Category Archives: Rheumatology

VA Ambulatory Report 2.21.18 – Sarcoid Diagnosis and Hematuria

We had two great case discussions today.

First Jeff presented an image of incidentally discovered pulmonary nodules and hilar lymphadenopathy.  The patient was diagnosed with sarcoid based on these imaging results alone.

Learning points:

  • How to diagnosis sarcoid:
    • If a patient is asymptomatic with characteristic radiographic findings – this is sufficient to make the diagnosis
    • For symptomatic patients, biopsy confirming noncaseating granulomas is required
  • For a new diagnosis of sarcoid monitor for complications and extrapulmonary involvement: EKG, eye symptoms, vitamin d levels, UA
  • In an asymptomatic patient with low grade radiologic changes, the management is observation only

 

Second, Akshai presented a patient seen in clinic the day prior with gross hematuria.

Learning points:

  • Gross hematuria should strongly increase your suspicion of malignancy. The incidence of malignancy in microscopic hematuria vs. gross hematuria  is ~2% vs. 20%.
  • Smokers are 4-7 times more likely to develop bladder cancer than non-smokers
  • CT Urography is the test of choice to evaluate for malignancy and nephrolithiasis.  CTU provides both functional and anatomic information about the kidney and ureter.  The pre-contrast phase evaluates for nephrolithiasis and hydronephrosis.  The post contrast phase evaluates renal and urothelial malignancies and can assess kidney function in the setting of obstruction.

 

Evernote link: https://www.evernote.com/l/AMqIYrPCjnpO-r9H6V0dfZj9zk4E5gV0uRw

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VA Ambulatory Report 1.10.18 – Aches and Pains all over – PMR and RA

Thank you Monica for presenting the case of an elderly gentleman presenting with chronic fatigue, muscle aches, wrist and hand joint pain, and weight loss found to have PMR and RA.

Learning Peals  Screen Shot 2017-10-11 at 12.58.11 PM

  1. LT’s pearl: Many patients with PMR present with acute onset of symptoms
  2. Other lab findings that may be present in patients with PMR: mild normocytic anemia, elevated alk phos (although more common in patients with GCA)
  3. Patients with PMR only need temporal artery biopsy if they have symptoms of arteritis. About 5-30% of patients with PMR will have GCA

 

Polymyalgia Rheumatica Refresher

  • Clinical Manifestations
    • Proximal pain and morning stiffness
      • Shoulder pain is the most common symptom
      • LT’s pearl: Many patients report acute onset of symptoms
      • Distal symptoms occur in ~50% of patients including peripheral arthritis, carpal tunnel, pitting edema of hands and feet)
    • Systemic symptoms (fever, weight loss, malaise) are present in ~1/3 of patients
    • Laboratory Findings
      • Elevated inflammatory markers
      • Non specific findings (not present in all patients)
        • Mild normocytic anemia
        • Elevated Alk Phos – although more common in patients with GCA
  • Diagnosis
    • No other systemic disease to explain symptoms
    • Onset of symptoms after age 50
    • Proximal aching and morning stiffness lasting >30 minutes for at least 2 weeks
      • LT’s pearl: Many patients report acute onset of symptoms
      • Shoulder pain is the most common presenting symptom
    • ESR > 40
    • Rapid resolution of symptoms with prednisone
      • Lack of response strongly suggests another diagnosis
    • There are no set diagnostic criteria, but the ACR/EULAR have proposed criteria to be used in research studies but do not recommend use for diagnosis in individual patients
    • Atypical PMR
      • Asymmetric symptoms
      • Low ESR
        • Some patients have normal or mildly elevated ESR (7-20% at time of diagnosis)
          • Check CRP
          • If both ESR and CRP are normal, much less likely to be PMR
  • Does my patient with PMR have GCA?
    • 50% of patients with GCA have PMR. Of those with PMR, 5-30% get GCA
    • Clinical manifestations of GCA
    • Only need to get temporal artery biopsy if the patient is having symptoms of temporal arteritis
  • Treatment
    • Goal of therapy: Improve symptoms
    • Starting dose: 15-20 mg prednisone per day
    • After a period of quiescence (2-4 weeks) then start a slow taper by 10-20% every 2-4 weeks
    • 50% of patients will have recrudesce of their symptoms and need re-treatment with steroids or increase in their steroids

Evernote Link: https://www.evernote.com/l/AMr2DzbkfG9K56vgSepfENvXq9AhGchMp04

Moffitt Pearls 10/30/17 – Palpable Purpura + AKI

Thank you, Lily for presenting a case this morning of a middle-aged woman from Mexico being treated for a metastatic GI malignancy p/w flank pain, AKI and palpable purpura found to have obstructive nephropathy and candiduria .

