Background
Methods
Data source, study selection and data extraction
Results
Reference | Age | Sex | Organ manifestation | Serum IgG4 | Therapy | Comments |
---|---|---|---|---|---|---|
Miglani 2010 [24] | 13y | M | AIP-1
H+
| El (603 mg/dl) | Pred 20 mg/d | Initially suspected of malignancy. Pred tapered and stopped in 4 months. |
Ibrahim 2010 [25] | 3y | F | IgG4-R cholangitis
H+
| El (258 mg/dl) | Pred 2 mg/kg/d and Aza 1.5 mg/kg | Relapse after tapering pred and required a low (2 mg/d) maintenance dose of pred and Aza. |
Mannion 2010 [14] | 13y | F | AIP-1 and IgG4-R fibrosing mediastinitis, renal and hepatic manifestation
H+
| El (73.4 mg/dl) | Pred and MMF | Good results by MMF, pred tapered and stopped successfully. |
Zakeri 2011 [20] | 17y | M | Riedel’s thyroiditis H + a
| NM | Pred 40 mg/d | Pred tapered and stopped in 3 months. |
Melo 2012 [28] | 11y | M | IgG4-R sialadenitis
H+
| NM | Pred | |
Griepentrog 2013 [7] | 10y | F | IgG4-ROD
H+
| N (L U) | Lateral orbitotomy | No further treatment was required. |
Griepentrog 2013 [7] | 14y | F | IgG4-ROD
H+
| N (L U) | Pred, dosage unknown, and MMF | MMF because of relapse after tapering pred, successful. |
Kalapesi 2013 [10] | 5y | F | IgG4-ROD
H+
| El (1.52 g/l) | Pred 1 mg/kg and MMF (600 mg/m2) | Weaned off pred and maintained on MMF successfully. |
Naghibi 2013 [15] | 16y | F | IgG4-related colitis, in the past AIP-1
H+
| El (210 mg/dl) | Adalimumab | Refractory disease to pred 0.5 mg/kg, Aza and infliximab. Adalimumab successful. |
Pifferi 2013 [26] | 15y | M | IgG4-R pulmonary disease
H+
| El (1090 mg/dl) | Pred 0.6 mg/kg/d | Treatment for 4 weeks. |
Sane 2013 [11] | 12y | F | IgG4-ROD and nephrotic syndrome
H+
| N (L U) | Methylpred and rituximab | The nephrotic syndrome also resolved. Initial good response to pred 40 mg, but relapse occured. |
Pasic 2013 [12] | 10y | F | Mikulicz disease/IgG-ROD
H+
| EL 9.02 g/l | NM | |
Caso 2013 [16] | 17y | M | IgG4-R lymphad and scleritis
H+
| El (4.43 g/l) | Rituximab and pred 10 mg daily | Refractory to MMF, good results with rituximab. |
Hasosah 2014 [17] | 7y | F | IgG4-R mesenteritis and pericarditis
H+
| El (149 mg/dl) | Pred, aza and colchicine (doses unknown) | Relapsed despite aza, further treatment with 5 mg prednisone as maintenance therapy. |
Jariwala 2014 [5] | 7y | M | IgG4-ROD
H+
| El (109.3 mg/dl) | Pred 1 mg/kg/d and Aza 2 mg/kg/d | Good clinical results. |
Mittal 2014 [6] | 14y | M | IgG4-ROD
H+
| El (4.3 g/l) | Pred 0.6 mg/kg/d | Initial improvement, but lost to follow-up. |
Notz 2014 [29] | 13y | F | IgG4-R dacryoadenitis
H+
| N (23.9 mg/dl) | Pred 40 mg/d for 3 months | |
Prabhu 2015 [8] | 15y | F | IgG4-ROD and sinonasal disease
H+
| El (579 mg/dl) | Rituximab | Insufficient response to prednisone. |
Prabhu 2015 [8] | 15 y | F | IgG4-R sinonasal disease
H+
| El (206 mg/dl) | Pred (dosage unknown) | |
Batu 2015 [9] | 14y | F | IgG4-ROD
H+
| N (7.5 g/l) (0-12.5 g/l) | Pred (dosage unknown) | Pred was tapered and stopped, MTX as maintenance therapy. |
Batu 2015 [9] | 9y | F | IgG4-ROD
H+
| N (3.7 g/l) | Methylpred and cyclophosphamide | No response to pred, MTX or MMF. Now stable disease. |
Corujeira 2015 [18] | 22Mo | F | IgG4-R pulmonary disease and IgG4-R lymphad
H+
| El (805 mg/dl) | Pred 2 mg/kg/d | Pred tapered over period of 6 months. |
Gillispie 2015 [13] | 7y | F | IgG4-ROD, nerve and renal disease
H+
| N (L U) | Pred and rituximab | Refractory to pred, responsive to rituximab. |
Nada 2015 [19] | 10y | M | IgG4-R hepatic mass and coagulopathy
H+
| El (420 mg/dl) | Pred 2 mg/kg/d | Coagulopathy also resolved after treatment. |
Rosen 2015 [27] | 17y | M | IgG4-R cholangitis
H+
| El (242 mg/dl) | Pred 30 mg/d | Pred weaned in 3 months. |
Patients
Organ manifestation
Diagnosis
Therapy
Discussion
Epidemiology
Symptoms and organ manifestation
Pancreas Autoimmune pancreatitis type 1 | Lymph nodes Ig4-related lymphadenopathy of several lymph nodes |
Liver and bile duct IgG4-related sclerosing cholangitis IgG4-related cholecystitis IgG4-related hepatopathy | Other abdominal manifestations Inflammatory pseudotumors Retroperitoneal fibrosis Small bowel obstruction caused by peritoneal IgG4-RD IgG4-RD of stomach with chronic ulcer IgG4-related esophagitis |
Kidneys Interstitial nephritis Glomerular lesions such as membranous nephropathy | Skin manifestation Erythematous, subcutaneous papules or nodules of IgG4 origin |
Urological manifestation IgG4-related prostatitis Ureteral IgG4-RD Testicular inflammation as a manifestation of IgG4-RD | Orbital and ophthalmic manifestation Inflammatory pseudotumors of orbit Scleritis Retinopathy due to IgG4-RD with hypergammaglobulinemic hyperviscosity Trigeminal and orbital nerve compression Nasolacrimal duct obstruction |
Pulmonary manifestation Interstitial lung disease/interstitial pneumonia Bronchial damage/asthma-like clinical presentation Plural manifestation of IgG4-disease Pulmonary arterial hypertension | Cardiovascular manifestation IgG4-related periaortitis IgG4-related aortitis Pericarditis IgG4-related coronary artery disease |
Thyroid Riedel’s thyroiditis Fibrosing Hashimoto thyroiditis | Salivary and lacrimal gland IgG4-RD Mikulicz’s Küttner's tumor or IgG4-related submandibular gland disease |
Nervous system Infundibular hypophyistis Hypertrophic pachymeningitis IgG4-related hypophysitis Intracerebral inflammatory pseudotumors Neuropathy | Other manifestations IgG4-related fibrosing mediastinitis IgG4-related myositis Multifocal fibrosclerosis Increased risk of malignancy: lung, colon and especially MALT lymphoma. |