All posts by vmcimchiefs

Myxedema Coma

We discussed an interesting case of a 69yo female who presented with acute onset AMS and hypothermia diagnosed with myxedema coma with TSH 383 and free T4<0.1. She had been on treatment with Atezolizumab for high-grade urothelial cancer. Immune checkpoint inhibitors can cause multiple toxicities including hypophysis, siccca syndrome, thyroiditis, myocarditis, pneumonitis, ATN, AIN, MCD, GN, vasculitis, colitis and perforation.

While treating myxedema coma, always give glucocorticoids FIRST to avoid precipitating adrenal crisis in those with undiagnosed adrenal insufficiency. We also treat with thyroid hormone, supportive measures and treating underlying etiology.

Diffuse Alveolar Hemorrhage

We describe a middle aged male with history of APLS on chronic warfarin presenting for subacute abdominal pain and dry cough found to have bilateral adrenal hemorrhage with hospital course complicated by acute hypoxic respiratory failure. Patient then had frank hemoptysis from DAH likely due to 1) catastrophic APLS and 2) ANCA Vasculitis (MPO+). Differential for DAH is as below. As we worked up massive hemoptysis leading to intubation, patient was ANA, ANCA and p-ANCA positive leading to ANCA vasculitis leading to DAH. At the same time, the bilateral adrenal hemorrhages led and DAH gave rise to concern for CAPS – catastrophic AntiPhospholipid Syndrome.

Classification for CAPS

  1. Evidence of involvement of three or more organs, systems and/or tissues
  2. Development of manifestations simulatenously or in less than a week
  3. Confirmation by histopathology of small vessel occlusion in at least one organ or tissue
  4. Lab confirmation of the presence of antiphospholipid antibodies (lupus anticoagulant, anticardiolipin and anti-beta2-glycoprotein I antibodies)

All 4 criteria leads to a definite diagnosis of CAPS. Our patient met 3 of 4 criteria for CAPS leading to probable CAPS.

It is important to suspect DAH even when there is no frank hemotpysis but there is hypoxemia, anemia and diffuse GGO. Review differential fro DAH below. Patient received PLEX initially for possible CAPS and then received Rituximab IV + Methylprednisolone for ANCA associated vasculitis.

Pleural Effusion

We reviewed a case of a elderly male with dyspnea and productive cough over 6 weeks in setting of unintentional weight loss found to have imaging findings of L lung white out from large pleural effusion. Chest tube results showed pH 7.47, LDH 325, Protein 5.3, Triglyceride 31, Glucose 91, Serum LDH 344, Serum Protein 8.2. RBC 15k, WBC 921, with 62% lymphocytes, 15% neutrophils and 22% monocytes. We reviewed 2 different criteria for determining exudative vs transudative pleural effusion

Our patient had a large exudative effusion requiring indwelling chest tube. Patient was found to have left pleural mass that was biopsied with results indicating poorly differentiated carcinoma, staining + for GATA3 (urothelial carcinoma and sometimes primary lung carcinoma). Patient was discharged an indwelling pleural catheter.

Pulmonary Arterial Hypertension due to Systemic Sclerosis

We discussed a young male with chief complaint of dyspnea on exertion that was progressive. Other symptoms included GERD, skin tightening on face and fingers, Raynaud’s in digits who was found to have hypoxic respiratory failure and severe RV enlargement. Right heart cath showed mean PA pressure of 32 mm Hg with wedge pressure 3mmHg and RVSP 52mmHg, CI 4.15L/min/m2. He was diagnosed with PAH (group 1 PH) likely due to underlying ILD from connective tissue disease, specifically Limited Cutaneous Systemic Sclerosis (CREST).

PH is defined as mean PA pressure (PAP) of >20mmHg at rest. Here is the 5 groups of PH. Remember PAH is a specific subgroup of PH. Group 1, 3 and 4 require mean PAP >20mmHg; Wedge<15mmHg and PVR>3 WU. Group 2 and 5 require mean PAP >20mmHg, wedge>15mmHg and PVR <3Wu.

Remember ANA is positive in 70% of systemic sclerosis but other antibodies are associated with more specific complications of systemic sclerosis. Here is a table to summarize.

Silicosis

We discuss a interest case of an elderly male from Mexico with risk factors for stone cutting presenting with a worsening dry chronic cough in setting of newly diagnosed HCC. Chest CT shows numerous centrilobular and perilymphatic nodules and calcified mediastinal lymphadenopathy. Our differential for “random” (perilymphatic & centrilobular) is broad.

Our patient underwent endobronchial ultrasound guided transbronchial needle aspiration + BAL with Cone Beam CT guided Transbronchial Cryobiopsy. Results showed silicosis and pulmonary Cryptococcus.

Actinomycosis

We discussed an elderly homeless male with uncontrolled DM with recent dental surgery presenting with subacute painless swelling of his right mandible with acute development of purulent drainage diagnosed with cervicofacial actinomycosis.

