Dieulafoy lesion causing obscure overt GI bleed!

Today we talked about an elderly man with recent ACS on DAPT, HFrEF 25%, and h/o colonic angiodysplasia induced LGIB who presented with acute onset of obscure overt upper GI bleed, found to have a dieulafoy lesion on repeat EGD!


Clinical Pearls

  • Overt GI bleed refers to bleeding that is clinically evident (i.e. hematemesis, hematochezia, melena, etc.)
  • Occult GI bleed refers to slow bleed that primarily manifests as iron deficiency anemia and/or positive guaiac stool.
  • Obscure GI bleed refers to evident GI bleed without a clear source on EGD/colo.
    • Most common cause is angiodysplasia
    • First step in the work up of obscure GI bleed is to repeat EGD/colo.  Up to 50% of cases are successfully diagnosed this way.
  • Tachycardia is the first sign of blood loss and suggests up to 30% total blood volume loss.  Hypotension develops once blood loss >30%.
  • For people at high risk of thromboembolic events requiring anticoagulation, restart anticoagulation/antiplatelet therapy as soon as possible after acute bleeding is resolved (prior to discharge!). Be sure to discuss risk of rebleeding with endoscopist prior to restarting these agents.

Obscure GI bleed

Etiology:

The following is a simplified breakdown of diagnoses to consider in obscure GI bleed.  Keep in mind that many diagnoses can present as overt or occult GI bleed.  We have listed them here under the more common way in which they present:

Obscure GI bleed breakdown

  • Remember that NSAID induced ulcers can occur anywhere in the GI tract as far distally as the splenic flexure!
  • Angiodyplasia is the most common cause of obscure GI bleed.

Work up:

  • Repeat EGD/colo is the first step.  Up to 50% of the cases are diagnosed in this way.
  • If EGD/colo inconclusive, then
    • Obscure active bleed
      • Tagged RBC scan: technetium 99m-labeled RBC or sulfur colloid nuclear scans. Can detect slow bleeds with accuracy varying from 24-91%.  They can only identify a general area where bleeding is occurring (not accurate) and a follow up separate intervention is indicated if a source is identified.
      • Angiography: Can identify faster bleeds, more effective at localizing bleed, but less sensitive than tagged RBC (27-77%). Allows intervention at the same time.
      • Enteroscopy: push, single/double balloon enteroscopy are sensitive (up to 80%) and allow for intervention but are operator dependent and may not always be available
      • Intraoperative endoscopy: laparotomy or laparoscopy (sensitivity 58-80%). Last resort.
    • Obscure occult bleed
      • Capsule endoscopy: allows for imaging of the small bowel and can has a high sensitivity (83%). May be difficult to localize lesion based on imaged. Not a good tool in active bleed.
      • Enteroscopy as noted above

Management:

  • Treat the underlying etiology!
  • In the case of dieulafoy lesions, treatment with electrocautery, hemoclips, argon plasma coagulation, injection therapy, or a combination is effective.
  • For people with DAPT or on anticoagulation with warfarin, the current recommendation is to start these agents as soon as hemostasis is achieved.  The decision on when to start depends on the type of lesion, risk of bleed (based on edoscopist’s opinion), and risk of thromboembolic event (based on this cohort study and this meta-analysis).

Hemodynamics in GI bleed:

  • Tachycardia is the first vital sign abnormality in GI bleed and is noted with 15-30% blood volume loss.  With lower amounts of blood loss, tachycardia may be present upon standing.  If a patient is tachycardic while laying supine, blood loss is closer to 30%.
  • Blood loss >30% total body volume results in hypotension.  This typically begins as a widening of pulse pressure (drop in diastolic BP) followed by a drop in systolic BP.

 

Myasthenia Crisis Secondary to… a Thymoma! 12/26/2018

Our case today is a 49 year old woman with no medical history, presenting with 1 month of difficulty swallowing, voice changes, and more recently dysphagia with liquids and solids, and shortness of breath. Her symptoms are worse during the night time to the point that she couldn’t swallow her own spit/secretions. She presented with respiratory failure requiring intubation, and on CXR/CT she was found to have an anterior mediastinal mass concerning for a… thymoma!


Let’s first briefly review Myasthenia Gravis before moving onto Myasthenia Crisis, and lastly, Thymomas.

