All posts by cauriemma

Moffitt Pearls 10.16.17 – JIA and RA

Thank you to Faren for presenting a mini-case of a young woman presenting with subacute oligoarticular arthritis found to have severe erosive arthritis of the R wrist, elevated CCP and RF factor, consistent with oligoarticular JIA vs. RA, given unclear timeline of symptom onset.

Key Pearls

  1. The ddx for Oligoarticular arthirits is broad. Major categories include infection, crystalline disease, and Rheumatic disease. Check out this previous post for more details.
  2. The ddx of oligoarticular JIA includes Lyme disease, psoriatic arthritis, enthesitis-related arthritis (ERA), inflammatory bowel disease (IBD), pigmented villonodular synovitis, and malignancy, all of which may initially involve four or fewer joints.
  3. One should have a low threshold to treat STIs in high risk patients, especially when unsure if a patient will return to clinic for results. In this study to evaluate treatment compliance among asymptomatic adolescents (ages 14-21) with positive STI results, 27% of patients never filled their prescriptions for STI treatment! Consider single dose regimens for GC, Chlamydia, and trichomonas when concerned.

Think of Juvenile Idiopathic Arthritis as an umbrella term for several different categories of disease.

Oligoarticular JIA is the most common JIA category. It is more common in females than males, and the peak incidence is two to three years of age. The typical presentation is limping without complaint. The large joints (particularly knees and ankles) are most commonly affected, but the hips are virtually never the first involved joint. Systemic manifestations other than uveitis are characteristically absent.

Juvenile Idiopathic Arthritis (JIA) Sub-types:

  • Systemic JRA (formerly called Still’s disease) (10-15% of JIA cases): patients have rash and intermittent fever, in addition to arthritis of any number of joints.
  • Oligoarticular JRA (~50% cases): involvement of < 5 joints after 6 months of illness. This disease is now also broken down into several distinct subgroups with varying prognosis!
  • Polyarticular JRA (30-40% cases): involvement of >4 joints after six months of illness. As with oligoarticular JRA, it is now recognized to contain many distinct subgroups with varying prognosis.

Interestingly, annual optho screening is required for those with oligoarticular JIA given uveitis or iridocyclitis occurs in up to 25% of patients, often initially without symptoms.

Evernote: https://www.evernote.com/shard/s462/sh/4974b89d-0e90-4151-b6bc-3d72a5545186/40644d003ae2f96479188a75e520b3c8

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Moffitt Cardiology Pearls 10/10/17 – Congenital Heart Disease and SVC Syndrome

Thank you so much to Nick and Matt for presenting the case of a young man with a history of congenital pulmonary atresia and hypoplastic RV decades status post a bidirectional Glenn procedure coming in with shortness of breath and chest pain. The patient was found to have a severely dilated aortic root with resultant aortic insufficiency and severe pulmonary hypertension.

 

KEY PEARLS:

  1. When assessing a patient with congenital heart disease, it is VITAL to obtain records outlining the pathophysiology and previous procedures in order to understand their current physiology and what might go wrong.
  2. As Anne Thorson shared with us, in a patient with congenital heart disease who was previously doing well and then decompensates, ALWAYS consider the possibility of infection (particularly endocarditis) as the cause.
  3. SVC syndrome is a clinical diagnosis – see some physical exam features below.

    Cyanotic Congenital Heart Lesions: There are 5 main lesions, often called the “5 Ts”, of congenital heart defects that can result in cyanosis at birth or in the neonatal period. Most of these conditions will require intervention within the first months of life.

     

    Here’s a review on cyanotic congenital heart disease in adults. Of note, it’s from 1975… my guess is many things have changed since then and we see significantly more adult survivors of congenital disease.

Pulmonary atresia and hypoplastic RV is NOT typically a cyanotic heart lesion. However, the small RV + atretic pulmonic valve can lead to a volume and pressure overloaded state on the right side the heart. The Bidirectional Glenn Procedure reduces blood flow to the hypoplastic RV (thus offloading the pressures) by diverting the SVC directly into the pulmonary arteries. Blood return from the IVC continues to enter into the RA.

 


While we most commonly see SVC Syndrome in the setting of extrinsic compression or internal invasion of the SVC in malignancy, remember that there are multiple other risk factors as well!