1. Fistulization from the perimandibular region is the most easily recognized manifestation of cervicofacial actinomycosis

2. Presents as a chronic, slow progressive, NONTENDER, indurated mass that can involve into draining sinus tract or fistula and sometimes progress into an abscess

3. It can mimic NOCARDIA because it is Gram + filamentous rod however it is NOT acid fast

4. Treatment for severe infection with CTX IV or Penicillin G IV OR for mild infection with PO amoxicillin or PO penicillin V

5. Duration of treatment is 2-6 mo for MILD disease or 6-12 mo for SEVERE disease

Takayasu Arteritis

We discussed a case of a young woman with chronic, progressive, R shoulder and arm pain that worsens with activity and improves with rest. R radial pulse was difficult to feel and BP in RUE was lower than BP in LUE by >20mmHgb (SBP). Patient’s CTA showed thickening of aortic arch and great vessel branches with elevation of ESR. We discussed the differential for aortic pathology.

Unilateral weakness was attributed to vascular etiology (due to claudiatication symptoms, pulselessness and BP differential) and inflammatory etiology (given high ESR). Patient was diagnosed with Takayasu Arteritis, a type of granulomatous vasculitis affecting aorta and major branches such as coronary and pulmonary arteries. Patient improved on steroids and rituximab with follow up CTA showing normal aorta.

SVC syndrome due to malignancy

We discussed a case about a young man with no significant past medical history, who presented with acute-subacute diffuse facial and neck swelling with associated headaches + dizziness + dyspnea on exertion, found to have SVC syndrome due to malignancy.

Differential diagnosis for facial swelling
Consider a framework based on location

  • Orbital / periorbital: cavernous sinus thrombosis; orbital cellulitis; myxedema coma
  • Subcutaneous: cellulitis
  • Submandibular: Ludwig’s angina; salivary gland pathology
  • Oral: Angioedema; anaphylaxis; dental infection; parotid infection
  • Misc: Trauma; SVC syndrome; malignancy; thyroid disease

Etiologies of SVC syndrome

  • Malignancy (majority of cases): NSCLC, SCLC, lymphoma, thymoma, germ cell malignancy
  • Non-malignant: Thrombus (catheter-associated); infections; aortic aneurysms; thyroid diseases; fibrosing mediastinitis

Treatment

  • Guided by severity of symptoms
  • Goal: treat underlying cause (e.g. malignancy) + relieve symptoms
  • Glucocorticoids (for steroid responsive malignancies e.g. lymphoma), chemotherapy, radiation therapy, surgical resection
  • Thrombus tx: Intravascular stenting, thrombolysis

Listeriosis

We discussed a case about an elderly woman with insulin-dependent T2DM and rheumatoid arthritis (on methotrexate and hydroxychloroquine), who presented with subacute-chronic post-prandial abdominal pain, loose stools, mental status changes, found to have chronic SMA stenosis & Listeria bacteremia.

Individuals at highest risk of Listeria

  • Immunocompromised
  • Extremes of age (neonates & elderly)
  • Pregnant women

Clinical syndromes of Listeria

  • Febrile gastroenteritis
  • Invasive infections
    • Bacteremia
    • CNS infection (meningitis, encephalitis)
  • Focal infections (rarer, mostly case reports; e.g. septic arthritis, endocarditis, SSTI)

Treatment

  • Antibiotics of choice: Ampicillin or Penicillin G
  • Synergistic effect: Gentamicin
    • Watch out for nephrotoxicity and ototoxicity with Gentamicin

Takotsubo cardiomyopathy

We discussed a case of an elderly woman with history of HTN and pre-diabetes, who presented with acute hypertensive emergency and dizziness, found to have evolving EKGs (ST elevations in anterior/lateral precordial leads) and elevated troponins concerning for STEMI, due to Takotsubo cardiomyopathy.

Takotsubo cardiomyopathy

  • Transient regional LV systolic dysfunction
  • Absence of angiographic evidence of obstructive CAD or acute plaque rupture
  • Affects women > men
  • Typically seen in older adults (~60s)

Pathogenesis

  • Pathogenesis is not completely known, but theorized to be due to catecholamine excess. This leads to diffuse catecholamine-induced microvascular spasm / dysfunction.

Signs and symptoms

  • Physical or emotional stress trigger (not always present! one study reported lack of stress trigger in 28.5% of cases)
  • Presents like ACS

Complications to watch out for

  • Heart failure
  • Arrhythmias
  • Mitral regurgitation
  • Cardiogenic shock, cardiac arrest
  • Stroke (embolization from an apical thrombus that forms due to severe systolic heart failure)

Workup

  • EKG findings
    • ST elevations (most common) >>> ST depressions > other nonspecific findings (e.g. QT prolongation, T wave inversions)
  • Elevated troponin
  • Elevated NT pro-BNP

Diagnosis
Mayo Clinic Criteria can help with diagnosis of takotsubo cardiomyopathy.
All four criteria are required for diagnosis.

Echo

  • Apical ballooning of LV reflecting regional wall motion abnormalities
  • Reduced EF
Apical ballooning seen on cardiac cath. Notice the lack of contraction at the LV apex vs the level of contraction at the LV base.
Apical 4 chamber view of a patient with Takotsubo cardiomyopathy

Treatment

  • Conservative, supportive treatment
  • Thromboembolism
    Treatment recommendations are extrapolated from studies of patients who’ve suffered an MI and subsequently developed intraventricular thrombus
    • Presence of intraventricular thrombus
      • Treat with Vitamin K antagonist (Warfarin) for ~3 months
    • In patients with low bleed risk and severe systolic dysfunction (LV EF <30%) and no evidence of intraventricular thrombus
      • Can consider prophylaxis with Vitamin K antagonist (Warfarin) until LV dysfunction resolves OR for 3 months (whichever is shorter)