VA Resident Report: How many recent instances of Acute Rheumatic Fever in the United States?

The Bottom Line: Acute rheumatic fever is no longer a nationally notifiable disease in the United States, and its annual incidence in the continental United States declined in the late 20th century to approximately 0.04–0.06 cases per 1,000 children. Exceptions to these low acute rheumatic fever incidence rates in the United States include Samoan persons living in Hawaii and residents of American Samoa, an American territory in the South Pacific. Acute rheumatic fever rates in Hawaii have been as high as nearly 0.1 cases per 1,000 children, with even higher rates among persons of Samoan and Hawaiian ethnicity. Acute rheumatic fever occurs most commonly among children aged 5–15 years.

References: Acute Rheumatic Fever and Rheumatic Heart Disease Among Children — American Samoa, 2011–2012

Morbidity and Mortality Weekly Report (MMWR)

May 29, 2015 / 64(20);555-558

Summary: Acute rheumatic fever is a nonsuppurative, immune-mediated consequence of group A streptococcal pharyngitis (strep throat). Recurrent or severe acute rheumatic fever can cause permanent cardiac valve damage and rheumatic heart disease, which increases the risk for cardiac conditions (e.g., infective endocarditis, stroke, and congestive heart failure) (1,2). Antibiotics can prevent acute rheumatic fever if administered no more than 9 days after symptom onset. Long-term benzathine penicillin G (BPG) injections are effective in preventing recurrent acute rheumatic fever attacks and are recommended to be administered every 3–4 weeks for 10 years or until age 21 years to children who receive a diagnosis of acute rheumatic fever 


VA Resident Report: Is there an increased risk for patients with Terminal Complement Deficiency and HIV?

The Bottom Line: The Advisory Committee on Immunization Practices has previously recommended routine vaccination of persons aged ≥2 months who have certain medical conditions that increase risk for meningococcal disease, including persons who have persistent deficiencies in the complement pathway (e.g., C3, properdin, Factor D, Factor H, or C5–C9); persons receiving eculizumab (Soliris, Alexion Pharmaceuticals) for treatment of atypical hemolytic uremic syndrome or paroxysmal nocturnal hemoglobinuria (because the drug binds C5 and inhibits the terminal complement pathway)

MacNeil, J., Rubin, L., Patton, M., Ortega-Sanchez, I., & Martin, S. (2016). Recommendations for Use of Meningococcal Conjugate Vaccines in HIV-Infected Persons – Advisory Committee on Immunization Practices, 2016. Morbidity and Mortality Weekly Report MMWR /, 65(43), 1189-1194.

In addition, ACIP recommends routine vaccination with serogroup B meningococcal (MenB) vaccine for persons who have persistent complement component deficiencies; persons who have anatomic or functional asplenia; microbiologists who routinely are exposed to isolates of N. meningitidis; and persons identified to be at increased risk because of a serogroup B meningococcal disease outbreak.

EUHM Resident Report: What is the rate of lymphogranuloma venerum developing in the distal rectum and causing painful bleeding?

Anorectal LGV is a common cause of acute proctitis and proctocolitis among HIV-infected MSM who practice unprotected anal sex, and it is frequently associated with other rectal STDs. The implementation of routine screening and prompt diagnosis of these rectal infections should be mandatory in all clinical settings attended by HIV and STD patients.

Cabello Úbeda, A., Fernández Roblas, R., García Delgado, R., Martínez García, L., Sterlin, F., Fernández Guerrero, M., & Górgolas, M. (2016). Anorectal Lymphogranuloma Venereum in Madrid. Sexually Transmitted Diseases., 43(7), 414-419.

In this study, most subjects reported acute proctitis symptoms like tenesmus (85%), pain (88%), constipation (62%), or anal discharge (96%). Proctoscopy was performed in all patients showing mucopurulent exudate (25 patients [96%]) and rectal bleeding with mucosal erythema or oedema (22 patients [85%])

What is the clinical presentation associated with various drugs that incite drug rash with eosinophilia and systemic symptoms (DRESS)?

Bottom line: Typically presents with fever, rash, and solid organ involvement. Commonly associated drugs include aromatic anticonvulsants and sulfonamides.

Walsh SA, et al. Drug reaction with eosinophilia and systemic symptoms (DRESS): a clinical update and review of current thinking. Clin Exp Dermatol. 2011 Jan;36(1):6-11.
Table 2 summarizes diagnostic criteria proposed by a consensus group.

