Category Archives: #Rheumatology

8/31 MR with Kat: Young(ish) woman with chest pain #2

A middle aged woman with a pmh of SLE, DM1, and CKD who initially presented with exertional shortness of breath and underwent a workup to rule out PE and ACS: negative V/Q scan, negative myocardial perfusion study, and a normal ECHO.  It was thought that her symptoms were potentially related to ILD for which she was scheduled for outpatient workup with PFTs and HRCT.  She then presented 6 weeks later with typical chest pain, ischemic EKG changes, and a positive troponin found to have a 98% lesion of her proximal LAD.
 
SLE confers a significant risk for CAD – patients with lupus inherently have a higher risk for CAD, they are also more likely to have other risk factors associated with CAD; + antiphospholipid antibodies and lupus nephritis confer the highest risk
– Ddx of SLE + chest pain: ischemia, pleuritis/pericarditis, PE, pHTN, and all other causes of chest pain seen in non-SLE patients
-This was an odd presentation given her previous negative myocardial perfusion scan
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8/19 Morning Report w/ Jessica Duhe + Jinwoo: EGPA

A young man w/ history of asthma and sinusitis presents with myalgias, intermittent fevers, and purpuric rash on palms/soles after traveling in South America for months. Initial labs showed a striking eosinophilia of over 70%. During his course, he developed abdominal pain and mononeuritis multiplex. He initially underwent an extensive infectious workup, but was ultimately diagnosed with EGPA (Churg Strauss) and improved slowly with immunosuppresion.
A few points that came up:
1) Mnemonic for eosinophilia – NAACP 
Neoplasm (HL, carcinomas, leukemia/lymphoma; also consider hypereosinophilic syndrome)
Asthma / Allergy
Adrenal insufficiency
Connective tissue disease (EGPA, SLE, RA, myositis)
Parasites (Strongyloides, ascaris, schistosoma, lice, scabies)
2) How does one distinguish between EGPA and Hypereosinophilic Syndrome?
Hypereosinophilic Syndrome just describes end-organ dysfunction due to eosinophils. It can be the primary process OR secondary to another process (such as parasitic infection). When the diagnosis of EGPA is made, usually patients are NOT said to have “hypereosinophilic syndrome”, as it is unclear what damage is due to the eosinophils versus the vasculitis itself.
3) Teaching points on EGPA
*ANCA is only 40% sensitive (this is unlike MPA or GPA, where it is quite sensitive)! The EGPA manifestations may differ in ANCA-negative (heart involvment, fever) versus ANCA-positive (vasculitis, renal, neuropathy) but these patterns are not always seen
*Our patient illustrated the typical phases of disease:
Prodromal – atopic disease, allergic rhinitis, asthma
-Eosinophilic phase – peripheral eosinophilia, organ infiltration including lung and GI
-Vasculitic phase – life-threatening systemic small and medium-vessel vasculitis w/ constitutional sx

8/18 Morning Report w/ TK: Statin-induced myositis

An older female w/ DM presents with generalized weakness, elevated CK, and hyponatremia following a week of N/V and recent PNA. Her CK did not trend down with fluids, and she developed mild AKI and hyponatremia. MRI showed evidence of myositis. Biopsy is pending, but the presumed diagnosis is statin-induced myonecrosis. CK trending down and patient near discharge following fluids and holding of her statin!
Teaching Points for statin-associated muscle disease:
*It can be caused directly by the statin (most common) or linked to an autoimmune-mediated necrotizing myositis (this version persists after the drug is discontinued and is associated with antibodies to HMG-CoA reductase)
*It exists along a spectrum:
-myalgia: muscle pain, normal CK
-myopathy: weakness, with normal or mildly elevated CK
-myositis: muscle inflammation
-myonecrosis: muscle inflammation with significant elevation of CK + necrosis on biospy
-rhabdo: myonecrosis + end organ involvement (ie AKI)
*Statin muscle disease usually occurs within weeks to months of starting the drug but CAN occur at any point (especially if there are drug-drug interactions later on)
*Risk increases with drug-drug interactions (cyclosporine, gemfibrozil, PI, niacin, digoxin, antifungals, warfarin, and MACROLIDES which this patient had recently been prescribed.

7/22 Morning Report w/ Sunaina: Pulmonary-renal syndromes

An elderly woman w/ history of IDA, aortic aneurysm + AI, and monoclonal gammopathy presents with SOB, found to have rapidly progressive anemia, AKI, and R>L pulmonary infiltrate concerning for a pulmonary-renal syndrome. She was ultimately diagnosed with ANCA vasculitis with +MPO, possibly related to hydralazine use and with some SLE overlap. Despite steroids and cytoxan, her renal failure progressed quickly requiring dialysis, and she ultimately transitioned to comfort care several months after the initial diagnosis.
*Ddx for pulmonary-renal syndrome – 3 buckets of disease plus one mimicker:
-ANCA vasculitis (microscopic polyangiitis versus granulomatosis w/ polyangiiitis)
-Anti-GBM disease (aka Goodpasture’s)
-Vasculitis from other cause (lupus, HSP, IgA, infective endocarditis)
-Mimicker: Other acute GN (like poststrep GN) can lead to volume overload w/ resultant pulmonary edema and hemoptysis but not true DAH (can distinguish on BAL)