Key Pearls:

  1. Classic approach to AKI: pre-, intrinsic-, post-renal hold-up even when the patient becomes more complicated. Always remember to consider carefully a patient’s volume status and run the med list.
  2. The DDx for purpura can be broken down first by whether or not the lesions are palpable. If NOT palpable, we call these petechiae or ecchymoses based on the size (<3mm = petechiae). This should prompt consideration of the coagulation cascade, platelets, uremia. If palpable, this suggests inflammation and a possible vasculitis. See an approach to palpable purpura below.
  3. Yeast in urine is usually a colonizer and not a pathogen. However, treatment for funguria is indicated in patients with renal transplant and immunosuppression. See link for more info http://www.sjkdt.org/article.asp?issn=1319-
Candiduria: A Review of Clinical Significance and Management – Zakeya Abdulbaqi Bukhary

Selected Highlights:

  • Treatment of Candida can be guided by in vitro susceptibility testing (although all labs do not send these).
  • Fluconazole gains high concentrations of active drug in the urine, is better tolerated and is less likely to be associated with the emergence of resistance during therapy.
  • The greatest concern for fluconazole resis­tance is related to C. glabrata and C. krusei isolates which require maximal doses of amphotericin B.
  • As HH mentioned – Echi­nocandin anti fungal agents (caspofungin, micafungin, and anidulafungin) can be used although low sub-therapeutic levels are achieved in the urine because the drug has poor glomerular filtration with subsequent diminished tubular secretion. 

More Info at:   http://www.sjkdt.org/article.asp?issn=1319-

purpura

Causes of Non-Palpable Purpura based on size of lesion

  • Remember these lesions are macular and are typically NOT inflammatory
  • Petechiae (small lesions < 3 mm)
    • Abnormal palatele function
    • Increased intravascular pressures
    • Thrombocytopenia
      • Idiopathic
      • Drug-induced
      • Thrombotic
    • DIC and infection
  • Ecchymoses (larger lesions > 5 mm)
    • Coagulation defects
    • DIC and infection (purpura fulminas)
    • External trauma
    • Hypergammaglobulinemic purpura

See the following link for more information in a case based ppt from the American Academy of Dermatology Titled “Petechiae, Purpura and Vasculitis.”

Break down Vasculitis based on vessel size

vasculitis

 

Moffitt Pearls 10.24.17 – Cardiology Report – SLE and Tamponade

Thank you to Laura for presenting a fascinating case of a middle aged woman with a hx of SLE c/b APS presenting with progressive dyspnea and CP. Her Physical exam was notable for an elevated JVP and muffled heart sounds and negative pulses paradoxes (initially). Her INR was 10, she was thought to have a mild SLE flar and she was found to have a large, progressive hemorrhagic pericardial effusion resulting in tamponade requiring urgent pericardial drainage.

Key Pearls

  1. Think about tamponade in a patient who presents with hypotension, tachycardia and elevated neck veins.
  2. Tamponade is a CLINICAL diagnosis with the BECK’s triad: 1) elevated JVP 2) muffled heart sounds 3) hypotension with pulses paradox.
  3. Echocardiographic signs suggestive of tamponade include 1) presence of pericardial effusion 2) diastolic collapse of the RV or RA and 3) IVC dilation. See link for amazing videos. (https://web.stanford.edu/group/ccm_echocardio/cgi-bin/mediawiki/index.php/Tamponade)
  4. Remember that there are several drug-drug interactions with warfarin. As we saw in this case this patient’s INR rose to 10 after she was treated with levofloxacin!!

