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Table 1 Differential diagnosis: comparison of the features of GVCVID with sarcoidosis

From: The critical role of histology in distinguishing sarcoidosis from common variable immunodeficiency disorder (CVID) in a patient with hypogammaglobulinemia

Parameter

GVCVID

Sarcoidosis

Comment

Clinical features

 Infections

More common

Uncommon

Favours sarcoidosis

 Lymphadenopathy

Common

Common

Does not differentiate

 Evans syndrome

Relatively common

Exceedingly rare

Only two cases described in sarcoid: strongly favours CVID

 Interstitial lung disease

Less common

Common

No obvious interstitial lung disease: favours CVID

 Steroid responsive renal disease

Very rare

Described in sarcoidosis

Strongly favours sarcoidosis

 Raised intracranial pressure

Rare

More common

Favours sarcoidosis

 MRI showing cauda equina involvement

No reports

Very rare

Cauda equina involvement described only in sarcoidosis

Laboratory features

 Switched memory B cells absent

Consistent with CVID

Reduced memory B cells

Favours CVID

 Angiotensin converting enzyme levels (ACE)

ACE levels can be elevated in GVCVID

ACE levels can be normal in sarcoidosis

Non discriminatory

 Absent TRECs

Favours LOCID but on MMF

Not described

Favours CVID

 CSF findings: only increased protein

 

Cells expected

Favours CVID

 Initial IgG normal but subsequent decrease, but normalised after stopping immunosuppression

Decreased

Increased

Strongly favours sarcoidosis and excludes CVID

 Lymph node: disrupted architecture

Disrupted architecture in CVID

Plasma cells and germinal centres present

Strongly favours sarcoidosis

  1. Most clinical and laboratory abnormalities can occur in both disorders. Overall the findings strongly favour sarcoidosis. The normalisation of IgG and the histological findings subsequently excluded CVID. See text for abbreviations