Category Archives: Rheumatology

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)!


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!


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


Moffitt Pearls 8.8.17 – Undifferentiated systemic Rheumatic Disease & ILD

Thank you, Saate, for getting us started this am with a young man reporting 10 days of cough with complete white out of the left side of his chest found to have a large empyema!

Thank you, Emma and Gabby, for co-presenting the fascinating case of a young woman with history of RA coming in with persistent cough and fever.

**Key Pearls**

Ditty Pearl: Copper deficiency is an uncommon nutritional deficiency seen in patients after gastric bypass and results in a myelopathy localizing to the posterior columns and bilateral corticospinal tracts

Image Pearl: Tracheal deviation towards or away from a dense hemithorax can help distinguish volume loss (atelectasis) from a fluid occupying processes (i.e. lung collapse from massive pleural effusion or hemothorax)

Case Pearl: Rheumatologic diseases most frequently associated with pulmonary involvement, specifically ILD are scleroderma, myositis, dermatomyositis, MCTD and rheumatoid arthritis

Undifferentiated systemic Rheumatic Disease and Overlap Syndromes

  • As many as 25% of rheum disease in patients with systemic symptoms cannot be definitely diagnosed (don’t feel bad Emma and Gabby!)
  • Evaluation of a patient w/ possible autoimmune disease includes the following:

1) How was their disease diagnosed (clinical history and serologies)?

2) What therapies or diagnostic evaluations have they had?

3) What Rheum disease processes fall within their clinical presentation? (Use clinical history to dictate further work-up & serology evaluation)



VA Intern Report Pearls 6.8.17: Behcet’s and the Silk Road

Case summary

Thanks to Colin Purmal for presenting the case of a 26F presenting with diffuse abdominal pain, bloody diarrhea and oral ulcers, who was diagnosed with Behcet’s disease.

Top pearls

1. Oral ulcers have a fascinating differential, including infection, autoimmune disease, malignancy, medication-induced, and vitamin deficiencies. But the most common cause is the aphthous ulcer!

2. Consider treating severe infectious bloody diarrhea (e.g. systemically ill, immunocompromised or elderly) with ciprofloxacin. The benefits outweigh the risks except in cases with high concern for EHEC or Salmonella typhi.

3. Behcet’s disease is a key “Crohn’s disease mimicker.”

Oral ulcers

  • Pocket reference Ddx:

Infection– primary HSV, HIV,TB, CMV, EBV, coxsackie, GC/CT, syphilis, fungal

Autoimmune– Behcet’s, SLE, Crohn’s, blistering skin disease (Pemphigus)

Malignant– SCC, lymphoma



Vit Deficiencies

Antibiotics in infectious bloody diarrhea

  • Most acute infectious diarrhea does not require antibiotic therapy.
  • 2001 IDSA guidelines for dysentery (to use the Oregon Trail-approved terminology) recommend obtaining stool cultures and treating empirically for 3 days in 3 cases: 1) toxic-appearing, 2) elderly, 3) immunocompromise.
  • The main benefit of antibiotic therapy is reduced duration of symptoms.
  • Ciprofloxacin is first-line, but Azithromycin can be used in pregnancy or if high suspicion for resistant Campylobacter (e.g. recent SE Asia travel).
  • Contraindications: 1) high concern for enterohemorrhagic E. coli (concern for precipitating HUS), 2) high concern for Salmonella typhi (concern for promoting carrier state and relapse)

IDSA Guidelines:

Crohn’s disease mimickers

  • Thinking about Crohn’s disease?
  • Consider Behcet’s disease in…patients from eastern Asia or the Middle East (the Silk Road!) with oral ulcerations (more frequent and more severe); genital ulcerations (more specific but less sensitive); systemic symptoms, including ocular symptoms, rashes, pan-vasculitis, weird clots (Budd-Chiari or cerebral venous thrombosis), pathergy (a 20G needle prick causes a papule or pustule within 48 hours).
  • International Study Group for Behcet’s Disease Diagnostic Criteria:
Recurrent oral ulceration: Aphthous (idiopathic) ulceration, observed by physician or patient, with at least three episodes in any 12-month period
Plus any 2 of the following:  
Recurrent genital ulceration Aphthous ulceration or scarring, observed by physician or patient
Eye lesions Anterior or posterior uveitis cells in vitreous in slit-lamp examination; or retinal vasculitis documented by ophthalmologist
Skin lesions Erythema nodosum-like lesions observed by physician or patient; papulopustular skin lesions or pseudofolliculitis with characteristic acnelform nodules observed by physician
Pathergy test Interpreted at 24 to 48 hours by physician

Behcet’s review:

Moffitt Pearls 6.5.17 – Palpable Purpura

Thank you to Kenny for presenting a fascinating case of an elderly woman with a remote history of breast cancer presenting with SOB and weight loss found to have lower extremity edema, a diastolic murmur and finally skin findings that included palpable purpura and splinter hemorrhages!!!