Cardiac Tamponade

Definition: Hypotension that immediately reverses with pericardial drainage

Physiology:

  • Pericardial effusion of significant volume OR rapidly accumulated leads to increased pressure in pericardial space throughout the cardiac cycle
  • During inspiration, as RV volume increases, the RV is unable to expand into the  left, decreasing LVEDV and thereby decreasing cardiac output, causing a decrease in SBP during inspiration maximally stretched pericardium. Therefore, the interventricular septum bulges to left, decreasing LVEDV and thereby decreasing cardiac output, causing adecrease in SBP during inspiration
  • Diastolic equalization of pressures
  • Acute vs. subacute tamponade: small volumes at fast rates vs. abilility for pericardial stretch if fluid gradually accumulates
  • In both acute and subacute, there is a point at which intrapericardial pressure decompensation reaches an almost vertical ascent with a small amount of fluid -> acute

 

Etiologies:

  • Acute tamponade:
    • Usually due to traumatic rupture of ventricle as a result of a procedure or blunt trauma; also in aortic dissection or myocardial infarction with ventricular rupture; hemorrhage as in our case
  • Subacute tamponade:
    • Infection: More commonly purulent than viral
    • Malignancy: Particularly lung, breast, Hodgkin’s, mesothelioma
    • Post-MI, post-CT surgery, post-procedure
    • Uremia
    • Post-XRT
    • Drugs
    • Collagen-vascular disease: in particular SLE
    • Idiopathic
    • HIV
    • External to pericardial sac (pleural effusions have caused tamponade physiology)

 

Treatment:

    • IV fluids to temporize (sometimes brings out tamponade physiology and physical
    • signs)
    • Pericardiocentesis: paraxiphoid (left), needle at 15 degree angle to skin, toward left
    • shoulder, with patient sitting forward
    • Pericardial drain or window

VA Ambulatory Report – Polyarthritis and Drug Induced Lupus

Case Summary: Thank you Alicia for presenting your clinic patient a 20 yo F with refractory epilepsy presenting with subacute migratory polyarthritis after starting Oxcarbazepine concerning for Drug-Induced Lupus on top of SLE.

 

PearlsScreen Shot 2017-10-11 at 12.58.11 PM.png

  • Patients with drug-resistant epilepsy should be seen at a specialized epilepsy center
  • Thanks Chris Sha for teaching us the top three causes of infectious polyarthritis: Disseminated Gonococcal disease, spirochetes, and viral (HIV, HBV)
  • Approach to arthritis: Consider basing it on number of joints and inflammatory vs. non-inflammatory
  • Differentiating drug-induced lupus from SLE is based on clinical picture and  laboratory findings.
  • Anti-TNF drugs are increasing in use and can cause an atypical presentation of drug induced lupus.

 

Drug-resistant epilepsy

  • No set definition, but has been proposed that drug-resistant epilepsy may be defined as failure of adequate trials of two antiseizure drug schedules (whether as monotherapies or in combination) to achieve sustained seizure freedom
  • Other forms of treatment may be considered; vagal nerve stimulation, surgery
  • If seizures are not controlled after 12 months, the patient should be referred to a specialized epilepsy center
  • Ambulatory EEG monitoring?
    • More cost effective than in-hospital EEG. Can increase the yield of detecting a seizure due to longer time on monitors and patient’s are less sleep deprived when sleeping in their normal environment.
    • Gold standard for differentiating non-epilieptic seizures from epileptic seizures is still in-patient EEG.

 

Flashback to Katie Auriemma’s post on an approach to polyarthritis from the Moffitt Pearls earlier this week.

Drug-induced lupus (DIL)

  • Common meds: procanamide, hydralazine, penicillamine, INH
    • Also seen with TNF-alpha inibitors
      • Can be more atypically presentation of DIL including negative anti-histone antibodies, hypocomplementemia positive dsDNA antibodies
  • More often causes superficial lupus symptoms (joint pain, rash) over visceral involvement (anemia, nephritis, serositis, etc)
  • Diagnosis
    • History of taking a known offending medication
    • Development of one feature of lupus
    • Serology: Positive ANA.
      • Positive anti-histone antibody is strongly suggestive.
        • Can be seen in idiopathic SLE but those patients will also have positive subserologies
      • You can also see a positive ANCA
    • Resolution of symptoms within weeks of stopping the offending agent

 

How do I differentiate between SLE and DIL? 