Risk factors for SVC syndrome:

  • Thoracic mass – lung cancer, lymphadenopathy, lymphoma, teratoma, thymoma
  • Vascular disease – aortic aneurysm, vasculitis, AV fistula
  • Scarring or Fibrosis – related to chronic infection (like histo as Harry mentioned or tuberculous mediastinitis as described by Schechter in 1954), radiation, or instrumentation
  • Cardiac causes – pericarditis, atrial myxoma OR, as in this case, severe pulmonary hypertension in patient whose SVC connects directly to the pulmonary veins!
  • Clot – with or without underlying malignancy or central venous catheter

Here’s another, more recent, review of SVC syndrome, describing the pathology, risk factors, diagnosis, and management.

Harry mentioned Pemberton’s sign, described in the linked NEJM images in Clinical Medicine article. Below are 2 YouTube videos showing the classic finding:  signs of SVC syndrome brought on by extending the arms over the head. The second is quite dramatic.

1) https://www.youtube.com/watch?v=r1dkasbE7v8

2) https://www.youtube.com/watch?v=m_ZecWGnb2A

 

Evernote: https://www.evernote.com/shard/s462/sh/d5befae6-e090-4f3e-ab15-7dfa8ed3e73a/6e88cb495f580e320334ed1c2932afb5

Moffitt Pearls 10/9 – Polyarthritis

Key Pearls:
1)  There are a number of ways to approach arthritis including time course (acute vs. chronic), # of joints affected (mono vs. oligo vs. poly), size of joints affected (small vs. large), and symmetry.  See below for more about the DDx based on # of joints affected.
2) Arthritis vs. arthralgia?  Look for evidence of articular inflammation on the physical exam!  The key finding is synovitis.
3) The positive LR for synovial fluid WBCs >100K is 28 for septic arthritis! This was published by some of our very own (see below)!
4)  The seronegative spondyloarthropathies consist of ankylosing spondylitis, IBD-associated arthritis, psoriatic arthritis, reactive arthritis, and undifferentiated spondyloarthritis.

Check out this fantastic post from Grant Smith on Septic Arthritis and Reactive Arthritis!

 
Approach to Arthritis by # of Joints affected:
 

Monoarthritis (1 joint affected):

  • Infection (Staph, GC can be either mono or poly)
  • Crystal Arthropathy (gout, CPPD)
  • Rheum disease (RA, SLE, sarcoid, spondy)
  • Inflammatory osteoarthritis
  • Trauma/Hemarthrosis
  • Tumor (giant cell tumor, sarcoma, metastasis)

Oligoarthritis (definition varies:  at least 2 and up to 3 to 5) and Polyarthritis (at least 4):

  • Infection (GC, Lyme, endocarditis, viral)
  • Crystalline
  • Rheum disease (RA, SLE, sarcoid, ankylosing spondylitis, psoriatic, IBD, poly/dermatomyositis, vasculitis)
  • Inflammatory osteoarthritis
  • Post-infectious/reactive
  • Other systemic disease: FMF, malignancy

Migratory Arthritis:

  • Rhematic fever
  • Infective endocarditis
  • HSP
  • Serum sickness
  • Viral arthritis
  • Septicemia (staph, strep, mening/gonococcal)
  • Pulmonary infection: mycoplasma, histoplasmosis
  • Lyme disease
Discriminating Factor
Possible Diagnoses
Temperature >40.0 C
Still’s disease, bacterial arthritis, SLE
Fever preceding arthritis
viral arthritis, lyme disease, reactive arthritis, Still’s disease, endocarditis
Migratory arthritis
Rheumatic fever, gonococcus, meningococcus, viral arthritis, SLE, acute leukemia, Whipple’s disease
Effusion disproportionate to pain
TB arthritis, endocarditis, IBD, GCA, lyme disease
Pain disproportionate to effusion
Rheumatic fever, FMF, acute leukemia, AIDS
Positive RF
RA, viral arthritis, TB arthritis, endocarditis, SLE, sarcoidosis, systemic vasculitis
Morning stiffness
RA, PMR, Still’s disease, some viral and reactive arthritis
Symmetric small joint
RA, SLE, viral arthritis
Leukocytosis (>15K)
Bacterial arthritis, endocarditis, Still’s disease, systemic vasculitis, acute leukemia
Leukopenia
SLE, viral arthritis
Episodic recurrences
Lyme disease, crystal-induced arthropathy, IBD, Whipple’s disease, FMF, Still’s disease, SLE

