Systemic Lupus Erythomatosus

Today we had the chance to briefly discuss the new diagnosis of SLE in a South-East Asian male presenting with fever, chills, pleural effusions, and a drug-induced maculopapular rash. He was found to have pancytopenia (including both neutropenia and lymphopenia), an elevated creatinine, pancolitis on CT, pleural and pericardial effusions.

The ACR criteria for lupus suggest that patients must have 4 of the 11 criteria, although not necessarily concurrently. This lack of "simultaneous manifestations", unlike in our patient, can make earlier SLE much harder to diagnose without a high index of suspicion. This is important because the later the diagnosis, the more chance for irreversible damage, such as worsening lupus nephritis.

Here is an article on sex disparities in SLE. Interestingly, 4-22% of SLE occurs in males. Case control/cohort studies suggest that men experience more renal disease with SLE, and also more hematological manifestations. Serositis may also be more common in men. However, these results are conflicted and may be more complex in terms of race interactions and other factors than initially believed. Men may have a poorer long-term prognosis than women suggested by higher disease activity scores (SLICC score).

SLE, perhaps more so than any other rheumatological condition, is manifest by gross disparities in outcomes among patients of varying socioeconomic class and race/ethnicity. This has been extensively studied. Many factors play a role, inlcuding fixed factors, such as age, sex, race, and geography, and modifiable factors such as access to healthcare, insurance, social support. It is important to remember that these disparities exist in a sociopolitical context as well. Many texts classify socioeconomic status as a fixed factor, but I would argue that it is potentially modifiable with more adequate social assistance, and policies that recognize and address the social determinants of health. Here is a review of the disparities in SLE.

Non-typhoidal salmonella bacteremia and HIV

Morning report today focused on an HIV+/HBV coinfected man with a CD4 count of 7, presenting with diarrhea, cachexia, and cirrhosis following travel, ultimately diagnosed with Salmonella enteriditis bacteremia.

Salmonella infections can be classified as typhoid fever, or those caused by Salmonella typhi or paratyphi, and other non-typhoidal salmonella (NTS) infections. NTS clinical syndromes can take 4 forms:

1) diarrheal disease (colitis)

2) invasive bacteremia

3) focal or suppurative infection

4) asymptomatic carriage in stool.

HIV+ patients are at much higher risk for invasive NTS bacteremia when compared to HIV negative populations, and are much more likely to have recurrence of disease. Recurrent often occurs with the same strain and is thought to be related to reticuloendothelial persistence. The presentation can be quite non-specific and may not include any diarrhea at all.

See here for a brief review article on salmonella in immunocompromised hosts.

Treatment is typically with fluoroquinolones. Plasmid-mediated resistance does exist, but in relatively few isolates in the US - our patient was successfully treated with cipro as well. See here for a study about this if bugs and drugs are your thing!

Lots more to discuss: differential diagnosis of esophageal ulcers in and HIV+ patient, differential diagnosis of diarrhea in HIV+ patients, and HBV/HIV coinfection, but I'll have to save those for future blogs! Thanks to Dr. Vellend for being our guest.

On being a good physician

Today with Dr. Kitchens we had the special privilege of reflecting on our memorable cases and our most recent reads. It was great to hear that we all indulge our passions and interests outside of medicine, whether it is through stories of harpooning and sinking ships or reading to your loved ones.

The medicine teams right now are reading:

My name is red by Orhan Pamuk

The Kiterunner by Khaled Hosseini

Complications by Atul Gawande

A Spot of Bother by Mark Haddon

The Island Beneath the Sea by Isabel Allende

Never Let me Go by Kazuo Ishiguro

Consumption by Kevin Patterson

Omar Khayyam's Rubaiyyat

Among others.

I'm glad we had the chance today to reflect on what makes us good physicians, and not just good doctors. Thanks Dr. Kitchens!

I'll leave you with some of the poetry we heard about today (Omar Khayyam):

Here with a Loaf of Bread beneath the Bough,

A Flask of Wine, a Book of Verse, and Thou

Beside me singing in the Wilderness

And Wilderness is Paradise now.

Palliative care

Today we had an introduction into palliative care by Dr. LaDelfa.

Often, in medicine, we are called to support individuals through extremely ill health - and often we focus on trying to stall, postpone, or even prevent death. As physicians, though, we can sometimes play an even more important, kinder, and helpful role - that of facilitating a dignified and comfortable death.

Dr. LaDelfa highlighted some of the key elements of end of life care.

1. Provide effective symptom management

2. Help restore a patient's sense of control over their own life

3. Provide patients with the opportunity to deal with their existential concerns (psychological, religious)

4. Help patients feel relieved from their physical, financial, and emotional burdens

5. Provide patients with (or facilitate) time to engage in discussions with their families and friends to affirm their lives - that they had meaning, that they experiences successes, experienced love.

