialysis, surgery (esp. coronary, neurosurgery), childbirth, and scuba diving barotrauma.

Take-Home Points

  • In the appropriate clinical scenario, especially those involving respiratory, cardiac, and neurologic findings where invasive procedures were utilized, the diagnosis of venous air embolism should be entertained.
  • Immediate management of an air embolism involves administration of 100% oxygen by nonrebreather mask (NRM) or ventilator and placement of the patient in the left lateral decubitus (Durant maneuver) and Trendelenburg positions. Hyperbaric oxygen therapy has also been used if there is no clinical improvement.
  • The purpose of the Durant maneuver and Trendelenburg position is to trap air along the lateral right ventricular wall, preventing right ventricular outflow obstruction and embolization into the pulmonary circulation.

  • Gordy S, Rowell S. Vascular air embolism. International Journal of Critical Illness and Injury Science. 2013;3(1):73. doi:10.4103/2229-5151.109428 Malik N, Claus PL, Illman JE, Kligerman SJ, Moynagh MR, Levin DL, Woodrum DA, Arani A, Arunachalam SP, Araoz PA. Air embolism: diagnosis and management. Future Cardiol. 2017 Jul;13(4):365-378. doi: 10.2217/fca-2017-0015. Epub 2017 Jun 23. PMID: 28644058.

SAEM Clinical Image Series: An Uncommon Cause of Shortness of Breath

shortness of breath

A 102-year-old female presents with intermittent epigastric abdominal pain for the last two days. Episodes have no relieving or exacerbating factors. The pain originates in the epigastrium and radiates diffusely to the abdomen and back, resolving on its own within minutes of onset. She has had one episode of nonbilious, non-bloody emesis. Her last bowel movement was two days prior and she hasn’t been able to pass gas. The pain is associated with mild shortness of breath which has been progressively worsening since the onset of symptoms. Her family was concerned and called EMS because the shortness of breath has worsened and the episodes of pain have been progressively worsening in intensity. The patient denies fever, chills, hematuria, urinary frequency, chest pain, headache, dizziness, syncope, recent traumatic events, and any other associated symptoms.

General: Well-appearing; no acute distress; awake, alert, and oriented to date, place, and person

Cardiovascular: Regular rate and rhythm; S1/S2 present; 2+ systolic ejection murmur; capillary refill <2 seconds; 2+ pulses in all extremities

Respiratory: Lungs clear to auscultation bilaterally with diminished breath sounds in the left lower lobe; no signs of respiratory distress; no accessory muscle use

Abdomen: Soft; non-tender; non distended; no palpable masses; no guarding or rebound tenderness; no signs of peritonitis

Extremities: Full range of motion of all extremities; nonambulatory at baseline

Complete blood count (CBC): WBC 10.8 x 10^3/mcl; Hgb 12 g/dl; Hct 40.1%; Plt 375 x 10^3/mcl

Basic metabolic panel (BMP): Na 139 mmol/L; K 3.7 mmol/L; Cl 97 mmol/L; CO2 31 mmol/L; Glucose 170 mg/dL; BUN 10 mg/dL; Cr 0.58 mg/dL; Ca 10.2 mmol/L

Liver function test: AST 19 U/L; ALT 7 U/L; Alk Phos 144 U/L

Lipase: 11 U/L

Venous blood gas (VBG): pH 7.33; pCO2 61.1 mmHg; pO2 38 mmHg; BE -7 mmol/L

Lactic acid: 1.56 mmol/L

Small bowel obstruction (SBO) secondary to a spigellian hernia with an associated hiatal hernia. 

The CT demonstrates a spigellian hernia causing a small bowel obstruction. Spigellian hernias are hernias in the spigellian fascia which is located between the semilunar line and the lateral edge of the rectus abdominus muscle. These hernias constitute 0.12% of abdominal wall hernias, making them very rare and difficult to diagnose clinically. Spigellian hernias often go unnoticed until they are strangulated and require surgery. This patient not only had a rare spigellian hernia but also had a hiatal hernia causing the stomach to enter the pleural space. It’s possible that the bowel obstruction worsened the hiatal hernia with the backup of gastric contents and gas.

