- Patients are treated based on presence of cardiac dysfunction (ie GDMT for HFrEF) / arrhythmias
Find out more in this great review article: https://www.ncbi.nlm.nih.gov/pubmed/25443708
In this week’s Ambulatory Cox’s Conference, Dr. Esther de Boer presented a case of a young female who presented with altered mental status in the setting of severe thrombocytopenia. Our expert discussant, Dr. Nagalla guided us through the differential diagnosis of microangiopathic hemolytic anemia (MAHA).
The differential diagnosis for our patient with altered mental status and severe thrombocytopenia included intracranial hemorrhage in the setting of immune thrombocytopenia (ITP), cerebral vascular accident in the setting of heparin-immune thrombocytopenia (HIT), thrombotic thrombocytopenic purpura (TTP), disseminated intravascular coagulation (DIC) in the setting of sepsis, paroxysmal nocturnal hemoglobinuria (PNH), HELLP syndrome in the setting of pregnancy, acute liver failure, and tick-born illnesses such as ehrlichiosis. In addition to severe thrombocytopenia, the patient was also found to have severe anemia and evidence of hemolysis on laboratory evaluation. A peripheral smear revealed numerous schistocytes consistent with MAHA. The patient was started on plasma exchange and an ADAMTS13 activity level was sent off which later returned as < 5% and a high ADAMTS13 inhibitor titer consistent with acquired TTP.
Thrombotic Thrombocytopenic Purpura Pearls:
In this week’s Ambulatory Cox’s Conference, Dr. Jasmine Sukumar presented a case of a 50 year old man with history of alcohol cirrhosis, uncontrolled diabetes, and rectal cancer on chemotherapy who presented with right sided vision loss for 1 day. Our expert discussant, Dr. Pearlie Chong guided us through infectious complications to consider in an immunocompromised host.
The differential diagnosis for our patient with acute persistent vision loss included diabetic retinopathy with vitreous hemorrhage, retinal detachment, glaucoma, retinal artery or vein occlusion, optic neuritis, and giant cell arteritis. On exam the patient was found to have swelling and redness of the right eye with associated cranial nerve deficits. Based on the exam and the patient’s past medical history, we had to consider potentially less common etiologies of vision loss including intracranial malignancy (or metastasis), CMV retinitis, or invasive fungal infection. Imaging of the head and sinuses revealed thickening of the right ethmoid and maxillary sinus as well as a large soft-tissue mass in the retroorbital area. Surgical biopsies were performed that showed broad, irregularly branched hyphae with rare septations consistent with Rhizopus oryzae.
· Infections in humans are most commonly caused by Rhizopus, Mucor, and Rhizomucor. These organisms are ubiquitous in nature and commonly found in soil and decaying vegetation.
· In addition to immunocompromised patients, those with hemochromatosis (particularly those receiving deferoxamine therapy) and burn/trauma patients are also at risk of mucormysosis.
· Most common clinical presentation in rhino-orbital-cerebral infection caused by inhalation of spores into the paranasal sinuses.
o Common presenting complaints are fever, headache, nasal congestion, purulent nasal discharge, and sinus pain.
o If the infection spreads beyond the sinuses, nearby structures can be involved resulting in perinasal swelling, palate eschars, and erythema or necrosis of the skin overlying the sinuses or orbit.
o Signs of orbital involvement include periorbital edema, proptosis, and blindness as in our patient. Spread to the nearby cavernous sinus can result in cranial nerve palsies.1
· Mucormycosis can also present with pulmonary, gastrointestinal, cutaneous, renal, or isolated CNS involvement. Disseminated disease occurs in severely immunocompromised patients.
· Interestingly, many diabetic patients had ketoacidosis at the time of presentation.2
· As Mucormycosis is a rapidly progressive, fatal disease, prompt diagnosis and empiric treatment is crucial.
· Diagnosis relies on identification of the organism in tissue by histopathology, which requires a tissue biopsy. Cultures often yield no growth.
· The 1,3-beta-D-glucan assay and the Aspergillus galactomannan assay will be negative in these patients.
· Treatment requires both surgical deridement of involved tissues as well as antifungal therapy.
· It is also important to eliminate any potential predisposing factors for this infection including hyperglycemia, deferoxamine administration, immunosuppressive medications, and neutropenia.
· First line therapy is intravenous amphotericin B at 5-10mg/kg daily. After a patient has shown clinical improvement, the patient can be transitioned to posaconazole.3
· Some data suggests improved patient outcomes with addition of caspofungin to amphotericin B compared to amphotericin B monotherapy. However larger studies are needed to establish whether combination therapy is truly beneficial.4
· Despite appropriate antifungal therapy, aggressive surgical debridement is required for adequate disease control.