From the JAMA Rational Clinical Exam 2007 Article, “Does This Adult Patient Have Septic Arthritis?”, by our very own Mary Margaretten and Jeff Kohlwes:
  • Summary LRs for Synovial Fluid studies:
    WBCs >100K 28
    WBCs >50K 7.7
    WBCs >25K 2.9
    PMNs > 90% 3.4

Seronegative Spondyloarthropathies:  Group of conditions linked by common clinical features and immunopathologic mechanisms. Distinguished from one another usually on basis of history and clinical findings.  “Seronegative” implies RF negativity.  All subtypes involve  the axial skeleton.  Extra-axial involvement, such as uveitis, calcaneal enthesitis or peripheral arthritis can occur in all subtypes.

  • Ankylosing spondylitis – 90% HLAB27 +
  • Psoriatic arthritis – 60% HLAB27 +.
  • Reactive arthritis – 85% HLAB27 +
  • IBD-associated or Enteropathic arthritis
  • Undifferentiated spondyloarthritis – no definite radiologic signs of sacroiliitis

Moffitt Pearls 10.6.17 – Thrombocytopenia, TTP, HUS

Thank you, Marko and Brad, for presenting a fascinating case of an older male presenting with fatigue and jaundice found to have profound thrombocytopenia, anemia, and AKI concerning for TTP!

Key Pearls

  1. The approach to thrombocytopenia can be organized by decreased production, increased consumption, and sequestration.
  2. TMA vs MAHA? What’s the difference again?? Microangiopathic hemolytic anemia refers to non-immune hemolysis (i.e. Coombs-negative hemolysis). As a result, you see schistocytes on blood smear. Thrombotic microangiopathy is a specific pathologic abnormality in the walls of arterioles and capillaries that lead to microvascular thrombosis. Not all MAHAs are a result of a TMA, but nearly all TMAs will cause MAHAs.
  3. There’s a new scoring system used to predict TTP – The PLASMIC score – you can use this to help determine when to start plasma exchange in a patient while you’re waiting for the ADAMTS13 to return.

Approach to Thrombocytopenia:

  • Decreased Production
    • Bone marrow process – can further guide ddx based on bone marrow biopsy
      • Hypocellular – aplastic anemia, MDS, drugs, alcohol, cirrhosis
      • Hypercellular – MDS, leukemia, megaloblastic anemia
      • Marrow Replacement – myelofibrosis, malignancy, granulomatous disease
    • Megakaryocyte process – most commonly EtOH use, as alcohol is a direct megakaryocyte toxin. Usually won’t get lower than ~100 without a secondary process as well.
  • Increased Destruction or Consumption
    • Immune-mediated
      • Primary: ITP
      • Secondary: infection, lupus, APS, lymphoproliferative disease, drugs (heparin!, antibiotics, H2 blockers), alloimmune
    • Non-Immune-mediated
      • MAHA, Plavix, vasculitis, HELLP, CVVH
  • Sequestration – splenomegaly – often from portal HTN

 


Approach to a Suspected TMA (from UpToDate):




The PLASMIC score

Published in the Lancet Haematology earlier this year, the PLASMIC score is a model that uses 5 independent variables to predict ADAMTS13 deficiency.  This model was developed in a cohort of 214 patients with thrombotic microangiography, of which 29% had TTP.  It was subsequently validated in 2 different cohorts.

  • Plts <30
  • Cr <2
  • INR < 1.5
  • MCV <90
  • Presence of hemolysis

Citations:

  1. Bendapudi PK, Hurwitz S, Fry A, Marques MB, Waldo SW, Li A, Sun L, Upadhyay V, Hamdan A, Brunner AM, Gansner JM, Viswanathan S, Kaufman RM, Uhl L, Stowell CP, Dzik WH, Makar RS. Derivation and external validation of the PLASMIC score for rapid assessment of adults with thrombotic microangiopathies: a cohort study. Lancet Haematol 2017.
  2. Jamme M, Rondeau E. The PLASMIC score for thrombotic thrombocytopenic purpura. Lancet Haematol 2017.

Evernote: https://www.evernote.com/shard/s462/sh/ea34eab3-06e4-4541-8664-b508bf3ab3ee/f0669d43862b43e7f513d48b1324aa8e

 

Moffitt Pearls 10.4.17 – Massive Hemoptysis

Thank you, Monica, for presenting a case of an elderly male transferred from an OSH for SOB and hemoptysis found to have severe bronchiectasis and imaging evidence of a prior pulmonary artery embolization.

