Granulomatous disease in common variable immunodeficiency

Abstract

Common variable immunodeficiency (CVID) is a primary immunodeficiency of unknown etiology characterized by low levels of immunoglobulin (Ig)G, failure to make specific antibodies in response to infection or immunization, and variable T-cell abnormalities. Multisystemic granulomatous disease is a well-documented complication of CVID, and its presence is associated with significant morbidity and early mortality. Although the lung is the most common organ system affected, granulomas are also found frequently in other organs, including skin, liver, spleen, and the gastrointestinal tract. Autoimmune disorders are common in these patients, and there appears to be an increased propensity to develop lymphoproliferative disorders. Common physical, radiographic, and laboratory abnormalities in patients with CVID and granulomatous disease include splenomegaly, hilar and mediastinal lymphadenopathy with ground glass or nodular opacities in the lung parenchyma, and reduced T-cell numbers and function. The etiology of granulomatous disease in patients with CVID is unknown, and optimal treatment of granulomatous disease in CVID remains to be established. Further studies are needed to elucidate the underlying etiology of granulomatous lymphoproliferative interstitial lung disease and to delineate appropriate treatments for this disease.

Common variable immunodeficiency: Crossroads between infections, inflammation and autoimmunity

“…These patients commonly show polyclonal lymphoid infiltration of the lung, and biopsies can reveal features of reactive lymphoid hyperplasia or granulomatous inflammation (13). In a subset of patients, granulomas can also be accompanied by intense lymphocytic infiltration, a condition described as granulomatous lymphocytic interstitial lung disease (GLIDL) (14). Granulomas are found in the lungs of more than 50%…”

“…In these cases, histology consistently revealed lymphocytic portal infiltration and, less frequently, the presence of granulomas. Furthermore, patients with liver NRH are prone to develop other autoimmune diseases such as ITP, AHA, Sjogren's syndrome, thyroiditis, type 1 diabetes, vitiligo, rheumatoid arthritis, celiac-like villous atrophy, inflammatory bowel diseases, pernicious anemia, vasculitis, and psoriasis, suggesting the possibility of a common pathogenesis (14).…”

Granulomatous disease: Distinguishing primary antibody disease from sarcoidosis

“…Systemic symptoms such as fatigue, weight loss and fever may also occur. As in sarcoid, arthralgia, hepatosplenomegaly and lymphadenopathy are also prominent and clinically, there may be difficulty in distinguishing the two conditions [5,6]. Granulomatous disease can affect the same organs in both diseases.…”

Fever of Unknown Origin in a Patient with Common Variable Immunodeficiency Associated with Multisystemic Granulomatous Disease

“…The (3,4). Different autoimmune diseases are frequently associated with granulomatous CVID(over 50% of patients), particularly rheumatoid arthritis, haemolytic anaemia or primary biliary cirrhosis (Table 1).…”

“…Nevertheless, in most series of FUO in the literature, granulomatous diseases make up less than 5% of diagnostic categories, usually labeled as "collagen diseases" or "inflammatory disorders" (14). The (4). In patients with severe hypersplenism or refractory haemolytic anaemia, splenectomy has frequently been performed in the past (8).…”