IgG4-Related Disease Presenting as Cervical Lymphadenopathy

Abstract

Cervical lymph node enlargement as the first and sole manifestation of IgG4-related disease (IgG4-RD) is rare and is often difficult to distinguish from lymphoma. Here, we report a case of a 63-year-old man initially presenting with bilateral posterior neck masses. Ultrasonography revealed multiple matted, ovoid, homogenous, hypoechoic, and enlarged lymph nodes below the right parotid gland. In addition, there was heterogeneous echotexture with small and indistinct hypoechoic nodules over bilateral parotid and submandibular glands which suggested sclerosing sialadenitis. Pathology of the tissues obtained by core needle biopsy revealed reactive hyperplasia, but a diagnosis of lymphoma could not be excluded. Subsequently, excisional biopsy and serological tests were done. The diagnosis of IgG4-RD was confirmed due to marked elevation of serum IgG4 levels and pathological evidence of IgG+ and IgG4+ plasma cell infiltration in the lymph node specimen. The patient's neck masses subsided gradually after 1 week of oral steroid therapy. The differential diagnosis of IgG4-RD should always be considered when sclerosing sialadenitis is presented with cervical lymphadenopathy.

Immunoglobulin G4-related periodontitis: case report and review of the literature

“…The recommended dosage is prednisone 0.6 mg /kg/day for 2 to 4 weeks. After 3–6 months, it is gradually reduced to 5 mg/day, and then 2.5–5 mg/day for 3 years [ 37 , 40 , 43 , 44 , 48 ]. The dosage of corticosteroids can be adjusted according to the severity of the patient’s condition.…”

Unique Ultrasonographic Findings of Isolated IgG4-Related Lymphadenopathy

“…Considering that non-IgG4-related lymphadenopathy or even nonspecific reactive lymphadenopathies may show a significant increase in IgG4-positive cells [13][14][15][16] and the present patient had an infection at the ipsilateral lower extremity without a sign of extranodal IgG4-related disease involvement, we had to be very careful in diagnosing IgG4related disease. The diagnostic criteria for IgG4-related disease are as follows [2,11,17]:…”

“…Considering that non-IgG4-related lymphadenopathy or even nonspecific reactive lymphadenopathies may show a significant increase in IgG4-positive cells [ 13 , 14 , 15 , 16 ] and the present patient had an infection at the ipsilateral lower extremity without a sign of extranodal IgG4-related disease involvement, we had to be very careful in diagnosing IgG4-related disease. The diagnostic criteria for IgG4-related disease are as follows [ 2 , 11 , 17 ]: (1) clinical examination showing characteristic diffuse/localized swelling or masses in one or more organs; (2) hematologic examination showing elevated serum IgG4 concentrations (over 135 mg/dL); and (3) histopathologic examination showing marked lymphoplasmacytic infiltration and storiform fibrosis, as well as organ infiltration by IgG4-positive plasma cells. If all three criteria are met, it is considered definite; if the first and third criteria are met, it is considered probable; and if the first and second criteria are met, it is considered possible.…”

“…The lymph node is an organ frequently involved in IgG4-related disease [ 4 ]. Lymphadenopathy in IgG4-related disease usually occurs in two forms [ 5 , 6 , 11 ]. First, it occurs in the form of localized or generalized lymphadenopathy associated with an extranodal lesion.…”

“…However, image findings, including US, of IgG4-related lymphadenopathy are not well known so far. Only Wu et al [ 11 ] reported on US imaging of an IgG4-related disease that occurred in the form of cervical lymphadenopathy.…”