Key Pearls

  1. Review on the approach to hemoptysis: Truly hemoptysis? How much blood? Where is it coming from?
  2. Another approach to hemoptysis can be based on imaging – are the findings focal or diffuse?
  3. A rare and forgotten cause of hemoptysis in patient’s with prior TB infection is Rasmussen’s aneurysm.

Initial Approach to Hemoptysis:

    1. Is it really hemoptysis? Easy to confuse with hematemesis, nasopharyngeal bleeding.
    2. How much bleeding is there? Massive is > 500cc in a 24 hour period
    3. Where is the bleeding coming from?
      • Airways
      • Parenchyma
      • Vasculature
      • Coagulation disorder
      • Trauma

 


Approach to Hemoptysis Based on Imaging:

  • Are the abnormalities Diffuse vs. Focal?
    • Diffuse findings are suggestive of diffuse processes, such as DAH, bleeding from anticoagulation, vasculitis, infection)
    • Focal findings suggest a malignancy, focal infection (necrotizing pneumonia, aspergilloma, TB cavity)

Management of Massive Hemoptysis: ABCs + Ps

  • ABCs first! Is the patient able to protect their airway? Consider early intubation if concerned.
  • Positioning: bad lung down –> try to prevent the active bleeding from causing impaired oxygenation in the good lung fields
  • Prepare: Hemorrhagic shock from hemoptysis is rare but it can happen. Make sure you have adequate access, active type and cross, and initiate transfusions when needed.
  • Pills (i.e. medications – not actually pills though…): Reverse any anticoagulation, consider use of tranexamic acid (anti-fibrinolytic agent)
    • check out this review on tranexamic acid for hemoptysis! Bottom-line – probably effective, but more studies needed!
  • Procedure: Call you consultants.
    • Remember, that while bronchoscopy is the procedure of choice to diagnose diffuse alveolar hemorrhage, the therapeutic options from a bronch are VERY limited.
    • Call IR early for pulmonary artery embolization.
    • Thoracic surgery may be helpful in the setting of severe bleeding that won’t be amenable to one or a few IR interventions.

Thanks, Vaibhav for telling us about Rasmussen’s aneurysm, a source of bleeding as a sequelae of past pulmonary TB infection.  This results from aneurysmal dilatation of a pulmonary artery adjacent to or within a tuberculous cavity.  Check out this case report on this “rare and forgotten” cause of hemoptysis!


Evernote: https://www.evernote.com/shard/s462/sh/f0a06626-3098-46cd-8b42-c93f5fa2f523/752bb3039a25c4a4ec6512e47e10a032

Moffitt Pearls 9/26/17 – hemoptysis & pulm abscess

Key Pearls:
1)  A simple approach to hemoptysis includes (1) ruling out mimics, (2) assessing the volume, and (3) considering source of bleeding.  See below for more details!
2)  Management of pulmonary abscess is a little counter-intuitive – unlike abscesses in other parts of the body – we DO NOT typically drain them. Instead, patient’s get LONG courses of antibiotics with polymicrobial (especially anaerobic) coverage.
3)  No teeth?  No pulmonary abscess.  Periodontal disease serves as the nidus for bacterial growth leading to pulmonary abscesses. If a patient has a mass or an abscess and is edentulous, your suspicion for cancer should be way up!

Hemoptysis Framework (adapted from a previous post from Lekshmi Santhosh!)
Step 1: Rule-out hemoptysis mimics.
  • Could this be nasopharyngeal bleeding?  Could this be hematemesis?
Step 2: How much bleeding is there?
  • Massive  Hemoptysis = >500 cc blood in 24 hours
Step 3: Where is the blood coming from?  Here’s were you could consider a mnemonic, but sometimes a structural approach might be easier
·      AIRWAY DISEASES – Airway trauma, bronchiectasis, foreign body, malignancy
·      PARENCHYMAL DISEASES – Mostly infection – lung abscess, TB, vasculitis
·      VASCULAR DISEASES – PE, mitral stenosis, congenital heart disease
·      COAGULATION DISEASES – Severe thrombocytopenia, anticoagulant meds
·      TRAUMA – Iatrogenic post-procedure or post-traumatic (including cocaine use)
 
Approach to DAH
1.      Vasculitis: Ddx includes MPA, GPA, and Churg-Strauss
2.      Pulmonary-Renal Syndrome: Goodpasture’s, connective tissue dz, IgA nephropathy
3.      Autoimmune diseases: SLE & APLS
4.      Medications: Complications of anticoagulation, drug-induced thrombocytopenia
5.      Idiopathic pulmonary hemosiderosis (or in women, consider pulmonary endometriosis)

Pulmonary abscess clinical pearls (from a previous post from Anna Parks!)

