Immunologic and Rheumatologic Disorders

Immunologic and Rheumatologic Disorders


Kimberly L. DiMaria

Cheryl N. Bartke


  • Anaphylaxis occurs most commonly among children and adolescents.

  • Although the incidence of allergies and severe allergic reactions is on the rise, the estimated lifetime prevalence of anaphylaxis rate remains low at 0.05% to 2%.

  • Fatal cases of anaphylaxis are rare.


  • Most common triggers are food (e.g., eggs, shellfish, nuts), medications (e.g., antibiotics, nonsteroidal anti-inflammatories), and stinging.

  • Can occur through a variety of mechanisms, which are classified into the following categories:

    • Immunologic IgE-mediated/IgE-dependent/type I hypersensitivity reaction.

    • Immunologic non-IgE-mediated/IgE-independent.

    • Nonimmunologic/direct mast cell activation.

    • Idiopathic.

  • An allergen, such as radiocontrast, can precipitate an anaphylactic response through more than one mechanism, either an IgE-mediated response or a direct mast cell activation (i.e., causing histamine release).

  • Regardless of the mechanism, the clinical manifestations and initial management are identical.

Clinical Presentation

  • Rapid onset: minutes to hours.

  • Hives, itching, abdominal pain, emesis, stridor, wheezing, shortness of breath, laryngeal edema, hypotension, and shock.

  • If not treated promptly, can lead to cardiorespiratory collapse and death.

Diagnostic Evaluation

  • Clinical diagnosis based on pathopneumonic symptomology.

  • No specific laboratory test can confirm the diagnosis of anaphylaxis.

  • However, plasma histamine levels, serum total tryptase levels, and serum IgE levels, if measured during or following an anaphylactic episode, are all elevated.

  • Skin testing is indicated to confirm allergens or triggers.

    • Care should be taken to ensure that testing is carried out in a health care facility that can manage anaphylaxis, should the patient develop a reaction to a trigger.


  • ABC assessment.

  • Additional key examination components include pulmonary, neurologic, and skin examinations, and frequent vital signs.

  • Discontinue exposure to trigger/allergen if possible (e.g., a patient receiving intravenous [IV] chemotherapy).

  • Epinephrine administration, intramuscular/subcutaneous; should not be delayed if anaphylaxis is suspected.

    • Delays in administration are associated with worse outcomes.

    • Administration should occur promptly on recognition of mild anaphylaxis symptoms; it is not necessary to wait until potentially life-threatening symptoms develop.

    • 0.01 mg/kg of 1:1,000 (1 mg/mL) into vastus lateralis muscle.

      • Maximum dose: 0.3 mg for children; 0.5 mg for adults.

      • If possible, inject with an autoinjector.

    • Epinephrine is the only effective treatment for anaphylaxis. It will decrease laryngeal edema, treat hypotension and shock, cause bronchodilation, and increase cardiac output by increasing heart rate and myocardial contraction.

    • There is no absolute contraindication of epinephrine administration for treatment of anaphylaxis.

    • Repeat the dose every 5 to 15 minutes as needed.

    • An estimated 20% of patients will require multiple doses of epinephrine.

    • May consider initiating IV epinephrine infusion.

  • Place patient in a recumbent or supine position with lower extremities elevated above the heart.

  • Administer oxygen; secure airway if necessary.

  • Obtain IV access and administer 0.9% normal saline.

    • 30 to 40 mL/kg often required to treat hypotension.

  • Consider administration of adjunctive medications after epinephrine administration.

    • H1-antihistamine: diphenhydramine.

    • Nebulized β-agonist: albuterol.

    • Glucocorticoids: methylprednisolone IV or prednisone PO.

    • H2-antihistamine: ranitidine.


Megan Trahan


  • Humoral.

    • Isolated immunoglobulin deficiency (IgM, IgA, or IgG subclass).

    • X-linked agammaglobulinemia.

    • Common variable immunodeficiency.

    • Transient hypogammaglobulinemia of infancy.

    • Hyper-IgM syndrome.

  • Cellular.

    • 22q11.2 Deletion syndrome.

  • Combined antibody and cellular defects.

    • Severe combined immunodeficiency (SCID).

    • Wiskott-Aldrich syndrome.

    • Ataxia telangiectasia syndrome.

  • Phagocytic.

    • Chronic granulomatous disease (CGD).

    • Hyper-IgE syndrome.

    • Leukocyte adhesion deficits.

  • Complement.

    • Early complement defect (C2, C3, or C5).

    • Late complement defect.

Clinical Presentation

  • Common warning signs of primary immunodeficiency (Table 17.1).

  • In addition to infection, it may present with autoimmune diseases or lymphoid malignancy.

  • Evaluate for syndromes associated with primary immunodeficiency (e.g., 22q11 deletion [DiGeorge syndrome] or ataxia telangiectasia).

    TABLE 17.1 Warning Signs of Primary Immunodeficiency

    10 Warning Signs of Primary Immunodeficiency

    ≥4 new ear infections within 1 y

    Recurrent, deep skin or organ abscesses.

    ≥2 sinus infections within 1 y

    Persistent thrush in mouth or fungal infection on skin.

    ≥2 mo on antibiotics with little effect

    Need for IV antibiotics to clear infections.

    ≥2 pneumonias within 1 y

    ≥2 deep-seated infections including septicemia.

    Failure of an infant to gain weight or grow normally

    A family history of primary immunodeficiency.

    Adapted from Jeffery Modell Foundation. (2013). 10 warning signs of primary immunodeficiency. Retrieved from

  • Evaluate immune organs: tonsils, spleen, and lymph nodes.

  • Specific disease types and common presentations.

    • Humoral: sinopulmonary infections with encapsulated organisms.

    • Combined (SCIDs): failure to thrive, respiratory tract or gastrointestinal infections, candidal skin infections, Pneumocystis jiroveci pneumonia.

    • Phagocytic (CGD): infection with catalase-positive organisms (Escherichia coli, Pseudomonas, Klebsiella, Serratia, Salmonella, Candida, and Aspergillus).

    • Complement.

      • Early complement defects: sepsis.

      • Late complement defects: Neisseria infections.

Diagnostic Evaluation

  • Complete blood count (CBC): evaluate for anemia, thrombocytopenia, lymphopenia, or neutropenia.

  • Quantitative immunoglobulins (IgG, IgA, IgM, IgE).

  • Total protein and albumin.

    • Low total protein with normal albumin suggests immunoglobulin deficiency.

  • Antibody titers to vaccinations.

  • Complement activity (CH50, C3, and C4).

  • Nitroblue tetrazolium dye test: evaluate for CGD.


  • Depends on specific immunodeficiency.

  • Treat infection with appropriate antimicrobial based on suspected or confirmed organism.

  • Administer leukocyte-poor Cytomegalovirus (CMV)-negative blood when blood product administration is necessary.

  • Consider postexposure prophylaxis for varicella zoster.

  • Avoid live virus vaccines.

Juvenile Idiopathic Arthritis

Jan A. Odiaga


  • Juvenile idiopathic arthritis (JIA) encompasses a complex group of disorders comprising several clinical entities with the common feature of arthritis.

  • Each subtype of JIA is characterized by a different mode of presentation, disease course, and outcome.


Jan 30, 2021 | Posted by in NURSING | Comments Off on Immunologic and Rheumatologic Disorders

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