scholarly journals Posner-Schlossman Syndrome in Common Variable Immunodeficiency

2020 ◽  
Vol 2020 ◽  
pp. 1-3
Author(s):  
Madiha Huq ◽  
Neha Sanan ◽  
Phuong Daniels ◽  
Robert Hostoffer
Keyword(s):  
Intraocular Pressure ◽  
Common Variable Immunodeficiency ◽  
Anterior Uveitis ◽  
Rare Condition ◽  
Elevated Pressure ◽  
Normal Limits ◽  
First Case ◽  
Autoimmune Conditions ◽  
The Right ◽  
Common Variable

Introduction. Posner-Schlossman syndrome (PSS) is a rare glaucomatocyclitic crisis with clinical features including recurrent episodes of unilateral elevated intraocular pressure. Autoimmune and infectious causes have been proposed as potential etiologies of PSS. We report the first case of PSS in the setting of common variable immunodeficiency (CVID). Case Report. A sixty-two-year-old Caucasian female with a medical history of CVID and ulcerative colitis presented to the emergency room with complaints of acute right-sided vision changes. She reported image distortion, blurriness, and loss of central vision. Physical exam was significant for mildly injected right conjunctiva, visual acuity of 20/70 in right eye, and 20/25 in left eye. The right intraocular pressure was measured at 34 mmHg and left at 12 mmHg. The gonioscopy and dilated fundus examination were unremarkable. Cup to disc ratio was within normal limits, and no afferent pupillary defects were recorded. The patient was acutely treated with three rounds of dorzolamide/timolol and 0.2% brimonidine which decreased the right eye intraocular pressure to 24 mmHg. On follow-up exam with an ophthalmologist, anterior uveitis including an elevated pressure of 41 mmHg on the right and 18 mmHg on the left eye was noted and a PSS diagnosis was confirmed. Conclusion. PSS remains a rare condition with uncertain etiology and no associated systemic conditions. PSS has been postulated to be linked to autoimmune conditions. CVID is associated with many autoimmune disorders including Sjogren’s, rheumatoid arthritis, and colitis. There have been a few reported CVID-associated ocular diseases including granulomatous uveitis and conjunctivitis, chronic anterior uveitis, and birdshot retinopathy. We describe the first case of PSS in a patient with CVID.

scholarly journals A rare case of peliosis hepatis in primary immune deficiency

2020 ◽  
Vol 8 ◽  
pp. 2050313X2093199
Author(s):  
Evelyn Angulo ◽  
Sydney Joyner ◽  
Nasma K Majeed ◽  
Sharmilee Nyenhuis
Keyword(s):  
Common Variable Immunodeficiency ◽  
Rare Condition ◽  
Liver Function Tests ◽  
Peliosis Hepatis ◽  
Primary Immune Deficiency ◽  
First Case ◽  
Regenerative Nodules ◽  
Elevated Liver Function Tests ◽  
Underlying Mechanisms ◽  
Common Variable

Peliosis hepatis is a rare condition characterized by blackish-blue blood-filled cavities in hepatic parenchyma caused by dilatation of hepatic sinusoids. Peliosis hepatis has been described in secondary immunodeficiencies and certain medications. We present the first case of peliosis hepatis in a patient with a primary immunodeficiency, common variable immunodeficiency. A 44-year-old African-American male presented with gastrointestinal bleeding and elevated liver function tests. His medical history included common variable immunodeficiency and chronic kidney disease. The patient had jaundice, regenerative nodules on liver pathology, and low immunoglobulin levels. A magnetic resonance imaging of the abdomen with contrast revealed a cirrhotic liver, a 5 × 3 cm lesion, and poorly defined nodules which had decreased enhancement. A computed tomography-guided liver biopsy revealed peliosis hepatis, focal nodular hyperplasia, and fibrosis. No other etiology of his liver disease was found. The etiology of peliosis hepatis in patients with primary immunodeficiencies remains unclear. Additional studies are needed to understand the underlying mechanisms.

scholarly journals Fatal liver mass rupture in a common-variable-immunodeficiency patient with probable nodular regenerating hyperplasia

2022 ◽  
Vol 18 (1) ◽  
Author(s):  
Mongkhon Sompornrattanaphan ◽  
Ranista Tongdee ◽  
Chamard Wongsa ◽  
Anupop Jitmuang ◽  
Torpong Thongngarm
Keyword(s):  
Portal Hypertension ◽  
Common Variable Immunodeficiency ◽  
Multiple Organ ◽  
Late Complications ◽  
Liver Mass ◽  
Cvid Patient ◽  
Hepatic Rupture ◽  
Fatal Liver ◽  
First Case ◽  
Common Variable