Myasthenia Gravis

Epidemiology

  • Bimodal: Early peak in 2-3rd decades (female predominance) and late peak 60-80s (male predominance).
  • F in post-partum period have inc risk.
  • Possible association with: neuromyelitis optical, autoimmune thyroid disorders, SLE, RA.

Diagnosis

  • Bedside
    • Ice-pack test: Improvement of ptosis after application of an ice pack = positive. Sensitivity around 80ish %, limited to patients with ptosis and not helpful for those with extraocular muscle weakness.
      • myastheniagravis-myasthenia-diagnosis-clinical-icepack-original.jpeg Source: NEJM, Grepmed
    • Edrophonium test is no longer used very often, in a nut shell, it is a Acetylcholinesterase inhibitor with rapid onset (within 30-45 seconds), produces improvement of affect muscles after injection.
      • 80-90% sensitivity but high rates of false positive. Not very specific.
  • Serology (seropositive in 90% of MG patients).
    • AChR Ab
      • Titers do not correlate with disease activity
      • 85% positive in generalized MG
      • Highly specific, extremely low false positives (LE, certain motor neuro dz, polymyositis)
    • MuSK-Ab
      • Seen in 38-50% with generalized MG who are AChR Ab negative.
    • Thymoma patients with MG: 98-100% will have positive AChR-Ab.
      • NPPV for thymoma in the absence of AChR-Ab is 99.7%
    • Seronegative: 6-12%, more likely to have purely ocular myasthenia.
  • EMG: Can help confirm diagnosis
    • Single fiber EMG
      • Abnormal in > 90% of those with generalized MG, less so in ocular MG
      • Most sensitive diagnostic test for MG, 90-95% sensitivity (les for ocular MG), 91% specific.
    • Repetitive nerve stimulation
      • Readily available but less sensitive vs SFEMG
      • Nerve is stimulated multiple times, and the compound muscle action potential is recorded, test is considered positive if progressive decline in CMAP readings with the first 4-5 stimuli.
      • Sensitivity 75-80%

Management

  • Symptomatic: Pyridostigmine, max daily dose 7mg/kg
    • Too much pyridostigmine can cause cholinergic crisis, leading to—weakness. Chances of this dec by limit daily dose of pyridostigmine to less than 960mg daily
  • Chronic immunotherapy: Required for those with sx on pyridostigmine or recurrence of sx on pyridostigmine after initial improvement.
    • Steroids or immunosuppressives i.e. azathioprine, mycophenolate, cyclosporine.
  • Thymectomy: Recommended for age < 60, has been considered beneficial even without presence of a thymoma.

Myasthenic Crisis

Definition: Weakness severe enough to impair muscles of respiratory requiring mechanical ventilation.

  • 10-20% of pts with MG will experience at least one crisis, annual risk 2-3%
  • For 13-20% of pts with MC, the crisis is their first clinical manifestation of MG and initial diagnosis.
  • Most occur in the first few years after diagnosis of MG.

Presentation

  • Progressive generalized or bulbar weakness leading to respiratory failure.
  • Variable presentation in terms of degree of weakness (general vs respiratory)
  • May be precipitated by: infection, surgery, pregnancy, childbirth, medication tapering, certain drugs (beta blockers, antibiotics), magnesium

Evaluation

  • Airway/Breathing: Monitor respiratory muscle strength frequently, should be admitted to MICU
  • Indications for intubation:
    • FVC < 15 – 20 mL/kg
    • NIF < -25 to -30 cmH2O (i.e. 0 to -24)
    • Respiratory fatigue
    • PCO2 > 50
    • Difficulty with secretions

Management

  • Intubation if signs of impending respiratory failure.
    • Elective intubation, rather than emergent, is preferred.
  • Rapid IVIG or plasma exchange, FAST
    • Plasmapheresis directly removes acetylcholine receptor ab in the circulation
  • High dose glucocorticoids, azathioprine, cyclosporine, or mycophenolate
  • Wean as respiratory muscle strength improves after completing or IVIG or plasma exchange.
  • Aggressive pulmonary toilet.
  • Pyridostigmine generally avoided after intubation temporarily since it might increase secretions, leading to more complex pulmonary care.
    • Can be resumed after extubation.

