Immunoglobulin G4-related disease (IgG4-RD) is a chronic immune-mediated fibroinflammatory disorder that often manifests with tumor-like masses and/or painless enlargement of multiple organs. Serum IgG4 level is often but not always elevated. Symptoms depend on which organs are affected. Diagnosis typically requires biopsy. Treatment is with corticosteroids and sometimes rituximab.

IgG4 is the least common of the 4 subtypes of IgG. Its function likely varies with the context; in allergic disease, it is thought to have an immune-inhibitory role in preventing anaphylactic reactions to allergens. It has also been reported to have a role in autoimmunity and malignancy, but its function in these contexts is less well established. IgG4-RD has a wide range of manifestations that are unified by their histopathologic findings and response to treatment.

Most patients are middle-aged to older men, but the disorder can affect people of any age and sex.


Common general manifestations of IgG4-RD include lymphadenopathy and weight loss. Weight loss is particularly common when there is multiple organ involvement and/or exocrine pancreatic insufficiency. Fever is highly uncommon in IgG4-RD and should prompt consideration of alternative diagnoses.

Other manifestations are specific to the affected organs.

Pancreatic involvement may be painless, sometimes with jaundice if there is an obstructing pancreatic mass, or may cause abdominal pain and nausea if acute pancreatitis is present. Some patients present with a more smoldering and insidious chronic pancreatitis and symptoms of exocrine pancreatic insufficiency (eg, flatulence, abdominal distention, steatorrhea, undernutrition, weight loss), and/or endocrine pancreatic insufficiency (eg, asymptomatic hyperglycemia or frank diabetes mellitus).

Retroperitoneal fibrosis most often manifests with flank or back pain but is often asymptomatic and identified incidentally on abdominal imaging. Aortitis is almost always asymptomatic and identified only incidentally by imaging or postoperatively after aortic resection.

Salivary and lacrimal gland involvement usually causes painless, bilateral enlargement but may be asymmetric. Dry mouth and/or eyes is uncommon.

Orbital involvement may cause proptosis, orbital pain, periorbital edema, or pain with extraocular movements.

Pulmonary involvement may be asymptomatic, or cause cough, dyspnea, or pleurisy.



Serum IgG4 level

Serum complement levels (C3 and C4)

Selective imaging


Homoeopathy today is a rapidly growing system and is being practiced all over the world. It strength lies in its evident effectiveness as it takes a holistic approach towards the sick individual through promotion of inner balance at mental, emotional, spiritual and physical levels. When  is concerned there are many effective medicines available in Homoeopathy , but the selection depends upon the individuality of the patient , considering mental and physical symptoms

Few homoeopathic medicine can be thought of in the treatment of Immunoglobulin G4-related disease are:

lycopodium, chelidonium, opium, arsenic alb., nux vom., etc.