Israel Medical Association Journal

Background: Immunoglobulin G4-related disease (IgG4-RD) is a chronic, immune-mediated condition characterized by fibro-inflammatory lesions with lymphoplasmacytic infiltration. Diagnosis traditionally relies on histopathological findings, including the presence of IgG4+ plasma cells. However, due to challenges in biopsy accessibility, additional measures are needed to facilitate diagnosis.

Objectives: To identify additional parameters for characterizing IgG4-RD patients.

Methods: We compared several circulating factors between a cohort of patients with IgG4-RD disease seen at our hospital between 2017 and 2023 and healthy controls.

Results: Among 16 suspected patients, 13 were confirmed to have IgG4-RD, and 3 were classified as highly likely. Comparison with controls revealed differences in white blood cell count (WBC) (Folf change (FC) 1.46, P < 0.05), plasmablasts (FC 3.76, P< 0.05), plasmablasts CD38 (FC 1.43, P < 0.05), and CD27 (FC 0.66, P = 0.054), thus highlighting potential markers for IgG4-RD diagnosis. Treatments with steroids/rituximab tend to reduce plasmablast (FC 0.6) and IgG4 (FC 0.28) levels and to increase Gal-3 levels.

Conclusions: Levels of plasmablasts are a significant diagnostic feature in IgG4-RD. Healthy individuals have a lower level of plasmablasts. Elevated Gal-3 in serum of patients with IgG4-RD suggests a role in plasmablast activation. CD38/CD27 expression by plasmablasts emerges as a potential marker. Further research on a larger cohort is needed to confirm these findings.

Retroperitoneal infiltration, also known as retroperitoneal fibrosis (RPF), is a rare condition, which mostly occurs in men over the age of 40 years. This condition involves inflammation of the soft tissue of the retroperitoneal cavity, most commonly around the infrarenal abdominal aorta, iliac arteries, ureters, and abdominal organs. Clinical manifestations consist of severe pain in the lower back, abdomen, or flank, which may radiate to the inguinal region. Pain may be acute at the onset and can be mistaken for renal colic. Renal and ureteral involvement is common and can develop into acute kidney injury and hypertensive crises.

Autoimmune pancreatitis (AIP) is a rare disease that has been classified into two subtypes. Type 1 is believed to be mediated by immunoglobulin G4 (IgG4) and type 2 is related to granulocytic epithelial lesions, but the pathogenetic mechanisms in both are still unknown. The patho-mechanism of AIP type 1 is suggested to be secondary to autoimmunity or allergy due to the increased serum IgG4 and immunoglobulin E levels, abundant infiltration of IgG4, plasmacytes and lymphocytes in the pancreas, and fibrosis. Both types of AIP respond to steroid treatment. The relapse rate after remission is high and reaches 30–50% within 6–12 months in AIP type 1; however, in AIP type 2 relapse is rare. The maintenance therapy and therapeutic strategy for relapsing patients with type 1 is managed with low dose steroids, however there are no consensus guidelines. In this review we discuss the current understanding of AIP, highlighting the emerging potential role of eotaxin in pathogenesis, classification, and management of the disease

Background: The distribution of pathology and clinical characteristics of lacrimal gland diseases are different in different areas of the world.

Objectives: To evaluate the incidence rate, patient characteristics, and indications for surgical intervention of lacrimal gland lesions in a tertiary care center in Israel.

Methods: All biopsied or surgically removed lacrimal gland lesions at the Goldschleger Eye Institute from 2009 to 2015 were identified. The following data were collected: age, gender, indications for surgical intervention, diagnosis, treatment, and prognosis.

Results: We evaluated 28 lacrimal gland biopsies from 26 patients (11 men, 15 women). Mean age at biopsy was 47.5 years old. The most common presenting symptoms were: eyelid swollenness (57.14%), ptosis (32.14%), and proptosis (10.71%). All patients underwent computed tomography and magnetic resonance imaging. In 28 cases, infiltrations of the lacrimal gland were found. In nine cases infiltration of muscles or orbital extension were found. The most common pathologies were non-specified inflammation (44.82%), lymphoma (20.68%), and immunoglobulin G4-related disease (10.34%). The treatment was diverse according to the patient diagnosis. Prognosis of lacrimal gland disease was good; however, in five patients the systemic disease progressed.

Conclusions: Lesions of the lacrimal gland comprise a wide variety of pathological findings that require different treatment strategies. Lacrimal gland biopsies enable physicians to precisely recognize the pathology; therefore, it is important to consider this surgical method in any patient with lesions in the lacrimal gland.

Background: Human parvovirus B19 is a global and common infectious pathogen in humans, primarily in children.

Objectives: To assess the immunoglobulin G seroprevalence of B19 in children in Israel.

Methods: Overall, 128 previously healthy children (1.5–17 years old) hospitalized for various diseases other than acute human parvovirus B19 infection were assessed for IgG[1] to the virus by enzyme-linked immunosorbent assay. 

Results: The IgG seroprevalence increased from 22% in children aged 1.5–9 years to 52% in older children (P = 0.001).

Conclusions: Our data suggest that most acute parvovirus B19 infections in Israel occur in the early school years, and that by 18 years of age 50% of Israeli children have been infected by the virus.

[1] IgG = immunoglobulin G

Background: Recent studies have suggested a possible association between Chlamydia pneumoniae infection and coronary heart disease.

Objectives: To determine titers of antibodies to Chlamydia pneumoniae in patients with acute  myocardial infraction compared with titers in several control groups.

Methods: This prospective case-control study investigated 209 individuals. We assessed the serum IgG antibody titers to Chlamydia pneumoniae in 57 consecutive patients admitted with AMI to our intensive coronary care unit during a 4 month period. A serum sample was drawn upon admission after 6 weeks. Results were compared with those of four control groups: a) patients admitted with community-acquired pneumonia (n=18), b) patients with community-acquired urinary tract infection (n=42), c) patients with angiographically normal coronary artery disease (n=44), and d) patients with stable coronary artery disease (n=48). Serum immunoglobin G antibody titers to C. pneumoniae were determined using standard micro-immunofluorescene technology.

Results: Of 57 patients with AMI, 32 (56%) had a high lgG titer to C. pneumoniae (>=1:256) on the initial test, which remained unchanged (62%) after 6 weeks. The percentage of patients with high titers was significantly lower in the control groups: 5 of 18 patients (28%) in the pneumonia group (P<0.01), 11 of 42 (26%) in the urinary tract infection group (P<0.01), 11 of 44 (25%) with normal coronary arteries (P<0.01), and 17 of 48 (35%) with stable chronic ischemic heart disease (P<0.05).

Conclusion: The detection of high titers of lgG antibodies to C. pneumoniae in many patients with AMI, compared to control groups, suggest that chronic Chlamydia pneumoniae infection plays a role in the pathogenesis of atherosclerosis and acute ischemic events.