Thymoma

Epidemiology

  • Median age 40-60
  • Men ~ Women
  • No known risk factors but strong association with myasthenia gravis

Presentation

  • Local thoracic symptoms
  • Asx
  • Paraneoplastic symptoms
  • Up to ½ of pts with thymoma will have MG like sx.
  • MG is common with thymomas but rare in thymic carcinoma

Paraneoplastic presentation

  • Neuro: MG, polymyositis, Lambert Eaton, Isaac’s syndrome, stiff person syndrome
  • Heme: Pure red cell aplasia, hemolytic anemia, pernicious anemia, agranulocytosis
  • Derm: Alopecia areata, pemphigus, scleroderma, vitiligo, oral lichen planus
  • Endo: Addison’s disease, Cushing syndrome, panhypopit, thyroiditis
  • Other: Nephrotic syndrome, RA, sarcoid, hepatitis, hypogammaglobulinemia, myocarditis

Diagnosis

  • CT and/or MRI
  • Carcinoma findings: Necrotic, cystic, or calcified, irregular contour
  • Definitive dx requires tissue biopsy

Staging

  • Masaoka staging system vs American Joint Committee on Cancer (AJCC), with the former being more commonly used.

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Management

  • Surgical resection: as much as possible, including complete resection of the thymus. Potentially curative.
  • If extensive disease, can consider chemo followed by radical resection +/- RT for potentially resectable cases.
  • If complete resection cannot be done, maximal debulking followed by post-op RT.
  • Potential phrenic nerve damage due to tumor expansion or surgery. Can sacrifice one for surgical resection but if both are involved, then it’s a more complicated discussion.

Prognosis

  • Main determinant = staging and complete resectability of the tumor
  • Most commonly used staging system = Masaoka Staging System
  • Masaoka stage I and II: Favorable
  • Masaoka stage III: 27% recurrence after complete resection, 62% with incomplete resection. 10yr survival 83%
  • Masaoka stage IV: 10-yr survival is 47%

Cardiac arrest & anoxic brain injury

Today we discussed the case of a young man with Duchenne’s Muscular Dystrophy complicated by chronic respiratory failure and dilate cardiomyopathy who was found down at home in asystolic arrest.  Though ROSC was achieved en route to the hospital, patient suffered significant sequelae of anoxic brain injury.


Clinical Pearls

  • Most common causes of out-of-hospital cardiac arrests are
    • Acute MI
    • Cardiomyopathy
    • Primary arrhythmia
  • Most immediate threat to survival post cardiac arrest is cardiovascular collapse.
  • Most common cause of death in out-of-hospital cardiac arrests is neurologic injury.
  • Post cardiac arrest hemodynamic targets
    • SpO2 > 40%
    • PaCO2 > 40
    • MAP >65 (preferably 80-100 mmHg)
  • Read below for more info on the ongoing battle between therapeutic hypothermia (TH) and targeted temperature management (TTM) to reduce brain injury.

Post cardiac arrest management

Goals

  • Determining and treating cause of arrest
  • Minimizing brain injury
  • Managing cardiovascular dysfunction
  • Managing problems that arise from global ischemia and reperfusion injury

Most immediate threat to survival post ROSC is cardiovascular collapse.

  • Correct hypotension to maintain end-organ perfusion
  • Optimize oxygenation and ventilation
  • Correct electrolyte abnormalities

Determining cause and extent of injury:

