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A 50-year-old woman was referred to our hospital for shoulder joint stiffness. She had a history of polyclonal hypergammaglobulinemia and an elevated C-reactive protein level. Her laboratory data revealed an elevated serum immunoglobulin G4 (IgG4) level, hypergammaglobulinemia, and rheumatoid factor positivity in the absence of anticyclic citrullinated peptide antibody. [18 F ]-Fluorodeoxyglucose positron emission tomography showed significant [18 F ]-fluorodeoxyglucose uptake in multiple lymph nodes (axillary, hilar, para-aortic, and inguinal). Biopsy of the inguinal lymph node showed expansion of the interfollicular areas by heavily infiltrating plasma cells, consistent with multicentric Castleman disease (MCD). Immunohistochemical analysis revealed a 37.3% IgG4-positive:IgG-positive plasma cell ratio, indicating overlapping IgG4-related disease. However, serological cytokine analysis revealed elevated levels of interleukin-6 (9.3 pg/ml) and vascular endothelial growth factor (VEGF) (1210 pg/ml), which are compatible with MCD. Corticosteroid treatment resolved the serological and imaging abnormalities. IgG4-related disease can mimic MCD, and it is crucial to distinguish between these two diseases. Serum interleukin-6 and VEGF levels may help to discriminate MCD from IgG4-related disease.

Immunoglobulin G4 (IgG4)-related disease is a relatively recently proposed clinical-pathologic entity that is characterized by fibro-inflammatory lesions rich in IgG4-positive plasma cells and, often but not always, elevated serum IgG4 concentrations. IgG4-related disease was recognized as a systemic disease in 2003, when extrapancreatic manifestations were identified in patients with autoimmune pancreatitis. Since then, the disease has been reported as affecting virtually every organ system and has been identified in the biliary tree, salivary and lacrimal glands, periorbital tissues, lungs, lymph nodes, thyroid gland, kidneys, prostate gland, testicles, breasts, and pituitary gland. Its pathogenesis is poorly understood, but findings are consistent with both an autoimmune and an allergic disorder. Although definitive diagnosis requires histopathologic analysis, imaging plays an important role in demonstrating infiltration and enlargement of involved organs. Because of the systemic nature of the disease, imaging workup of IgG4-related disease should always include whole-body examinations to detect multiorgan involvement. Patients often present with subacute development of a mass in or diffuse enlargement of the affected organ, sometimes mimicking a neoplastic process. In every anatomic location, several inflammatory and neoplastic entities must be considered in the differential diagnosis. Because IgG4-related disease usually shows a marked response to corticosteroid therapy, radiologists should be familiar with its clinical and imaging manifestations to avoid a delay in diagnosis and unnecessary surgical interventions., ((©)RSNA, 2015.)

Immunoglobulin G4-related disease is a recently described systemic clinicopathological entity characterized by immunoglobulin G4-producing plasmacytic infiltration of tissue and frequently by elevated serum immunoglobulin G4 concentration. Manifestations of this disease have been documented in nearly all organs and locations, but coronary artery involvement is not widely recognized. We report the coronary findings of a patient with multi-organ immunoglobulin G4-related disease. Non-electrocardiogram-gated computed tomography of the chest demonstrated nodular and rind-like periarterial soft tissue thickening along the proximal coronary artery segments with improvement following steroid therapy.

IgG4-related ocular adnexal disease, a relatively recently described clinical entity, is important to diagnose because during the acute phase, it responds favorably to corticosteroid treatment. The diagnosis can be confirmed by simple blood tests and histology. IgG4-related dacryoadenitis and generalized orbital disease have been reported; however, this is the first report of IgG4-related disease of the lacrimal sac. We describe an 80-year-old female who presented with a painless erythematous indurated swelling in the right lacrimal sac area with complete obstruction of the right nasolacrimal system. A 9-mm lacrimal sac mass was noted on CT dacryocystogram. Blood tests revealed an elevated serum IgG4 level, and the lacrimal sac histology was characteristic of IgG4-related disease. Corticosteroid treatment was declined by the patient. She was kept under close observation for signs of progression, systemic involvement, and potential malignant transformation.

Objective: This article will illustrate and describe the spectrum of diseases associated with air cysts at high-resolution CT (HRCT)., Conclusion: HRCT is an important modality in the evaluation of interstitial lung disease to include cystic lung disease. Although most commonly associated with lymphangioleiomyomatosis or Langerhans cell histiocytosis, cystic lung disease is increasingly being recognized as a feature of other entities. Awareness of the spectrum of HRCT findings associated with these diseases may help the trained observer narrow the differential diagnosis.