In this week’s Ambulatory Cox’s Conference, Dr. Namrah Siddiq presented a case of a man with history of malignant melanoma on ipilimumab who presented with nausea, vomiting, and altered mentation. Further evaluation discovered that the patient had severe hyponatremia. Our expert discussant, Dr. Jessica Abramowitz guided us through immune checkpoint inhibitor related endocrinopathies.
Our differential diagnosis for an elderly patient with nausea, vomiting, and altered mental status initially included commonly encountered illnesses including viral gastroenteritis, urinary tract infection, dehydration, small bowel obstruction, malignancy (gastrointestinal tract tumor or metastatic disease), medication side effects, and metabolic disturbances. After we discovered the patient had a history of melanoma and was on ipilimumab, an immune checkpoint inhibitor that targets CTLA-4, our differential broadened to include ipilimumab-related enterocolitis, hepatitis, and hypophysitis. In the setting of severe hyponatremia, the patient was found to have an AM cortisol of 1 mcg/dL, undetectable TSH, and low free T4. Findings were consistent with hypophysitis, a known adverse effect of immune checkpoint inhibitors.
Immune Checkpoint Inhibitor Induced Hypophysitis Pearls:
R3 Dr. Allison Lange published a case report today in BMJ Case Reports. She took care of a patient with a history of dermatomyositis who presented to the hospital with fevers and arm pain. The patient was found to have CMV viremia which then precipitated HLH/macrophage activation syndrome. This case report represents the first such report of CMV viremia causing MAS in a patient with dematomyositis.
Dr. Lange wrote this report with Dr. Kazi (our program director), Dr Chen (of hematopathology), and Dr. Barnes (infectious diseases). Great work!
The paper can be found here:
In this week’s Ambulatory Cox’s Conference, Dr. Neela Thangada presented a case of a female with history of hypertension, type 2 diabetes, and adrenal insufficiency secondary to pigmented nodular adrenocortical disease who presented with a chief complaint of shortness of breath, one week in duration. Our expert discussant, Dr. Ian Neeland guided us through the differential diagnosis for acute, progressive shortness of breath.
The young female patient was also experiencing orthopnea, paroxysmal nocturnal dyspnea, and exercise intolerance, all symptoms concerning for heart failure. Exam was pertinent for jugular venous distention, muffled heart sounds, and diffuse crackles. Our differential for acute onset of heart failure included myocarditis, myocardial infarction, valvular disease (acute aortic insufficiency), cardiac tamponade, constrictive pericarditis, infiltrative cardiomyopathies, familial cardiomyopathies, endocrinopathies (hyperthyroidism, hypothyroidism, adrenal insufficiency, and acromegaly), and nutritional deficiencies leading to heart failure. A bedside echocardiogram was performed that revealed a pericardial effusion. The patient had a notable history of recent sternotomy for anterior mediastinal mass resection, which made post-pericardiotomy syndrome the likely diagnosis.
Post-Pericardiotomy Syndrome Pearls:
Yesterday, R3 Dr. Brandon Jakubowski presented a case he initially saw in PRIME clinic – a young lady who presented with a papular rash on her chin. The rash was initially treated conservatively, however progressed and involved painful pustular lesions on the patient’s arms. The patient was seen by dermatology and underwent a biopsy, however ended up going to the emergency room prior to the pathology resulting. In the ER, the patient was hypotensive and was noted to have absent upper extremity pulses. A CT scan was performed and aided in diagnosing Takayasu’s arteritis.
For research purposes, the American College of Rheumatology developed diagnostic criteria for Takayasu Arteritis in 1990. Patients must meet at least three of the following six criteria:
The modified Ishikawa diagnostic criteria is slightly more complicated but has a higher sensitivity and specificity (92.5% and 95%, respectively) than the original ACR criteria and is notable for removing the age criteria.
In the management of Takayasu Arteritis, immunosuppressants are the cornerstone of therapy, however optimal management is undefined. This particular patient received high dose steroids, then transitioned to tocilizumab when side effects were no longer manageable. It is important to understand that laboratory findings do not reliably track disease activity and therapy is essentially guided by patient symptoms.
Arend WP, Michel BA, Bloch DA , et al. The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum 1990;33:1129–34.doi:10.1002/art.1780330811
In last week’s Ambulatory Cox’s Conference, Dr. Stephen Philip presented a case of a female with history of rheumatoid arthritis who presented with a chief complaint of fever and rash. Initial laboratory studies unveiled an active urinary sediment concerning for glomerulonephritis. Our expert discussant, Dr. Biff Palmer guided us through the differential diagnosis for fever and rash with renal involvement.