  SLE DIL
Clinical presentation Average age of onset 20 -30 y

More likely to affect African Americans than Caucasians

Female: Male 9:1

Average age of onset 50 -70 y

More likely to affect Caucasians than African Americans

Female: Male 1:1

Lab Findings ANA positive > 95%

Anti-histone antibodies in 50%

Anti-dsDNA in 80%

Low C3/ C4

 

ANA positive in > 95%

Anti-histone antibodies in > 95%

Anti-dsDNA can be present

Normal C3/C4

 

VA Ambulatory Report 8.23.17 Polyarthritis

Thanks Hailyn for presenting this great case of a 40 yo female with a previous diagnosis of seronegative rheumatoid arthritis who presented to rheumatology clinic with new rash and ankle pain ultimately diagnosed with lupus.

Key learning points:

  • Use your physical exam: findings of synovitis can help narrow your differential to inflammatory causes of joint pain.  Nail changes such as pitting, splinter hemorrhages, and vascular irregularities are seen in several rheumatologic conditions.
  • About a quarter of patients with chikungunya will develop chronic polyarthritis
  • Drug induced lupus most often has superficial symptoms of skin and joint findings without visceral involvement.
  • ESR and CRP are indirect markers of inflammation but do not always directly correlate.  Lupus patients often have an elevated ESR and normal CRP due to the production of interferons that inhibit hepatocyte production of CRP.

 

What is the Seronegative rheumatoid arthritis?

  • Thanks Goop for pointing out the different terminologies used.   Some people use the phrase seronegative arthritis to describe a rheumatologic cause of arthritis that has not yet been determined.  Other names include arthritis NOS and palindromic arthritis.  Patients with these diagnoses may go on to develop a clear rheumatologic diagnosis such as rheumatoid arthritis or lupus.  Time (in addition to the appropriate serologies) is often a helpful diagnostic tool.

 

Characterizing joint pain: Think about the time course of symptoms, the number of joints involved, the asymmetry and size of involved joints, and if there is evidence of inflammation.

The rheumatology physical exam – synovitis and nail findings 

  • Synovitis
    • How do I assess for synovitis on physical exam?
      • Look for soft tissue swelling, warmth, joint effusion, and decreased range of motion
      • Joints with active synovitis feel boggy and cause lss ability to feel the bone prominences of the joint
    • Synovitis on physical exam suggests an inflammatory cause of joint pain and can help narrow your differential
  • Nail findings
    • Splinter hemorrhages: seen in endocarditis, scleroderma, psoriasis, RA
    • Pitting: Seen in psoriasis
    • Vascular changes
      • Get out that ophthalmoscope (or otoscope) and use some lubricant to magnify the vasculature bed of the nails
      • The vasculature of the nail bed normally is orderly and parallel
      • Patients with rheumatoid arthritis, SLE, dermatomyositis, or scleroderma the vasculature may be irregular, twisted, and dilated
    • Other resources for the physical of exam of the nails
      • The Standford 25:  http://stanfordmedicine25.stanford.edu/the25/hand.html#linseys
      • AAFP: http://www.aafp.org/afp/2004/0315/p1417.html

Chikungunya: In addition to the acute illness of fever, malaise, and polyarthralgia about 25-35% of patients develop a chronic inflammatory polyarthritis.

Drug induced lupus

  • Common meds: procanamide, hydralazine, penicillamine, INH
    • Also seen with TNF-alpha inibitors which are often used to treat RA and can cloud the clinical picture in a patient with an unknown rheumatologic disease such as this patient
  • More often causes superficial lupus symptoms (joint pain, rash) over visceral involvement (anemia, nephritis, serositis, etc)
  • Diagnosis
    • History of taking a known offending medication
    • Development of one feature of lupus
    • Serology: Positive ANA.  Positive anti-histone antibody is strongly suggestive. You can also see a positive ANCA
    • Resolution of symptoms within weeks of stopping the offending agent

 

What is the difference between ESR and CRP?