Kano Y.  The variable clinical picture of drug-induced hypersensitivity syndrome/drug rash with eosinophilia and systemic symptoms in relation to the eliciting drug.  Immunol Allergy Clin North Am. 2009 Aug;29(3):481-501
Reviews clinical presentation associated with specific drugs known to elicit DRESS.

Cephalosporins are not generally identified as causative agents in DRESS.  View case reports of DRESS associated with cephalosporins.
“Eosinophilia/chemically induced”[MAJR] AND (“cephalosporins”[MeSH Terms] ) AND “drug rash with eosinophilia”

Is there a danger in supplementing vitamin D in the setting of sarcoidosis?

The Bottom Line: In this first published RCT of vitamin D supplementation in sarcoidosis, it was found that patients with sarcoidosis and 25OHD <50 nmol/L, vitamin D supplements did not alter average serum calcium or urine calcium, but had no benefit on surrogate markers of skeletal health and caused one case of significant hypercalcaemia.

Bolland MJ, Wilsher MLGrey AHorne AMFenwick SGamble GDReid IR.  Randomised controlled trial of vitamin D supplementation in sarcoidosis.  BMJ Open. 2013 Oct 23;3(10):e003562.

Setting: Clinical research centre.
Participants: 27 normocalcaemic patients with sarcoidosis and 25-hydroxyvitamin D (25OHD) <50 nmol/L.
Intervention: 50 000 IU weekly cholecalciferol for 4 weeks, then 50 000 IU monthly for 11 months or placebo.

Strengths and limitations: The study had limited power to detect small differences in bone density and bone turnover markers.  Few participants had 25-hydroxyvitamin levels <25 nmol/L, and therefore the findings may not apply to individuals with very low vitamin D levels.

When is plasma exchange used as a therapy in Guillain-Barre syndrome?

The Bottom Line: Patients who receive PE are typically older, and have higher complication rates including respiratory failure.

Reference: Alshekhlee A, Hussain Z, Sultan B, Katirji B.  Immunotherapy for Guillain-Barré syndrome in the US hospitals. J Clin Neuromuscul Dis. 2008 Sep;10(1):4-10.   

Summary: In this large US cohort, it was discovered that patients in the older population were more likely to be treated with PE than IVIG and those receiving PE or combination therapy were about a decade older than those treated with IVIG alone. In addition, patients treated with PE and combination therapy had more respiratory complications and systemic infections and were more often discharged to skilled nursing or intermediate care facilities.

What are the criteria for diagnosing common variable immunodeficiency?

Bottom line:  Common variable immunodeficiency should be considered when a patient has

  • history of recurrent infections
  • documented impaired antibody production and/or “absent or highly impaired antibody response to vaccines or infectious agents
  • poor antibody response measured to protein antigens or polysaccharide antigens.”

SummaryDynaMed summarizes diagnostic criteria and cites the recent guideline (Transfus Med Rev 2010 Jan;24 Suppl 1:S28), which discusses diagnostic criteria on p. S30.

The guideline also includes a list of symptoms/history that should raise suspicion of common variable immunodeficiency, including:  ≥ 8 new ear infections within 1 year, ≥ 2 serious sinus infections within 1 year, ≥ 2 months on antibiotics with little effect, ≥ 2 pneumonia within 1 year, failure to thrive in infant, recurrent deep skin or organ abscesses, persistent thrush after 1 year old, need for IV antibiotics during infections, ≥ 2 deep seated infections, family history of primary immunodeficiency.

What is a low antigen content diet and how is it used in managing patients with cryoglobulinemia?

Bottom line:  Low antigen content (LAC) diet is similar to a hypoallergy restricted diet but foods with macromolecular antigens, such as meats, are further restricted.  Foods may be reintroduced in the same way as when checking food allergies.  Some small quasi-experimental studies suggest that an LAC diet is associated with improvement in symptoms of mixed cryoglobulinemia and reduced use of steroids, but there is no data from controlled trials.

Descriptions of the diet
Pietrogrande M, et al. Dietetic treatment of essential mixed cryoglobulinemia. Ric Clin Lab. 1986; 16: 413-416.
Clodoveo F, et al.  Low-antigen-content diet in the treatment of patients with mixed cryoglobulinemia.  Am J Med.  1989; 87(5): 519-524. 

Brief summary of evidence:
Pietrogrande M et al.  Recommendations for the management of mixed cryoglobulinemia syndrome in hepatitis C virus-infected patients.  Autoimmunity Rev.  2011; 10(8): 444-454.