Key Learning Points

  • Diastolic murmurs are graded from 1-4 (thank you Arvind!)
  • Photos such as those presented in report can be uploaded via the Haiku app
  • Here is diagnostic pathway for palpable purpura Palpable Purpura


As we discussed palpable purpura has an exhaustive list of etiologies – think big categories – Malignancy, Infectious and noninfectious endocarditis and primary rheum. In this patient in particular we had a discussion around sub-acute bacterial endocarditis given her diastolic murmur, splinter hemorrhages and palpable purpura.

Palpable Purpura

  •  A sign of vascular inflammation or damage, usually a small or medium vessel vasculitis
  • Palpable purpura is the hallmark of a leukocytoclastic vasculitis, but LCV is a histopathologic diagnosis


  •  Primary rheum
    • ANCA-associated
    •  Cryoglobuinemic vasculitis
    • Polyarteritis nodosa
    • Henoch-schonlein purpura (rare in adults)
    •      Urticarial vasculitiS
    •  SLE
  •   Infection
    •          HBV-associated PAN
    •          HCV associated cryoglobulinemia
    •          Purpura fulminans associated with DIC
  •   Malignancy-related
  •   Medication effect (meds can cause an LCV on their own!)

Recommendation for first line w/u of palpable purpura

  •          CBC w platelets
  •          ESR/CRP
  •          ANA
  •          ANCAs
  •          Complements
  •          UA
  •          Infectious w/u including Blood Cx, HIV, HCV, HBV, +/- ASo or strep throat culture and cryos

ZSFG Morning Report: Dactylitis ddx, IGRA’s, and a hint of NTM/TB

Thanks to Lily Kornbluth for presenting the case of a woman from China admitted with a swollen and painful finger which turned out to be mycobacterial dactylitis!

You’re probably asking yourself: Wait, aren’t those swollen digits just in psoriatic arthritis??? Do I have a framework or a DDX FOR DACTYLITIS??? Well, NOW YOU DO! Here’s a short list generated in report today with the help of Lisa Winston, Stephanie Cohen, Lily, and the #fabulous ZSFG resident crew!

dactyl 1

Dactylitis DDX:


  • NTM
  • TB
  • Syphilis
  • Leprosy
  • Fungi like Blasto, Cocci, Aspergillus
  • Bacterial tenosynovitis or septic arthritis or osteo w/ Staph, Strep, Gonorrhea, Nocardia, and Vibrio (this patient noted a history of food preparation…;)


  • Psoriatic arthritis
  • Reactive arthritis
  • Gout
  • Sarcoid
  • SLE
  • RA

Malignant: Leukemia

Heme: Sickle Cell dz

As we narrowed in on the dx, a diagnostic test returned: POSITIVE QUANTIFERON GOLD! Lisa Winston reminded us about the power of the QTF-Gold and that it’s more specific than the PPD! Here’s a quick refresher and an elegant representation of the box/test….
quant gold

QuantiFERON Gold or more technically since it just rolls off the tongue: Interferon-gamma release assays (IGRA’s):

  • What are they?
    • Diagnostic tools for latent TB indicating a cellular immune response to M. tuberculosis.
      • IGRAs can’t distinguish between latent and active TB aka don’t use them to dx active TB
      • IGRA’s available around the world: QuantiFERON-TB Gold In-Tube (QFT-GIT) assay, Quantiferon-TB Gold (QFT-G) assay, and the T-SPOT.TB assay

  • How do they work?
    • By capitalizing on the M. tuberculosis–specific antigens: early secreted antigenic target 6 (ESAT-6) and culture filtrate protein 10 (CFP-10)!
      • QFT-GIT assay is an ELISA-based test that uses peptides from three TB antigens (ESAT-6, CFP-10, and TB7.7). A positive test denotes IFN-gamma response to TB antigens that’s above the test cut-off.
      • Of note, ESAT-6 and CFP-10 are encoded by genes in the region of difference 1 (RD1) segment of the M. tuberculosis genome. These antigens are more specific for M. tuberculosis than the PPD because they are not shared with any BCG vaccine strains or most species of NTM.
        • HOWEVER: Two of the NTM that affect humans, Mycobacterium marinum and Mycobacterium kansasii, contain gene sequences that encode for ESAT-6 or CFP-10. So infxn with either of these NTM’s=positive QTF test 😦

  • What are their test characteristics?
    • IGRA’s have specificity >95% for diagnosis of latent TB, especially great as unaffected by BCG vaccination. The sensitivity for the IGRA’s T-SPOT.TB and QFT-GIT are 90 and 80%, respectively. PPD sensitivity as reference is 80%. Of note, IGRA sensitivity decreases in patients with HIV!

Once the deep skin biopsy came back “swimming in AFB’s,” we discussed how to approach mycobacteria based on rapid or slow growth. Here is a simplified algorithm to consider…
mycobact outline


*Lisa Winston, Stephanie Cohen, and ZSFG resident crew
*Pai M et al. Systematic review: T-cell-based assays for the diagnosis of latent tuberculosis infection: an update. Ann Intern Med. 2008 Aug 5;149(3):177-84. Epub 2008 Jun 30. PMID:18593687




MOFFITT MORNING REPORT PEARLS 5/24/17: Polyarthralgias and the Red Eye!