  • ESR
    • Measures the rate at which erythrocytes suspended in plasma settle in a test tube
    • An indirect measure of acute phase response due to increases in fibrinogen and immunoglobulins that then affect the sedimentation rate. However, many other things besides inflammation can affect the sedimentation rate.
    • Most often elevated due to inflammation (rheumatologic conditions, infections, malignancy, tissue injury) but there are several non-inflammatory causes of elevated ESR including:
      • Age
      • Female sex
      • Anemia
      • Renal disease
      • Obesity
      • Paraproteinemia
    • Causes of decreased ESR
      • Abnormalities in erythrocyte morphology
      • Extreme leukocytosis
      • Heart failure
      • Hypofibrinogenemia
  • CRP
    • Produced by the liver in response to IL-6 and other cytokines
    • Rises and decreases more rapidly than ESR
    • CRP is also a sign of inflammation and when markedly elevated if often an indication of infection or systemic inflammatory diseases
    • Mild elevations in CRP can be a sign of low grade inflammation  due to conditions such as atherosclerosis, obesity, OSA, insulin resistance, HTN
  • Discrepencies between ESR and CRP
    • Consider time course, non-inflammatory causes of elevated ESR, and low grade inflammation causing elevated CRP
    • Elevated ESR with normal CRP
      • Lupus: Often causes more marked elevations in ESR than CRP
        • Izzy thanks for teaching us that patients with lupus produce type 1 interferons which inhibit hepatocytes production of CRP
        • An elevated CRP in a patient with lupus can strongly suggest a bacterial infection over a lupus flare. The exception is lupus serositis which can large rises in CRP
      • Paraproteinemia
  • Review article from BMJ on the usefulness of inflammatory markers in clinical practice: http://www.bmj.com/content/344/bmj.e454

 

Moffitt Pearls – Rheumatology Report – 8.21.2017 – Evan’s Syndrome and SLE

Thank you to Leah for presenting a very interesting case of a young man with history of lupus c/b APS and DVT presenting with thrombocytopenia+ hemolysis found to have Evan’s Syndrome (ITP + AIHA)!

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Key Pearls

  1. Although the term is falling out of favor Evan’s syndrome describes the combo of ITP & AIHA.
  2. For unclear reasons, lupus flares are often accompanied by a dissociation between ESR (high) and CRP (nl or minimal elevation). Other findings of flare (vs. infection) include drop in complement, recued leukocyte count and + RBC casts and/or proteinuria.
  3. Direct oral anticoagulants have not been studied nor are they recommended as anticoagulants in patients with lupus and known DVT. Treat with warfarin or LMWH only!

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More on Evan’s Syndrome

  • Combination of Coombs-positive warm AIHA and immune thrombocytopenia (ITP).
  • Antibodies that cause platelet destruction are unique from those that cause AIHA.
  • Differential Diagnosis:
    • Infectious (eg, HCV, HIV)
    • SLE
    • Lymphoproliferative disorders
    • Common Variable immunodeficiency
  • Treatment (similar to ITP, however azathioprine and cyclophosphamide are used)
    • First line therapy: Glucocorticoid @ 1-2 mg/kg +/- IVIG
    • Second line: Azathioprine or rituximab
    • Third line: Splenectomy
    • Under investigation: Eltrombopag –> a TPO receptor agonist stimulating platelet formation

American College of Rheumatology Classification of SLE

[Always verify all prior diagnoses of rheumatological conditions]

  • SLE Criteria > 3 of 11 ( Se & Sp > 95% and all DO NOT have to be at the same time)
    • Cutaneous Manifestations
      • Malar Rash
      • Photosensitivity
      • Discoid Rash
      • Oral ulcer
    • MSK
      • Nonerosive arthritis – oligioarticualr; symmetrical, migratory
    • Cardiopulmonary
      • Serositis – pleuritic or pericarditis
    • Renal
      • Proteinuria or cellular Casts – > 500 mg/dL on UA or 3+ on dip

 

    • Neurological
      • Seizures OR psychosis

 

    • Hematological
      • Hemolytic anemia or leukopenia, or lymphopenia or thrombocytopenia
    • Serologies
      • +ANA
      • +anti-dsDNA or antiphopholipid Abs

 

Review of Hemolytic Anemias based on Smear

Picture2