  • Focused history
  • Exam:
    • Remember ABCs
    • Baseline neurologic exam
      • Make sure patient is off sedation or neuromuscular blocking agents
      • Brainstem reflexes:
        • Pupillary
        • Corneal
        • Oculocephalic
        • Gag
        • Cough
      • GCS ⇒ with special attention to motor score as it correlates with neurologic recovery
    • Work up
      • Causes
        • Most common are acute MI, cardiomyopathy, and primary arrhythmia ⇒ check ECG!
        • Labs
          • ABG
          • Basic electrolytes and liver function studies
          • CBC
          • Troponin q8-12 hours for 24 hours
          • Trend lactate
          • Toxicology studies
        • Management
          • Ventilator:
            • Target SpO2 >94% and PaCO2 > 40
              • Avoid hyperventilation because it leads to cerebral vasoconstriction and worsening cerebral perfusion
              • Avoid hyperoxia ⇒ a systematic review of 14 observational studies found that those with PaO2>300 mmHg had a higher in-patient mortality following cardiac arrest
          • Hemodynamics
            • Keep MAP >65 mmHg and preferably 80-100 mmHg to optimize cerebral perfusion
            • Prevent arrhythmia with meds only if patient has recurrent or ongoing unstable arrhythmia. No data on routine prophylactic use of these agents in other patients.
            • Coronary revascularization if indicated
          • Decrease brain injury
            • Targeted temperature management (TTM) and therapeutic hypothermia (TH)
              • Rationale
                • Neurologic injury is the most common cause of death in patients with out-of-hospital cardiac arrest
              • Indications
                • Anyone not following commands or showing purposeful movements following resuscitation from cardiac arrest
              • Contraindication
                • Active non-compressible bleeding
                • TH is associated with higher risk of bleeding in patients undergoing coronary cath or those who received thrombolytics
              • Timing
                • To be achieved as soon as possible and maintained for at least 48 hours
                  • Rate of good functional outcome is higher with 48 hours rather than 24
                • Avoid fever at all costs in the first 48 hours
              • Goal temperature
                • This is an area of much debate amongst neurologists and intensivists.  There are two main goal temperatures:
                  • 33ºC (TH)
                    • The studies in support of cooling to a temperature of 33 come from two landmark NEJM papers published back in 2002 (HACA and OHCA) which found that mild to moderate hypothermia improved neurologic outcomes post cardiac arrest.  The caveats were that all these patients had VF/VT arrest (not PEA/asystole), no baseline brainstem function was reported before randomization, and the sample sizes were small. Based on these findings, TH is recommended for anyone with the following:
                      • Deep coma (loss of motor response or brainstem reflexes)
                      • Malignant EEG patterns
                      • Early CT changes suggesting development of cerebral edema
                    • Adverse effects:
                      • Increased rates of infection
                      • Coagulopathy and bleeding
                      • Cold diuresis
                      • Bradycardia and QT prolongation induced cardiac arrhythmias
                  • 36ºC (TTM)
                    • The TTM trial published in 2012 is the largest study on the topic that randomized 939 patients with out-of-hospital cardiac arrests to 33 or 36 degrees temp regulation and found no difference in all cause mortality or neurologic recovery between the two groups.  They included all patients regardless of type of arrest (VF/VT/asystole/PEA).  In subgroup analyses, they found no difference in outcomes based on type of initial rhythm, shock on presentation, age, gender, or time from cardiac arrest to ROSC.
                • So what to do?
                  • General consensus seems to be that avoiding fever at all cost in the first 48 hours is the most important intervention.  Beyond that, keeping patients at 36 might be better as further cooling is associated with risks and no clear indication of benefit based on our best available evidence.
            • General critical care
              • Elevate HOB to 30 degrees
              • Stress ulcer ppx
              • DVT ppx
              • Early PT/OT
              • Seizures and myoclonic jerks
                • Common and a marker of more severe brain injury
                • Continuous EEG recommended if available
                • No benefit in prophylactic treatment

Anoxic brain injury

Nomenclature

  • Brain death: irreversible cessation of cerebral and brainstem function
  • Persistent vegetative state: subgroup that suffers from severe anoxic brain injury and progresses to a state of wakefulness without awareness
  • Minimally conscious state: can have some purposeful movements or interactions with the environment.

Clinical parameters associated with an unfavorable prognosis

Clinical parameters Unfavorable prognosis
Duration of anoxia >8-10 minutes
Duration of CPR >30 minutes
Pupillary light reaction Absent on day 3
Motor response to pain Absent on day 3
Brainstem reflexes Absent
Blood glucose on admission >300 mg/dL
Glascow coma score on day 3 <5
Table above adapted from UpToDate

Work up:

  • Somatosensory evoked potentials (SSEPs): absent response at 24-72 hours appears to be most useful in identifying those with poor prognosis
  • EEG: can be affected by sedative drugs, metabolic derangements, and sepsis so careful with interpreting its findings!
  • Lab test for neuron-specific enolase (NSE)
    • Markedly elevated levels are associated with poor outcomes but no clear cut-off established
  • Imaging:
    • CT/MRI: look for edema and inversion of gray-white densities associated with poor outcome.