Please see here for a wonderful essay written by Atul Gawande in the New Yorker on "Letting Go" - on how liberating, for patients and providers alike, the experiences of death and dying can be when we focus on quality of life rather than quantity. I feel as though this should be required reading for all medical practitioners!

Cardiac tamponade

Thanks to Dr. Parker for taking us through some really interesting echocardiographic findings in a patient with cardiac tamponade.

Tamponade occurs when the intrapericardial pressure rises to the point where it compromises venous filling of the right atrium, which can actually be seen to collapse on echocardiogram. Impaired blood flow to the right side of the heart means less blood to go to the left side, exacerbated by the RV's attempt to expand in a compressed space which leads to interventricular bulging into the LV. This decreased LV filling results in the clinical findings of hypotension and pulsus paradoxus. Acute tamponade is immediately life-threatening and will require pericardiocentesis - get an echo and a cardiology consult!!!

See here for a recent review of pericardial diseases.

Hypertension

On thursday we discussed an interesting patient with possibly autonomic dysreflexia, possibly due to a prior cord injury. This led to an interesting discussion of hypertensive urgency and its management.

Understandably, on internal medicine we often focus on hypertensive urgencies (typically >180/110 with no impending progressive end-organ damage) and emergencies (uncontrolled BP with acute progressive end-organ damage) because they are what we manage as an inpatient. Here is a recent review article on the management of these entities.

However, in the ambulatory setting we are often asked to initiate or modify ongoing antihypertensive therapy, and it is important for us to understand the guidelines as well as special situations in which certain agents are more evidence-based than others. Here is a summary of the most recent US guidelines, and here are the more recent Canadian Hypertension Education Program 2009 guidelines.

Rhabdomyolysis

Today we discussed an interesting case: a 39M on a depot-antipsychotic and benztropine presenting with rhabdomyolysis. This led to a discussion around neuroleptic malignant syndrome as well as electrolytes since his potassium and phosphate were surprisingly low, rather than high as expected.

Interesting points arising from today's discussion:

1. Management of rhabdomyolysis is supportive, with ++ IV fluids and alkalinization of urine (difficult to achieve). Target urine pH is 6.5 or higher but the utility of this has been debated in recent years. Practically, remember that the way to create an alkalinizing infusion is 1 L of D5W, draw out 150mL and discard, and add 3 amps of sodium bicarb (a standard ampule is 50meq in 50mL). Your new solution is isotonic to serum. Remember, run this in ADDITION to the NS "resuscitation" fluids you are running. See here for the National Guidelines Clearinghouse guidelines on management of rhabdo - very practical.

2. Phosphate: hypophosphatemia is cited as a potential etiology of rhabdo, and it is one of the lytes that we very rarely learn about. Causes of hypoPO4 can be classified as due to: 1) Decreased intestinal absorption (eg vit D deficiency, antacid abuse), 2) Internal redistribution (eg refeeding syndrome, sepsis), and 3) increased urinary excretion (ie EtOH abuse, hyperPTH, vit D disorders). See here for a old but very useful article summarizing Mg and PO4 disorders (this is part of a 5-part elytes series that I have found useful in the past). Remember, replacing PO is safer than IV, as the latter runs the risk of causing severe hypocalcemia.

3. Neuroleptic malignant syndrome is a life-threatening medical emergency that is caused by antipsychotic medications. It has been described with both the typical and atypical antipsychotics. It is manifest by fever, autonomic instability, rigidity, and mental status changes. It can result in rhabdomyolysis, renal failure, hypoxia, and metabolic acidosis. Recovery is typically within 7-10 days of discontinuing the drug, although recovery may be prolonged in depot injections. NMS has a broad differential diagnosis (including withdrawal from other Rx , which must be considered carefully - see here for a review.

4. Lastly, we discussed alternatives to statin therapy. There has been some talk about red yeast rice, and there is actually evidence to prove it! The best medicine is usually the one your patient will take. See here for a recent article on this alternative therapy.

Hypercalcemia II - A focus on sarcoidosis

Hello all. After a week of no blogging (I was on stay-cation), it was great to discuss an interesting case of a young man with hypercalcemia. We discussed the differential diagnosis and management of hypercalcemia, with a focus on sarcoidosis.

Please see here for my previous blog on hypercalcemia.

Key pearls from today:

- severe hypercalcemia (ie >3) is rarely due to hyperparathyroidism alone

- FLUIDS are key; there is no evidence to support the use of furosemide in the management of hypercalcemia. See here for a narrative review on the subject (narrative because there are no trials!). Bisphosphonates are also supported by the evidence, with caution needed in renal impairment.

- In the absence of renal failure, the PO4 level can provide a quick clue to etiology: In vitamin-D related hyperCa (ie granulomatous disease, vit D intoxication), PO4 will be increased as it is reabsorbed more avidly. A high PO4 makes hyperPTH or PTH-related protein less likely as PTH/PTHrp typically cause decreased PO4 reabsorption in the kidney.

See here for a review of sarcoidosis, the "great mimicker".