Objective: Hyper-IgD syndrome (HIDS) is a rare autosomal recessive disease characterized by recurrent fever, lymphadenopathy, diarrhoea, abdominal pain, headache, arthralgia and skin rash. Abdominal symptomatology may mimic inflammatory bowel disease. We report on four patients with HIDS who had been previously investigated for Crohn's disease (CD). The levels of IgD were measured in a series of patients with CD to evaluate the specificity of this assay in the differential diagnosis between the two conditions., Material and Methods: Diagnosis of HIDS was based on clinical criteria as well as immunological or genetic data. IgD levels were measured in the four subjects affected by HIDS, in 59 patients with CD and in a group of 160 healthy controls., Result: All patients underwent a variety of gastroenterological investigations because inflammatory bowel disease was suspected. Ultrasonography was pathologic in all the patients, showing enlargement of mesenteric lymph nodes. Abdominal leucocyte scintigraphy displayed diffuse signals of mild to moderate degree. IgD and IgA levels were elevated in three out of four patients. No difference in IgD values was found in CD patients as compared to the control group., Conclusions: Gastrointestinal complaints associated with recurrent fever and mesenteric adenopathy warrant genetic investigation for HIDS, in order to avoid unnecessary invasive investigations and treatment.

A 71-year-old woman was referred to our hospital because of hyperproteinemia and serum M-protein (IgG-lambda type). Chest computed tomographic (CT) scan revealed a tumor in each lung and transbronchial lung biopsy was performed. Histopathological examination showed monotonous medullary proliferation of morphologically mature plasma cells. These cells were cIgG+, cIg-lambda+, CD 20+, CD 79 a+, CD 138+, cIg-kappa-, and CD3-. Since there were very few non-neoplastic plasma cells and small lymphocytes in addition to the absence of reactive lymph follicles and fibrosis, the patient was diagnosed as having plasmacytoma. There was no proliferation of plasma cells in the bone marrow. Thus, the lesion was finally characterized as primary pulmonary plasmacytoma. Treatment with melphalan/prednisolone resulted in considerable decrease in the serum IgG level and regression of the pulmonary tumors. The effectiveness of the chemotherapy could confirm our diagnosis, although MALT-type lymphoma with plasmacytic differentiation cannot be completely ruled out.

The aim of this study was to evaluate selected diagnostic and clinical aspects of chronic hepatitis C (CH-C) in the group of 80 patients: 68 males aged 24-65 (mean 39.8 +/- 10,5) and 12 females aged 35-66 (mean 48.7 +/- 12.6). The epidemiological data allowed to divide the basic group into 3 subgroups: patients with transfusion-associated CH-C (subgroup I: 12 males, mean age 38 +/- 6.7 and 2 females aged 40 and 46), CH-C patients with parenteral hepatitis C virus exposure-other than blood transfusion (subgroup II: 25 males, mean age 40.6 +/- 8.2 and 5 females aged 43 +/- 15.1) and sporadic cases with unknown HCV exposure (subgroup III: 31 males, mean age 38.2 +/- 11.2 and 5 females, mean age 50.5 +/- 10.3). The duration of the disease (CH-C) was calculated from the incident of acute viral hepatitis or the first signs of liver damage caused by HCV to the confirmation of CH-C by liver biopsy. The following data were analyzed: a frequency of acute viral hepatitis with jaundice at the beginning of the disease, ALT flare-ups, mean highest activities of ALP and GGT, frequency of hypergammaglobulinaemia and sings of fatty liver in ultrasonographic finding (USG). In all patients but one anti-HCV antibodies (ELISA 2nd generation test by Abbott) were detected. In 64/80 subjects antibodies to HCV antigens: 5-1-1, C 100-3, C 33c and C 22 were determined by RIBA-2 test (Ortho). In 62/80 patients HCV-RNA in serum was determined by RT PCR. Liver biopsy was performed in 71/80 patients. Other co-existent liver diseases were excluded. The similarity between 3 subgroups was shown: similar percentage of males and females, similar patients mean age and the duration of the disease. It was shown that the acute beginning of the disease with jaundice has been observed twice as frequent in subgroups I and II compared with subgroup III. The same frequency of ALT flare-ups in all subgroups was observed (25-28.6%). No differences in mean highest ALP and GGT activities in 3 subgroups were observed. It was shown, however, that hypergammaglobulinaemia was detected more frequently in subgroup III (30.5%) compared with subgroup I (7.1%) and II (16.7%). The signs of fatty liver in ultrasonographic findings were also observed more frequently in subgroup III (30.5%) than in subgroup I (14.3%) or II (16.7%). In all patient but one, in which anti-HCV antibodies by ELISA test were detected, anti-C 33c and anti-C 22 antibodies by RIBA were present. HCV-RNA in serum was detected in 77.8% subjects from subgroup I. 73.9%-from subgroup II and 66.7%-from subgroup III. In all HCV-RNA positive patients anti-HCV antibodies were detected. The evidence of chronic active hepatitis confirmed by liver biopsy was shown in 63.6%, 67.8% and 71.8% of patients from subgroup I, II and III, respectively. In no case normal liver morphology was present. Authors concluded the distressing fact of the high incidence of chronic active hepatitis in patients unaware of HCV infection, without the incident of acute hepatitis at the beginning of the disease (over 1/3 of all described subjects). The differences of the clinical course of the disease between subgroups 1 + II and subgroup III suggest two different routes of HCV infection or the presence of two different HCV mutants in Polish population. Authors emphasise the necessity of HCV gene typing in CH-C patients, which might explain the surprisingly high incidence of chronic active hepatitis in the reported group. The use of the presented data for the general practitcioner making the diagnosis of crytogenetic liver disease is also accentuated.