Hey Everyone! Thanks to Vaibhav for presenting the case of a young man with recurrent fevers, arthralgias, and eye inflammation (possibly iritis). The workup was pending but he had undergone an extensive infectious and inflammatory workup previously that was negative. We’ll have to wait and see if anything pops up this time. Pearls below on polyarthralgias and the red eye:


Top Pearls:

  1. SLE joint symptoms usually manifest as a migratory, polyarticular, symmetric, non-erosive, inflammatory arthritis, though arthralgias without synovitis are not uncommon.
  2. When approaching polyarthralgia, look for the presence or absence of synovitis and the symptom duration as initial diagnostic branch points.
  3. Foreign body sensation localizes to the cornea, while photophobia could be corneal, anterior chamber/iris, or acute angle closure glaucoma.


For those who want more info:

*Pearl: Arthritis/arthralgias in SLE– occurs in >90% and often one of the earliest manifestations. 65-70% of cases are true arthritis with inflammation, and tends to be migratory, polyarticular, symmetric, and non-erosive. However, occasionally erosive arthritis does occur in SLE.

See the algorithm below for an approach to polyarthralgia, and the table below for major causes of inflammatory polyarthritis.

*Take-home points are that the presence or absence of synovitis and the symptom duration are the most helpful initial diagnostic features.


Inflammatory polyarthritis

The Red Eye: Classify by site of disease. The bolded ones require urgent/emergent ophtho referral!

  • Lids/lashes: hordeolum, chalazion, blepharitis
  • Conjunctiva: conjunctivitis (bacterial, viral, allergic), episcleritis/scleritis, hemorrhage
  • Cornea: abrasion, contact lens overwear, foreign body, infectious keratitis (bacterial, viral)
  • Anterior chamber/iris: iritis, hyphema, hypopyon
  • Lens: acute angle closure glaucoma

*Pearl: Foreign body sensation is fairly specific to corneal processes.

*Pearl: Photophobia could be corneal, anterior chamber/iris, or acute glaucoma.

Also see Rachel’s previous pearls on eye complaints in primary care:




Have a great day everyone!


MOFFITT CARDIOLOGY REPORT PEARLS 5/2/17: Kawasaki Disease and Coronary Aneurysms!

Hey Everyone! Thanks to Jin and Alayn for presenting the case of a young man with childhood Kawasaki disease coming in with progressive chest pain and dyspnea, and found to have a coronary aneurysm! He will undergo a cardiac stress test to figure out whether the symptoms are related to the aneurysm. Pearls below!


Top Pearls:

  1. Kawasaki disease is usually self-limited, but cardiac complications are common.
  2. Coronary aneurysms are the most common complication.
  3. Treat early with IVIG and aspirin to prevent these complications.


For those who want more info:

Kawasaki disease (KD) is rare in adults and is typically self-limited with an acute course of (average) 12 days. There DOES seem to be a genetic component to Kawasaki disease (increased frequency among family members of an index case and increased frequency in Asian and Asian-American patients).

*Cardiovascular complications include:

  • Coronary aneurysms (this is the major one, see below for more info!)
  • Heart failure
  • MI
  • Arrhythmias
  • PAD

*Diagnostic criteria: Fever lasting at least 5 days without other explanation, AND at least 4 of:

  • Bilateral conjunctivitis [>75%]
  • Oral mucous membrane changes (fissures, injection, strawberry tongue) [90%]
  • Cervical lymphadenopathy [25-70%]
  • Peripheral erythema or edema (palms/soles) [50-85%]
  • Polymorphous rash [70-90%]

*Treatment: IVIG (single dose), ASA during acute phase until inflammatory markers resolve. ASA is continued if aneurysms persist.

*Coronary artery aneurysms:

  • 30-40% of patients with acute Kawasaki disease!


  • 10-20% persist beyond one month (highest mortality is 15-45 days after disease onset)
  • May cause late ischemic heart disease
  • ECG and TTE for all patients with KD (CTA or MRA if TTE cannot image arteries)
  • Aneurysms most commonly found in proximal LAD, often at arterial branch points
  • “Giant” aneurysms >8 mm have high risk of morbidity/mortality (up to 50%), often anticoagulated in addition to aspirin
  • Who gets PCI? Ischemic symptoms + reversible ischemia on stress testing + >75% stenosis. CABG recommended if severe LV dysfunction or stenosis not amenable to PCI.

IN SUMMARY: If someone has suspected KD, treat early with IVIG and aspirin to prevent cardiac complications. Do ECG and TTE (and possibly CTA/MRA) in the acute phase looking for coronary aneurysms.

*Pearl: KD is not the only disease associated with coronary aneurysms. Other associations include PAN, Ehlers-Danlos, Loeys-Dietz, and other familial syndromes.




Have a great day everyone!