What is the appropriate acute management for a 24‑year‑old with common variable immunodeficiency presenting with severe left‑upper‑quadrant pain, low‑grade fever, chills, and diffuse pulmonary crackles two weeks after a treated chest infection?

Acute Management of Suspected Pneumonia with Sepsis in CVID

This patient requires immediate hospitalization, broad-spectrum intravenous antibiotics, blood cultures, chest imaging, and urgent assessment for splenic complications given the constellation of LUQ pain, fever, diffuse pulmonary crackles, and recent chest infection in the setting of common variable immunodeficiency.

Immediate Actions

Hospitalization and Monitoring

• Admit this patient urgently for inpatient management, as CVID patients with acute infections require prompt and aggressive antimicrobial treatment to prevent progression to sepsis and irreversible organ damage. 1
• The combination of fever (37.8°C), chills, diaphoresis, and diffuse pulmonary crackles indicates active infection despite recent treatment, which is common in CVID patients who remain susceptible to breakthrough infections. 1

Diagnostic Workup Priority

Pulmonary Assessment:

• Obtain chest radiograph immediately, followed by high-resolution CT if initial imaging is equivocal, as infectious lung disease occurs in the majority of CVID patients and bronchiectasis develops in 10-20% despite immunoglobulin replacement. 1, 2
• The diffuse crackles in all lung lobes suggest either multifocal pneumonia or progression of underlying structural lung disease (bronchiectasis or GLILD). 1, 2

Left Upper Quadrant Pain Evaluation:

• The severe LUQ pain (8/10 with movement) in a CVID patient warrants urgent abdominal CT with contrast to evaluate for splenic abscess, infarction, or rupture—particularly given the association of splenomegaly with GLILD and lymphoproliferative disease in CVID. 3, 1
• Approximately 10% of CVID patients develop GLILD, which is frequently accompanied by splenomegaly and carries increased mortality risk. 1, 2

Infectious Disease Workup:

• Draw blood cultures (at least two sets) before initiating antibiotics. 1
• Obtain complete blood count with differential to assess for cytopenias (which occur in 11-12% of CVID patients) and leukocytosis. 1, 2
• Check inflammatory markers (CRP, ESR) and comprehensive metabolic panel including liver function tests, as 40% of CVID patients have hepatic abnormalities. 1, 2

Antimicrobial Management

Empiric Antibiotic Selection:

• Initiate broad-spectrum intravenous antibiotics immediately covering encapsulated organisms (Streptococcus pneumoniae, Haemophilus influenzae) and atypical pathogens. 1, 2
• CVID patients are uniquely susceptible to recurrent infections with encapsulated organisms despite immunoglobulin replacement. 4
• Consider coverage for opportunistic infections if CD4 count is unknown or previously documented as low, as approximately 9% of CVID patients develop late-onset combined immunodeficiency with CD4 counts <200 cells/µL, presenting with opportunistic infections similar to Good syndrome. 3, 1

Antibiotic Duration:

• Plan for prolonged antibiotic courses (typically 2-3 weeks minimum) as CVID patients require more aggressive and extended treatment than immunocompetent hosts. 1

Immunoglobulin Replacement Assessment

Review Current Therapy

• Verify the patient's current IgG replacement regimen, most recent trough IgG level, and timing of last infusion. 1, 5
• The occurrence of a chest infection just 2 weeks ago followed by this acute presentation suggests either inadequate IgG dosing or the need for dose escalation. 1, 5
• Standard IVIG dosing is 0.4-0.6 g/kg/month, but patients with established bronchiectasis may require up to 1.2 g/kg/month based on clinical response. 1

Dose Optimization Considerations

• The primary endpoint is clinical response (reduction in infection frequency and severity), not simply achieving a numerical IgG target. 1, 5
• Breakthrough infections despite "adequate" IgG levels are clinical evidence that current dosing may be insufficient for this patient's disease burden. 5

Prophylactic Antibiotic Consideration

• After resolution of this acute infection, strongly consider adding long-term prophylactic antibiotics, as patients with recurrent bronchitis and pneumonia are at highest risk for developing bronchiectasis and derive the most benefit from continuous prophylaxis. 1, 2
• Prophylaxis may be continued for months, years, or indefinitely depending on clinical need. 1

Critical Pitfalls to Avoid

• Do not delay antibiotics while awaiting culture results—CVID patients can deteriorate rapidly with bacterial infections. 1
• Do not assume the LUQ pain is musculoskeletal—splenic complications in CVID can be life-threatening and require urgent imaging. 3, 1
• Do not discharge without pulmonary imaging—the diffuse crackles may represent progressive bronchiectasis or GLILD, both of which require specific management strategies. 1, 2, 6
• Do not attribute symptoms solely to the recent infection—this may represent a new, distinct infectious process or an opportunistic pathogen. 3, 1

Multidisciplinary Coordination

• Ensure joint care coordination between clinical immunology and respiratory medicine, as all CVID patients receiving immunoglobulin replacement require this collaborative approach. 1, 2
• Consider infectious disease consultation given the complexity of managing infections in primary immunodeficiency. 3