Dr. Meyers presented a complex case of a patient presenting with subacute progressive rash, fevers, and dyspnea found to have acute progressive hypoxemic respiratory failure with diffuse consolidations. After an extensive workup for infectious and rheumatologic disease, the patient was ultimately diagnosed with the amyopathic variant of dermatomyositis. Some takeaways per Dr. Meyers:
- Clinical amyopathic dermatomyositis (CADM) is defined by classic skin findings of dermatomyositis in the absence of weakness or muscle enzyme abnormalities in the setting of compatible serologic findings
- It tends to affect white and asian females slightly more often than the general population
- A subset of amyopathic dermatomyositis patients develop antibodies to MDA5 which is unfortunately associated with a more rapid decline and rapidly progressive interstitial lung disease (which can be clinically indistinguishable from ARDS)
- ILD associated with dermatomyositis and polymyositis can pose a clinical dilemma
- Pathology c/w NSIP, UIP, organizing PNA, and acute interstitial PNA
- Response of muscle and skin involvement correlate or predict response
- Systemic steroids initial therapy
- If CADM or antisynthetase syndrome, it is recommended to add second immunosuppression agent immediately at the time of diagnosis given the rapid and aggressive course assocaited with these syndromes
- The presence of ferritin levels >1500 correlates with a lower survival in these patients and may be helpful in determining whether earlier and more aggressive immunosuppression is warranted while awaiting the more definitive serologic tests which can take days to weeks to return. (1)
Thanks again to Dr. Meyers for this interesting case!
- Gono T, Kawaguchi Y, Hara M, Masuda I, Katsumata Y, Shinozaki M, Ota Y, Ozeki E, Yamanaka H. Increased ferritin predicts development and severity of acute interstitial lung disease as a complication of dermatomyositis. Rheumatology (Oxford). 2010;49(7):1354–60.
Thank you to Dr. Wendy Reeve for an interesting case of acute hypoxemic respiratory failure secondary to methotrexate pneumonitis.
Remember that methotrexate is a folic acid analog that inhibits cellular proliferation through intracellular depletion of folate requiring coenzymes. It can have a variety of toxicities and side effects which include everything from mild GI upset to fever, hepatotoxicity, nephrotoxicity, pulmonary toxicity, myelosuppression and increased risk of infections and lymphoproliferative disorders.
Methotrexate pneumonitis is more likely in individuals over 60 years old, those with RA and pleuropulmonary disease, those on DMARDs, those with low albumin and diabetes.The diagnosis of methotrexate pneumonitis is made with the aide of hte Searles and Mckendry criteria:
Dr. Kearney presented an interesting case of an individual with untreated HIV who presented with dyspnea found to have hypoxemic respiratory failure. The patient's history of a significant period of time off antiretroviral therapy (>3 years), the severity of the presentation of hypoxemic respiratory failure, and the severity of the lymphopenia (CD4# <35) data-preserve-html-node="true" raised suspicion for opportunistic infection. Sputum and blood cultures for fungus subsequently returned positive for Coccidioides immitis. Treatment had been started for coccidioidomycosis empirically with an azole and was subsequently increased to include amphotericin B once cultures returned positive. Highly active antiretroviral therapy was begun on admission as well. The patient's respiratory failure improved over the subsequent days, but unfortunately he developed recurrent hypoxemic respiratory failure and pseudosepsis concerning for IRIS.
The differential for dyspnea in the HIV+ patient is myriad. However, the CD4 count can offer a reliable indicator for the particular injuries for which the patient is at risk:
Prompt evaluation and a high index of suspicion for disseminated infection improved this patient's presenting syndrome. And while the patient subsequently developed IRIS after concomitant initiation of HAART, it is worth noting that it appears patients presenting with disseminated coccidioidomycosis do not appear to be at increased risk of developing IRIS with early HAART initiation per , though more study is needed.(1)
For more information on the immune reconstitution inflammatory syndrome, check out the review by Walker et al.(3)
- Available at: http://hivinsite.ucsf.edu/InSite-KB-ref.jsp?page=kb-04-01-05&ref=kb-04-01-05-tb-04&no=4. Accessed February 8, 2018.
- Mu A, Shein TT, Jayachandran P, Paul S. Immune Reconstitution Inflammatory Syndrome in Patients with AIDS and Disseminated Coccidioidomycosis: A Case Series and Review of the Literature. J Int Assoc Provid AIDS Care. 2017;16(6):540-545.
- Walker NF, Scriven J, Meintjes G, Wilkinson RJ. Immune reconstitution inflammatory syndrome in HIV-infected patients. HIV AIDS (Auckl). 2015;7:49-64.
OHSU Internal Medicine residents and faculty have been busy these last few weeks! Congratulations to everyone on your successes and recognition!