Emphysematous Cystitis Secondary to Proteus mirabilis 12/18/2018

Becky presented a case of a middle age man with NIDDM2, HTN, and history of phimosis s/p slit procedure 4 years prior, who presents with 3 months of dysuria, hematuria, urgency, frequency, and suprapubic pain. He was seen in the ED 2 months prior and his symptoms initially improved, but they gradually recurred until the pain was unbearable. Pt also started noticing bubbles in his urine, suspicious for pneumaturia. Given the amount of pain he was in, a CT AP was performed, which revealed a diagnosis of emphysematous cystitis!


Emphysematous UTI

Epidemiology

  • Rare, a few hundred case reports, one of the largest publication on current experience with this only has a sample size of 48.
  • Prior to 2006, 135 cases reported in the English literature

Risk Factors

  • Diabetes (main risk factor, median A1c > 9.9)
  • Elderly (Age > 60-70), women (2-6:1)
  • Immunocompromised
  • Neurogenic bladder
  • Obstructive uropathy (2nd most common risk factor)
  • Recurrent UTI

 

Presentation of Emphysematous Cystitis

  • Highly non-specific, presents similar to uncomplicated cystitis (dysuria, hematuria, abdominal pain, urgency/frequency. Pneumaturia, however, is unique to emphysematous cystitis.
  • Can progress rapidly, fatal if not recognized early on

Diagnosis

  • Abd/Pelvic imaging showing presence of gas in the bladder wall and/or lumen. CT has higher sensitivity.

Etiology

  • Infection
    • 2/3 cases = E.coli
    • ¼ Klebsiella
    • Rare = others, i.e. candida, clostridium, enterococci, staphylococcus, proteus
  • Vaginal fistula
  • Colovesical fistula (fecaluria might be seen)
  • Crohn’s disease
  • Malignancy of the colon or cancer
  • Instrumentation, obstruction, or trauma

Management

  • Early recognition and initiation of IV antibiotics, at least 10-14 days
  • Catheter drainage, bladder rest
  • Surgical debridement or cystectomy may be required for patients with poor response
  • 10% of patients required combined medical and surgical therapy.

Prognosis

  • Mortality up to 7-10% especially if not recognized early.
  • Early medical therapy decreases need for surgical intervention.

 

Presentation of Emphysematous Pyelonephritis

    • Critically ill, similar to complicated and severe pyelonephritis.
    • May be abrupt or develop over 2-3 weeks
    • 54% have concurrent bacteremia
  • Diagnosis/Prognosis: Based on CT scan findings
    • Class 1: Gas in the collecting system only
    • Class 2: Gas in the renal parenchyma without extension to the extrarenal space
    • Class 3A: Extension of gas or abscess to the perinephric space (between renal capsule and renal fascia)
    • Class 3B: Extension of gas or abscess to the pararenal space (between renal fascia and adjacent tissues)
    • Class 4: Bilateral involvement or one functional kidney with emphysematous pyelo
  • Management
    • IV antibiotics
    • Percutaneous Catheter drainage, bladder rest
    • Surgical debridement, nephrectomy
  • Prognosis
    • Mortality up to 25%, mainly in class 3 & 4 where incidence of thrombocytopenia, acute renal failure, encephalopathy, and septic shock.

This rare condition has been featured on NEJM Images in Clinical Medicine

Check out this article for more information on this condition.

Gradenigo Syndrome

Thanks to Amit for presenting the fascinating case of a middle-aged woman with history of DM2 who presented with subacute onset of unilateral periorbital pain, L CN 6 palsy, and L otorrhea, with MRI findings of petrous apicitis consistent with the super rare Gradenigo syndrome!


Clinical Pearls

  • Gradenigo syndrome is a rare and life threatening complication of otitis media and involves inflammation of the medial aspect of the temporal bone, specifically the apex of the petrous bone (a pyramid shaped bone jutting medially from the temporal bone)
  • Gradenigo is clinically characterized by a triad of otorrhea, diplopia (due to CN6 palsy), and hemifacial pain (CN5 palsy).
  • This is a very rare complication since most cases of otitis media are treated with antibiotics early on.
  • Remember that a common cause of an isolated CN 6 palsy in a diabetic patient is diabetic neuropathy/ophthalmoplegia.  A patient who has more cranial nerves affected than CN6 alone, you should be concerned about cavernous sinus thrombosis.

Gradenigo syndrome:

  • First described in 1904 by Guiseppe Gradenigo.

