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IgG 4 Sub class
Blood
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No Fasting Required
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Measures IgG4 levels.
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IgG 4 Subclass Test Guide
- Why is it done?
- Measures serum IgG 4 antibody levels, a specific immunoglobulin G subclass involved in immune responses
- Diagnoses IgG 4-related disease (IgG4-RD), a systemic fibroinflammatory condition affecting multiple organ systems
- Evaluates autoimmune pancreatitis (type 1), the most common form associated with elevated IgG 4 levels
- Investigates chronic sclerosing conditions affecting salivary glands, lacrimal glands, and bile ducts
- Monitors response to immunosuppressive therapy in confirmed IgG4-RD cases
- Assesses patients with persistent lymphadenopathy or mass lesions of unknown etiology
- Performed when clinical presentation suggests IgG4-RD or when other autoimmune diagnoses have been ruled out
- Normal Range
- Normal Reference Range: 4-86 mg/dL (40-860 mg/L) in adults; varies slightly by laboratory
- Units of Measurement: mg/dL (milligrams per deciliter) or mg/L (milligrams per liter)
- Normal Result: IgG 4 levels within laboratory reference range indicate absence of IgG4-RD; typically comprises 3-4% of total IgG
- Elevated Result: IgG 4 >135 mg/dL (>1350 mg/L) is considered significantly elevated; levels >200 mg/dL strongly suggest IgG4-RD
- Borderline Values: IgG 4 levels between 86-135 mg/dL require clinical correlation and may warrant repeat testing
- IgG 4/Total IgG Ratio: >40% indicates increased IgG 4 proportion; normal ratio is <4%; elevated ratio supports IgG4-RD diagnosis
- Note: Normal IgG 4 levels do not completely exclude IgG4-RD; diagnosis requires clinical, serological, and histological correlation
- Interpretation
- Moderately Elevated (86-135 mg/dL): May suggest early IgG4-RD or other conditions; requires correlation with clinical symptoms, imaging findings, and histopathology
- Markedly Elevated (>135 mg/dL): Consistent with IgG4-RD diagnosis, particularly when IgG 4/total IgG ratio >40%; higher levels correlate with disease activity
- Very High Levels (>200 mg/dL): Strongly indicative of active IgG4-RD; associated with multi-organ involvement and potential disease complications
- IgG 4/Total IgG Ratio Interpretation: Ratio >40% strengthens IgG4-RD diagnosis; ratio <40% with elevated absolute IgG 4 suggests other etiologies
- Factors Affecting Results: Active infection (viral, bacterial), other autoimmune conditions, malignancies, immunosuppressive therapy effects, and specific immunizations can influence IgG 4 levels
- Longitudinal Monitoring: Decreasing IgG 4 levels during treatment indicate therapeutic response; persistent elevation suggests inadequate disease control
- Clinical Significance: Elevated IgG 4 combined with characteristic histology (IgG 4-positive plasma cell infiltration >10 cells/HPF) confirms IgG4-RD diagnosis
- Negative Result Does Not Exclude Disease: Up to 30% of biopsy-proven IgG4-RD patients may have normal serum IgG 4 levels; localized disease may show normal systemic levels
- Associated Organs
- Pancreas: Type 1 autoimmune pancreatitis most commonly associated with IgG4-RD; can lead to pancreatic insufficiency and diabetes mellitus
- Salivary Glands: Chronic sclerosing sialadenitis affecting submandibular and parotid glands; presents with xerostomia and recurrent infections
- Lacrimal Glands: IgG4-RD involvement causes chronic sclerosing dacryoadenitis leading to dry eye (xerophthalmia) and visual complications
- Biliary System: IgG4-associated cholangitis and sclerosing cholangitis affecting common bile duct and intrahepatic ducts; risk of biliary strictures and cholangiocarcinoma
- Lymph Nodes: Generalized lymphadenopathy with IgG4-positive plasma cell infiltration; can mimic lymphoma
- Retroperitoneum: Retroperitoneal fibrosis causes chronic inflammation; may present with abdominal or back pain and potential ureteral obstruction
- Mediastinum: Mediastinal fibrosis and mass formation; may involve lungs with pulmonary nodules or interstitial lung disease
- Kidney: IgG4-associated nephropathy with tubulointerstitial nephritis; may progress to chronic kidney disease if untreated
- Vascular System: Large vessel vasculitis affecting aorta and branch vessels; risk of aortic aneurysm formation and dissection
- Associated Complications: Organ dysfunction from fibrosis, increased malignancy risk (particularly pancreatic cancer), and potential life-threatening vascular complications if disease progression not controlled
- Follow-up Tests
- Tissue Biopsy (Histopathology): Essential for confirming IgG4-RD diagnosis; demonstrates IgG 4-positive plasma cells >10 cells/high-power field and IgG 4/IgG ratio >40%
- Imaging Studies: CT or MRI of affected organs to assess extent of fibrosis, mass formation, and organ involvement; baseline imaging for disease monitoring
- Total IgG and IgG Subclasses: Measure total IgG and calculate IgG 4/total IgG ratio for diagnostic confirmation; assess other IgG subclasses
- Complete Blood Count (CBC): Monitor for lymphocytosis, thrombocytopenia, or anemia associated with systemic disease
- Comprehensive Metabolic Panel: Assess liver function, kidney function, and electrolytes; critical for monitoring organ involvement and therapy effects
- Erythrocyte Sedimentation Rate (ESR) and C-Reactive Protein (CRP): Markers of systemic inflammation; useful for monitoring disease activity during treatment
- Autoimmune Markers: Antinuclear antibody (ANA), anti-smooth muscle antibody (ASMA), and rheumatoid factor to exclude other autoimmune conditions
- Organ-Specific Tests: Pancreatic enzymes (amylase, lipase), liver function tests, chest imaging if respiratory involvement suspected
- Periodic IgG 4 Monitoring: Repeat testing at 3-6 month intervals during initial treatment phase to assess therapeutic response
- Maintenance Monitoring: Annual or biannual IgG 4 levels during maintenance therapy; more frequent if clinical deterioration suspected
- Malignancy Screening: Regular surveillance imaging and tumor markers given increased cancer risk associated with IgG4-RD, particularly pancreatic adenocarcinoma
- Fasting Required?
- Fasting Requirement: No
- The IgG 4 subclass test does not require fasting; serum IgG 4 levels are not affected by food intake or recent meals
- Patient can eat and drink normally before the test; no dietary restrictions are necessary
- Medications: May be taken as usual; most medications do not interfere with IgG 4 measurement; inform phlebotomist of current immunosuppressive therapy
- Recent Immunizations: Inform healthcare provider if immunization received within 1-2 weeks; may temporarily elevate immunoglobulin levels
- Sample Collection: Blood sample collected via venipuncture into serum separator tube (SST) or appropriate tube per laboratory protocol
- Timing Considerations: Test can be performed at any time of day; no specific time restriction exists
- Hydration: Normal hydration recommended; severely dehydrated patients may have artificially elevated immunoglobulin levels
- Active Infection: Ideally, test should be performed when acute infection not present, as active infection may transiently elevate IgG 4 levels
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