Range of diseases with elevated serum IgG4 levels
| Final diagnoses | Number of cases (n=190) |
|---|---|
| Definite IgG4-RD | 27 |
| Probable IgG4-RD | 38 |
| Chronic sinusitis | 46 |
| Recurrent pneumonia | 29 |
| Immunodeficiency | 10 |
| IgG deficiency | 4 |
| IgA deficiency | 3 |
| Common variable immunodeficiency | 2 |
| CD4 lymphocytopenia | 1 |
| Connective tissue disease | 11 |
| Sjögren's syndrome | 3 |
| Mixed connective tissue disease | 2 |
| Undifferentiated vasculitis | 2 |
| Systemic lupus erythematosus | 1 |
| Churg–Strauss syndrome | 1 |
| Behcet's syndrome | 1 |
| Rheumatoid arthritis | 1 |
| Pancreato-biliary disease | 9 |
| Alcoholic pancreatitis | 2 |
| Alcoholic hepatitis | 1 |
| Autoimmune hepatitis | 1 |
| Cholangiocarcinoma | 1 |
| Choledocholithiasis | 1 |
| Cholestatic hepatopathy | 1 |
| Cholestatic hepatopathy | 1 |
| Steatohepatitis | 1 |
| Interstitial lung disease | 5 |
| Interstitial pulmonary fibrosis | 2 |
| Pulmonary sarcoidosis | 2 |
| Bronchopulmonary aspergillosis | 1 |
| Infectious disease | 4 |
| Invasive aspergillosis | 1 |
| Osteomyelitis | 1 |
| Recurrent infection | 1 |
| Recurrent otitis media | 1 |
| Aortitis | 3 |
| Abdominal aortitis | 2 |
| Retroperitoneal fibrosis (asbestos) | 1 |
| Leukemia | 2 |
| Acute lymphoblastic leukemia | 1 |
| Chronic myelomonocytic leukemia | 1 |
| Miscellaneous | 6 |
| Chronic sialadenitis | 1 |
| Haemochromatosis | 1 |
| Hypereosinophilic syndrome | 1 |
| Pachymeningitis (idiopathic) | 1 |
| Pompe disease | 1 |
| Small-fiber neuropathy | 1 |