    Petrous pyramid
    Source: AO Surgery Reference
  • A rare and potentially life threatening complication of otitis media involving the inflammation of the apex of petrous pyramid (medial aspect of temporal bone). Occurs any time between 1 week to 3 months after acute otitis media (AOM) and up to 3 years after chronic suppurative otitis media (CSOM).
    • Should suspect this syndrome any time there is CN 6 palsy in the setting of otitis media, whether acute or chronic
  • Clinically, Gradenigo syndrome is characterized by triad of ear discharge, diplopia, and hemifacial pain
    • Suppurative otitis media (ear discharge and pain)
    • Trigeminal neuralgia involvement causes pain in the distribution of the nerve manifested as hemicranial headache and hemi-facial pain
    • Abducens nerve involvement causes ipsilateral lateral rectus palsy and lateral gaze palsy
  • Infection spread from suppurative otitis media to the petrous apex may be via pneumatized air cell tracts, through vascular channels, or as a result of direct extension through fascial planes
  • Organisms are not well studied but the most common one appears to be pseudomonas.  Staph, strep, pneumococcus, and TB have also been reported.
  • If left untreated, it can result in serious complications such as meningitis, intra-cranial abscess, sinus thrombosis
  • Treatment
    • Broad spectrum antibiotics IV for up to 6 weeks (to treat a presumed temporal bone osteomyelitis)
    • Fluoroquinolone ear drops
    • Tight glucose control
  • Differential diagnoses to consider:
    • Cavernous sinus thrombosis
      • Headache
      • cavernous sinus thrombosis
        Source: UpToDate

        Papilledema

      • CN palsies (see picture of what runs through cavernous sinus)
    • Ophthalmoplegic migraine:
      • Rare condition, often manifests in children and young adults
      • Diagnosis of exclusion
      • Most commonly affects CN3 (but can go to CN4 and CN6 as well)
      • Can sometimes precede the headache
      • Review article here
    • Diabetic ophthalmoplegia
      • Common cause of isolated CN6 palsy
    • Neoplasms
      • Nasopharyngeal cancer
      • Plasmacytoma
      • Pituitary adenoma
      • CN6 neuroma
      • Skull base tumors
      • Sohenoid sinus tumors
      • Squamous cell
    • Stroke
    • Demyelinating diseases
    • Vasculitis
    • Idiopathic intracranial hypertension

Complications of acute otitis media

  • Intratemporal
    • Tympanic membrane rupture (leads to hearing loss and pain relief!)
    • Labrynthitis (nausea, vomiting, tinnitus, vertigo)
    • Mastoiditis
    • CN palsies (including Gradenigo syndrome)
  • Extratemporal
    • Epidural, subdural, and brain abscesses
    • Skull base osteo
    • Otitic hydrocephalus (without meningitis or brain abscess)
    • Otitic meningitis
    • Lateral sinus thrombosis

Chylothorax Secondary to Follicular Lymphoma 12/13/2018

Tim presented a young man with no medical history presenting with a chronic cough with intermittent trace hemoptsis. Other than this cough and mild shortness of breath when he exerted himself, this pt had no other symptoms. A CXR revealed bilateral pleural effusion, and upon thoracentesis, milky fluid drained out with an elevated triglyceride content consistent with a chylothorax. Subsequent biopsy of a lymph node revealed a diagnosis of follicular lymphoma!


Since we are talking about pleural effusion, Light’s Criteria will inevitably come up. For both real life (and boards!) purposes, know this criteria really well!

Light Criteria:

  • SENSITIVE but NOT SPECIFIC for exudative effusions.

Capture

Any one of these criteria = exudative

  • Fluid protein/Serum protein > 0.5
  • Fluid LDH/Serum LDH > 0.6
  • Fluid LDH > 2/3 upper limit of normal of serum LDH

False positive is possible in certain settings:

  • Chronic diuretic use can falsely elevate fluid LDH (KNOW THIS)
  • Transudative effusion that’s been sitting there chronically can appear exudative like

The following tests can help us distinguish between a falsely positive exudative effusion from a true exudative effusion:

  • Pleural cholesterol > 45 mg/dL has high sensitivity and specificity for exudative effusions.
  • Can also use serum albumin – fluid albumin < 1.2g/dL to confirm exudative effusion

Pleural Fluid Analysis: Clues

Upon performing a thoracentesis, certain characteristics can potentially give us some clues to the etiology of the effusion…

Fluid Color

Capture2.JPG

Fluid WBC Count

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Fluid Predominant Myelocyte Type

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Lastly, how do you diagnose a chylothorax and what are some potential causes?