Dr. Josh Liu presented an interesting case today of an older gentleman with a history of CKD, HFpEF, PAD, and BPH who presented with subacute oliguria which progressed to anuria in the setting of recent treatment for prostatitis, found to have AKI on CKD and eventually diagnosed with ATN. We spent the majority of our time focusing on both differential diagnosis and initial management.
- The DDx for AKI is expansive!! It can be divided into pre-renal, intrinsic, and post-renal causes -- each of which have long lists of differentials!
Decreased effective arterial volume (hypovolemia, cardiorenal, systemic vasodilatation), renal vasoconstriction (NSAIDs, ACEI/ARB, contrast, etc), large vessel disease (RAS, thrombosis, dissection, vasculitis, etc)
Intrinsic renal disease:
ATN (ischemia, toxins, contrast-induced), AIN (allergic, infection, infiltrative, autoimmune), small vessel disease (cholesterol emboli, HUS/TTP, DIC, etc), glomerulonephritis.
bladder neck obstruction (BPH, prostate cancer, neurogenic bladder, etc), bilateral ureteral obstruction (malignancy, retroperitoneal fibrosis, nephrolithiasis, etc).
Work up including a detailed history and physical, electrolytes, UA, FeNa, renal ultrasound and potentially serologies or renal biopsy can be helpful when evaluating AKI.
Immediate management should focus on whether the patient needs urgent/emergent dialysis, including:
fluid overload, hyperkalemia, uremia, severe metabolic acidosis, or intoxication/toxins
We discussed management options including alternative imaging (when ultrasound is not available), when to call renal, temporizing measures, etc. This case actually presented at 6 pm near sign out time, thus we emphasized the importance of a safe, warm handoff to the night team as well.
Dr. Liu had several teaching points after reviewing a recent article published in Kidney & Blood Pressure Research, including: 1. AKI and certain risk factors (DM2, heart disease, severe Cr elevations) may increase risk of future AKI episodes, 2. AKIs are associated with worse CV and mortality outcomes, and 3. don't underestimate the effect of medications on the kidney!
Thanks to Sima Desai, Ken Scalapino, Joe Chiovaro, Claire Zeigler, Amarprit Bains, Joel Papak, Adam Obley, Alan Hunter, Joe Shatzel, Rebecca Harrison, and Kyle Kent for attending our first Clinical problem solving/ Razor case and their numerous pearls! And thanks to André Mansoor, APDs, Yale colleagues (Maximilian Stahl, Anne Liu and Pranay Sinha) for their advice and assistance.
This was a case of a young person with CAD risk factors, and recurrent presentations of arterial ischemia/ thrombosis who was ultimately diagnosed with polyarteritis nodosa.
This case highlighted some excellent pearls regarding clinical decision making:
- ) It is important to be cost conscious and thoughtful about ordering expensive diagnostic tests, but that the same time in a patient who is critically ill and may not re-present to the hospital, it is important to think more broadly and reasonable to order a broader panel of tests. Ultimately in order to practice cost conscious care, we must practice excellent diagnostic reasoning and ask how each test will change our decision making. Here is a great recent JAMA viewpoint about this.
- ) We discussed more "cost conscious" ways of ordering rheumatology studies (eg. consider ordering an ANA as a screening test before diving into an ENA panel and consider ordering only ESR or CRP and thinking about how the result would change management).
- ) A diagnosis of APLA rests on using the 2006 Sapporo criteria which include: a panel of tests (lupus anticoagulant, anti-cardiolipin and anti-beta2 glycoprotein) that need to be ordered and reconfirmed 12 weeks later, as well as evidence of thrombi or first trimester pregnancy loss.
Want to learn more?
Acute Limb ischemia
- Remember that the most likely cause by far is atherosclerotic disease or cardiogenic emboli
- In patients with multiorgan involvement and arterial thrombi it is important to consider more rare etiologies such as familial/ genetic conditions (including vasculopathies, CTD, hypercholesterolemia, thrombophilia), endocarditis, and vasculitis.
- Here is a nice table that outlines the severity of limb ischemia by stage as well as a great NEJM article regarding evaluation and treatment of acute limb ischemia
- Here is an article that was recommended by Joe Shatzel that he published on cryptogenic acute limb ischemia
- This is a very rare vasculitis leading to necrotizing inflammation of medium sized arteries that presents with multiorgan involvement
- Sometimes patients with this condition may be misidentified as drug seeking given their episodes of pain such as recurrent presentations to the ED for abdominal pain
- The ACR has criteria that can aid in diagnosis but overall this is a clinical diagnosis that should be entertained when other causes of arterial thrombi have been ruled out
- A quick literature search by Alan Hunter revealed that he did not find a specific connection between PAN and clubbing/ periungual erythema
- Treatment involves close follow up with our rheumatology colleagues, steroids and immunosuppressants
OHSU IM had an incredible showing at SGIM NW 2018! Congratulations to the residents selected to attend.