In a patient who presents with fevers and rash, the differential diagnosis is broad and includes infectious, autoimmune, neoplastic, and drug-related etiologies. Our patient had evidence of renal involvement with an active urinary sediment concerning for glomerulonephritis. Our differential diagnosis in this patient included systemic lupus erythematosus, vasculitis (IgA vasculitis, ANCA-associated vasculitis, or cryoglobulinemia), common viral illnesses (EBV, CMV, and HIV), infective endocarditis, disseminated fungal infection, and cutaneous malignancy. The patient underwent a skin biopsy which confirmed the diagnosis of Sweet’s Syndrome.
Sweet’s Syndrome Pearls:
· Also known as acute febrile neutrophilic dermatosis
· Characterized by abrupt onset of painful, erythematous papules, plaques, or nodules which is frequently accompanied by fever and leukocytosis.
· Neutrophilic infiltration to other organ systems can very rarely occur, including ocular, musculoskeletal, central nervous system, cardiovascular system, pulmonary system, gastrointestinal tract/liver, and kidneys. Renal involvement, as in our patient, is rare but commonly presents with proteinuria, less commonly with hematuria or renal insufficiency.1-2
· Most commonly associated with infections (after URI or GI infection), inflammatory bowel disease, pregnancy, autoimmune diseases (RA, SLE, dermatomyositis, sarcoid), malignancies (more commonly hematologic malignancies), and drug related (most common is G-CSF).
· Patients will have abrupt onset of painful inflammatory papules, plaques, or nodules. The distribution will be asymmetric with the upper extremities most commonly effected.
· Often associated with fever, arthralgias, headache, myalgias, and malaise.
· Extracutaneous involvement can occur, frequently ocular and musculoskeletal. However additional organ systems can also become involved.
· Laboratory studies often show a peripheral leukocytosis with neutrophilia. An elevated erythrocyte sedimentation rate and C-reactive protein is common, along with anemia and platelet abnormalities.
· Diagnostic criteria has been established – both major criteria is required along with two of four minor required.3
§ Abrupt onset of painful erythematous plaques or nodules
§ Histopathologic evidence of a dense neutrophilic infiltrate without evidence of leukocytoclastic vasculitis
o Minor Criteria:
§ Fever, > 38 C
§ Association with underlying maligiancy, inflammatory disease, or pregnancy OR preceded by URI, GI infection, or vaccination
§ Excellent response to treatment with systemic glucocorticoids or potassium iodine
§ Abnormal laboratory values at presentation (need 3 of the 4: ESR > 20 mm/hr, positive C-reactive protein, > 8,000 leukocytes, > 70% neutrophils).
· Corticosteroid therapy is first-line. Typically start with prednisone at 0.5 to 1mg/kg per day.
· After initiation of steroid therapy, symptoms are expected to improve within 48 hours.
· Once disease control is obtained, can begin to taper steroids with a plan to discontinue steroids within 4-6 weeks.
· Other therapies such as colchicine, dapsone, and potassium iodide have been used as steroid-sparing agents.4
1. Cohen PR, Kurzrock R. Sweet’s syndrome revisited: a review of disease concepts. Int J Dermatol 2003; 42:761-778.
2. Vignon-Pennanmen MD. The extracutaneous involvement in the neutrophilic dermatoses. Clin Dermatol 2000;18:339–47.
UTSW has been chosen to work with NEJM hosting a journal club. We will be reviewing the paper “Procalcitonin-Guided Use of Antibiotics for Lower Respiratory Tract Infection” (ProACT). The Journal has recruited three of the study authors to help us provide some insight into the design and execution of the trial.
Please visit here for the discussion: https://resident360.nejm.org/discussions/679
Anybody can access the discussion and contribute!
The paper can be accessed here: https://www.nejm.org/doi/full/10.1056/NEJMoa1802670
This morning at the VA we did a mini M&M based on this case regarding the appropriateness of PICC lines in a patient with CKD/ESRD with diabetes and osteomyelitis.
PICCs should generally be avoided in patients with an eGFR <45 ml/min/1.73m2 or placed only after consultation with a nephrologist. The reason for this is PICC placement can harm future candidacy for AV fistula placement, the safest and most effective method of hemodialysis.
There are alternatives for patients needing long-term IV access that may preserve the central and peripheral vasculature in the event HD is needed. The MAGIC criteria have helped to delineate the most appropriate intravenous catheter for your patient. The original paper, published in 2015, can be found here. The authors have also developed an app, which can be found here.