Chylothorax

  • Definition: Triglyceride > 110 mg/dL = slam dunk
    • 50 – 110: Less clear, cannot rule out, obtain liproprotein analysis. If presence of chylomicron is detected, likely chylothorax
    • < 50: Less likely
  • Non-traumatic
    • Malignant: Lymphomatous is most common, can also be other cancers i.e. lung, mediastinal mets, sarcoma, leukemia
    • Non-malignant: Idiopathic, benign tumors, protein losing enteropathy, thoracic aortic aneurysm, TB, Sarcoid, amyloidosis, thyroid goiter, tuberous sclerosis, congestive heart failure, mitral stenosis
  • Traumatic
    • Surgical is most common
    • External trauma
    • Trivial “trauma:” Stretching while yawning, coughing, hiccupping, sneezing (I’m not kidding)

Management of a malignant pleural effusion, as seen in this case, can be potentially challenging. After the patient was discharged, his pleural effusion on the right recurred within 3 days and completely filled up his right lung!

Several options are available for management of malignant pleural effusions. The decision is complicated and will goals of care discussion

  • Indwelling pleural catheter
    • Advantage: Pt managed, can drain at home
    • Disadvantage: Catheter related complications
  • Pleurodesis
    • Talc, slurry or poudrage, is the preferred agent. 60-90% success rate in reducing recurrence at 30 days.
    • Doxycycline can also be used but not as popular any more
    • Advantage: Eliminates the potential space for fluid reaccumulation
    • Disadvantage: Pain, potential for surgical failure, invasive
  • Combination: Talc + IPC
  • Pleurectomy

 

Leukocytoclastic vasculitis

Today, we talked about the case of a middle-aged man with history of diabetes, HTN, and A fib who presented with acute onset of progressive painful palpable purpura on his extremities, found to be cutaneous small vessel vasculitis on skin biopsy!


Clinical Pearls

  • Purpura implies problem at the level of vessel.  It can be divided into
    • Non-palpable purpura: petechiae (<3mm) or ecchymoses (>3 mm) and are usually associated with disorders of coagulation and platelets.
    • Palpable purpura: suggests inflammation and possible vasculitis.

Nomenclature:

  • Cutaneous small vessel vasculitis: disease limited to skin without any systemic vasculitis or glomerulonephritis
  • LCV: histopathologic term defining vasculitis of small vessels
  • Hypersensitivity vasculitis: small vessel necrotizing vasculitis
  • Immune complex small vessel vasculitis: associated with immune complex and/or complement deposition. If limited to skin, this is identical to cutaneous small vessel vasculitis. If not limited to skin, then other etiologies like cryo, SLE, Sjogren, RA, anti-GBM, IgA, etc.

Approach to purpura

Approach to purpura

  • Hypersensitivity (in the normal complement category of vasculitis) can result from medications/drugs as well as certain conditions such as HIV.
    • Numerous meds can cause LCV including some common ones such penicillins, cephalosporins, sulfonamides (including most loop and thiazide-type diuretics), phenytoin, and allopurinol have been most often implicated

Cutaneous small vessel vasculitis:

Clinical presentation:

  • Palpable purpura
  • + petechiae
  • Lesions can coalesce, ulcerate or be surrounded by hemorrhagic bullae
  • No visceral organ involvement in CSVV. However, it can occur later in the disease course.

Diagnosis:

  • Start with checking serum complement levels to guide your need for further laboratory work up!
  • Skin biopsy

Management and Prognosis:

  • Usually self limited and resolved within 2-4 weeks
  • If uncomplicated:
    • NSAIDs
    • Antihistamines
    • Rest, elevate, compression stockings
  • If complicated (presence of hemorrhagic blisters, cutaneous necrosis, or ulceration can lead to secondary infections, chronic wounds, and scarring)
    • Systemic glucocorticoids (oral steroids): pred 0.5 mg/kg of ideal body weight until new lesion formation ceases, then taper over 3-6 weeks
    • If relapse with prednisone: then colchicine or dapsone
    • If refractory: then azathioprine, methotrexate, and MMF

Example of palpable purpura with hemorrhagic blisters:

LCV skin example