Take-Home Points

  • Spigellian hernias are rare abdominal wall hernias with a myriad of potential complications.
  • Shortness of breath is frequently considered a pathology involving the lungs or pulmonary vasculature, however abdominal complaints, especially in this case, can cause significant respiratory distress.
  • Elderly patients may have difficulty verbalizing their exact symptoms, and it is good practice to gather collateral information from families to aid in caring for these patients.

  • Spangen L. Spigelian hernia. World J Surg. 1989 Sep-Oct;13(5):573-80. doi: 10.1007/BF01658873. PMID: 2683401.

 

High-Dose Nitroglycerin for Sympathetic Crashing Acute Pulmonary Edema

Background

Nitroglycerin (NTG) is an important intervention to consider for patients with Sympathetic Crashing Acute Pulmonary Edema (SCAPE) as it significantly reduces preload, and even modestly reduces afterload with high doses. For acute pulmonary edema in the ED, NTG is often administered as an IV infusion and/or sublingual tablet. Starting the infusion at ≥ 100 mcg/min produces rapid effects in many patients and can be titrated higher as tolerated, with doses reaching 400 mcg/min or greater. Combined with noninvasive positive pressure ventilation (NIPPV) and in some cases IV enalaprilat, patients often turn around quickly, from the precipice of intubation to comfortably lying in bed [1, 2]. But what does the literature say about starting with a high-dose NTG IV bolus followed by an infusion?

Evidence

A 2021 prospective, pilot study of 25 SCAPE patients proposed a clear and systematic protocol (below) for treating these critically ill patients with a combination of high-dose NTG bolus (600 – 1000 mcg over 2 mins) followed by an infusion (100 mcg/min) and NIPPV [3].There were no cases of hypotension after the bolus and 24 of the 25 patients were able to avoid intubation. Additionally, an earlier PharmERToxGuy post summarizes some of the previous studies evaluating the use of a high-dose NTG IV bolus for acute pulmonary edema.

It is important to note that some institutions may not allow IV push NTG or may limit the use of NTG boluses. Providers may then opt to implement dosing strategies such as bolusing from an IV infusion pump or initiating the infusion at a high rate for a short period (e.g., NTG 300 mcg/min for 2-3 minutes) before reducing the rate to a more traditional infusion rate (e.g., 100 mcg/min).

Bottom Line

  • A few small ED studies support the use of an initial IV NTG bolus followed by an infusion compared to the infusion alone [1, 2]
  • There is a low risk of hypotension following a single IV NTG bolus
  • Consider using the following protocol to identify which doses may be best for specific patients based on initial systolic blood pressure

Click for full-sized version [3]

 

 

Want to learn more about EM Pharmacology?

Read other articles in the EM Pharm Pearls Series and find previous pearls on the PharmERToxguy site.

References

  1. Wang K, Samai K. Role of high-dose intravenous nitrates in hypertensive acute heart failure. Am J Emerg Med. 2020;38(1):132-137. doi: 10.1016/j.ajem.2019.06.046. PMID: 31327485.
  2. Wilson SS, Kwiatkowski GM, Millis SR, Purakal JD, Mahajan AP, Levy PD. Use of nitroglycerin by bolus prevents intensive care unit admission in patients with acute hypertensive heart failure. Am J Emerg Med. 2017;35(1):126-131. doi: 10.1016/j.ajem.2016.10.038. PMID: 27825693.
  3. Mathew R, Kumar A, Sahu A, Wali S, Aggarwal P. High-dose nitroglycerin bolus for sympathetic crashing acute pulmonary edema: a prospective observational pilot study. The Journal of Emergency Medicine. Published online June 2021:S0736467921004674. doi: 10.1016/j.jemermed.2021.05.011.
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