Between 1985 and 1990, bi- or triclonal gammopathies were diagnosed in 6 patients (4 women and 2 men) aged 86-93 years. These gammopathies were diagnosed based on the existence of 2 or 3 monoclonal immunoglobulin peaks separated by immunoelectrophoresis of serum proteins (a technique using monospecific anti-gamma, -kappa and -lambda antisera, followed by immunofixation). The 2 biclonal gammopathies were associated with malignancies: on bronchial small cell carcinoma, the other colon cancer; both patients died soon after the discovery of their respective tumors. The 4 triclonal gammopathies had the following associations: 1 non-Hodgkin's large-cell lymphoma, 1 breast cancer with possible lymphoma and 2 severe inflammatory pathologies that we were unable to further identify; 3 patients in this group died. We present these data in light of the rarity of reported cases of bi- and triclonal gammopathies and the controversies surrounding their possible association with a malignant pathology.

A 27 year old white woman with a history of chronic eczema and episodes of serious infection of the chest, skin, and bone presented with acute respiratory failure. She was found to have a spontaneous right pneumothorax and a pneumatocele in the left upper lobe. Despite a left upper lobectomy she was left with chronic respiratory failure, bullous lung disease, and bilateral bronchiectasis. The hyperimmunoglobulinaemia E and recurrent infections syndrome was diagnosed only in adult life.

Bilateral pulmonary uptake in an asymptomatic patient with normal chest X-rays and a monoclonal gammopathy is described. At the 11-month follow-up the patient is asymptomatic and chest X-ray remains normal.

A 65-year-old woman with polyclonal hypergammaglobulinemia developed acute renal failure requiring hemodialysis after an intravenous pyelogram (IVP). An increased risk of renal injury after IVP for patients with polyclonal hypergammaglobulinemia has not been previously recognized, although a parallel risk to patients with myeloma has been well established. At autopsy, immunoperoxidase staining of the bone marrow demonstrated plasma cell subpopulations with intracellular staining for IgG, IgA, and IgM and chi and lambda light chains, proving that this patient did not have occult myeloma. Immunoperoxidase staining of casts in renal tubules also demonstrated polyclonal immunoglobulins.

Hyperimmunoglobulinemia E (Job or Buckley) syndrome is an immunodeficiency disease associated with recurrent pyogenic infections, chronic eczematoid dermatitis, and mucocutaneous candidiasis. Association of this condition with osteoporosis and recurrent fractures is presented by a case report and by review of other reports describing similar findings. Other immunodeficiency syndromes with associated skeletal abnormalities are discussed.

Susceptibility to recurrent staphylococcal cutaneous and respiratory infections beginning in infancy associated with extreme hyperimmunoglobulinemia E is a recently described primary immunodeficiency syndrome. Other clinical features include depressed cellular immunity and deficient antibody formation. Recurrent pneumonia and cyst formation with variable persistence and expansion characterized the radiographic couse in 11 patients. Five cysts resolved with continuous antistaphylococcal therapy; 2 were resected without recurrence; and 4 persisted after surgery and/or antibiotics (2--8 years). The cysts had dense, necrotic surfaces with fibrous walls, eosinophilic and other inflammatory cell infiltrates, and frequent, persistent, bronchial connections. Sinusitis (9/9) and mastoiditis (3/4) were also observed radiographically.