  • The illness script for pulmonary abscess is a patient who presents with indolent symptoms (weeks-months) including fever, night sweats, weight loss, cough, sputum production.
  • The vast majority of lung abscesses are caused by aspiration of anaerobes present in gingival crevices.
    • Thus the need for teeth!
    • Other more rare causes (that one might consider in an edentulous patient!) include bronchial obstruction (e.g. from neoplasm) or septic emboli (e.g. from Lemeirre’s disease or tricuspid valve endocarditis)
  • Predisposing factors for aspiration:
    • altered level of consciousness (EtOH, drug abuse, anesthesia, head trauma)
    • dysphagia
  • Consider smelling your patient’s sputum (!), as “putrid” odor is considered diagnostic of anaerobic bacterial infection
  • Clinical response can be sluggish– fevers regularly persist for 3-4 days and can last even up to 7-10 days.
  • Tons of prior posts on lung abscesses that are searchable on the blog!

Moffitt Pearls 9.13.17 – GI Morning Report

Thank you, John and all of Team C, for presenting a case of a middle-aged woman with a poorly characterized connective tissue disorder, pHTN, AS and CKD who presented with melenic stools.

Key Pearls

  • A practical approach to UGIB is dividing etiologies into variceal vs. non-variceal. This is an important early distinction given the increased risk of life-threatening bleeding and subsequent management for variceal bleeds compared to other etiologies.
  • While BRBPR generally suggests LGIB, if you see it in a patient with hemodynamic compromise, you should suspect a brisk upper source.
  • GAVE – gastric-antral vascular ectasia – “watermelon stomach” – think of this as a portal hypertension equivalent with regards to bleeding risk. It’s associated with both cirrhosis and systemic sclerosis.

Public Service Announcement:  Melanotic?  Melenic? If you’re talking about relation to melanoma, use “melanotic”. If you’re talking about blood in the stool, the word is melenic. J


A quick comment on the controversy of NGT for risk stratification of UGIB:

From a 2012 JAMA Rational Clinical Exam Series:

“Gastric Contents

Nasogastric lavage provides a method for sampling contents from the stomach. Although the routine use of nasogastric lavage in patients with suspected GIB remains controversial,14,15 it is frequently used in the United States and Canada. A study performed in Los Angeles revealed that 60% (n = 632) of patients underwent nasogastric lavage for UGIB,16 and 28% (n = 1869) of patients underwent the procedure in a study from Canada.17 A recent survey of gastroenterologists’ opinions about the role of nasogastric lavage for UGIB showed they were uncertain of its appropriateness (quantified with the RAND appropriateness scale) and also showed “extreme variation” (quantified with a disagreement index) in their opinions about its role.18 Some clinicians consider the presence of varices a relative contraindication for the use of nasogastric lavage. However, there are no published trials to suggest that nasogastric lavage worsens bleeding19 in patients with or without varices.”


Bonus from the Ditty:  Stages of Syphilis

Clinical Manifestations

1st-line Treatment

Early
  • Primary chancre
  • Secondary systemic illness w/various cutaneous findings
  • Early latent + serology but no symptoms; known to be negative within past year

Penicillin G benzathine 2.4 million units IM x1

Late
  • Tertiary symptomatic disease with cardiovascular involvement or gummas
  • Late latent + serology but no symptoms; >=12 months since last negative test OR unknown when last negative

Penicillin G benzathine 2.4 million units IM qWeek x3

Neuro
  • Early meningitis, hearing loss, vision loss
  • Late dementia (general paresis), tabes dorsalis

Aqueous penicillin G 3 to 4 million units IV q4h for 10-14d

Evernote: https://www.evernote.com/shard/s462/sh/a957b2fe-0af8-44bc-a193-4904242c8dbe/ae34ca0525da4271dba461023baab313