Abstract Background Nodular regenerating hyperplasia (NRH) is the most common liver involvement in common variable immunodeficiency (CVID). Most patients are asymptomatic with gradually increasing alkaline phosphatase (ALP) and mildly elevated transaminase enzymes over the years. We report the first case of fatal liver mass rupture in a CVID patient with probable NRH. Case presentation A 24-year-old man was diagnosed with CVID at the age of 1.25 years. Genetic testing revealed a transmembrane activator and calcium-modulator and cyclophilin-ligand interactor (TACI) mutation. He had been receiving intravenous immunoglobulin (IVIg) replacement therapy ever since then. The trough level of serum IgG ranged between 750–1200 mg/dL. However, he still had occasional episodes of lower respiratory tract infection until bronchiectasis developed. At 22 years old, computed tomography (CT) chest and abdomen as an investigation for lung infection revealed incidental findings of numerous nodular arterial-enhancing lesions in the liver and mild splenomegaly suggestive of NRH with portal hypertension. Seven months later, he developed sudden hypotension and tense bloody ascites. Emergency CT angiography of the abdomen showed NRH with intrahepatic hemorrhage and hemoperitoneum. Despite successful gel foam embolization, the patient died from prolonged shock and multiple organ failure. Conclusions Although CVID patients with NRH are generally asymptomatic, late complications including portal hypertension, hepatic failure, and hepatic rupture could occur. Therefore, an evaluation of liver function should be included in the regular follow-up of CVID patients.

Granulomatous lymphocytic interstitial lung disease as a complication of common variable immunodeficiency

2021 ◽  
Vol 31 (6) ◽  
pp. 816-821
Author(s):  
Alexandr V. Averyanov ◽  
Anastasia S. Perkina
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Common Variable Immunodeficiency ◽  
Clinical Case ◽  
Infectious Complications ◽  
Pathological Process ◽  
Malignant Neoplasms ◽  
Recurrent Infections ◽  
Autoimmune Conditions ◽  
Common Variable

Common variable immunodeficiency (CVID) is a rare immunodeficiency, the classic manifestation being recurrent infections. Other lesions are often found in CVID patients, such as malignant neoplasms, autoimmune conditions caused by abnormal cellular immunity, in addition to infectious complications. Usually, the pathological process involves the lungs. This article presents a clinical case of a patient with CVID complicated by granulomatous lymphocytic interstitial lung disease.

Case 2.37

2014 ◽  
pp. 130-131
Author(s):  
Christine U. Lee ◽  
James F. Glockner
Keyword(s):  
Common Variable Immunodeficiency ◽  
Celiac Axis ◽  
Porta Hepatis ◽  
Axial Diffusion ◽  
Hepatic Lobe ◽  
Diffusion Weighted ◽  
The Right ◽  
Delayed Phase ◽  
Common Variable

50-year-old woman being evaluated for common variable immunodeficiency Coronal SSFSE (Figure 2.37.1), axial fat-suppressed T2-weighted FSE (Figure 2.37.2), and axial diffusion-weighted (b=100 s/mm2) (Figure 2.37.3) images demonstrate a heterogeneous mass with lobulated margins in the right hepatic lobe, as well as extensive adenopathy in the celiac axis, porta hepatis, and portocaval space. Note also moderate splenomegaly. Arterial, portal venous, equilibrium, and delayed phase postgadolinium 3D SPGR images (...

Granuloma-like lesion at subcutaneous immunoglobulin site in a common variable immunodeficiency patient

Immunotherapy ◽  
2019 ◽  
Vol 11 (14) ◽  
pp. 1177-1180 ◽  
Author(s):  
Ankmalika Gupta ◽  
Beverly Wang ◽  
Sudhir Gupta
Keyword(s):  
Intravenous Immunoglobulin ◽  
Common Variable Immunodeficiency ◽  
Immunoglobulin Therapy ◽  
Subcutaneous Immunoglobulin ◽  
Primary Antibody Deficiencies ◽  
First Case ◽  
Granulomatous Lesions ◽  
Cutaneous Granuloma ◽  
Common Variable ◽  
Switching Treatment

Immunoglobulin therapy is the main stay in the treatment of primary antibody deficiencies. Granulomatous lesions are common complication in patients with common variable immunodeficiency (CVID). We present the first case of cutaneous granuloma-like lesion at site of subcutaneous immunoglobulin injections in a patient with CVID. These lesions resolve overtime following switching treatment to intravenous immunoglobulin. Unlike granulomas associated with CVID, granulomatous lesion in this patient did not require any specific therapy, and resolved over a period of 4 weeks following switching subcutaneous immunoglobulin to intravenous immunoglobulin.

Chronic Anterior Uveitis in Common Variable Immunodeficiency

2011 ◽  
Vol 19 (6) ◽  
pp. 448-449 ◽  
Author(s):  
Erica Z. Oltra ◽  
Cody Morris ◽  
Andrea D. Birnbaum ◽  
Howard H. Tessler ◽  
Debra A. Goldstein
Keyword(s):  
Common Variable Immunodeficiency ◽  
Anterior Uveitis ◽  
Common Variable

scholarly journals Spontaneous Cervical Epidural Hematoma with Hemiparesis Mimicking Cerebral Stroke

2014 ◽  
Vol 2014 ◽  
pp. 1-3 ◽  
Author(s):  
Mehmet Tiryaki ◽  
Recep Basaran ◽  
Serdar Onur Aydin ◽  
Mustafa Efendioglu ◽  
Ece Balkuv ◽  
...  
Keyword(s):  
Early Diagnosis ◽  
Epidural Hematoma ◽  
Rare Condition ◽  
Diagnosis And Treatment ◽  
Cerebral Stroke ◽  
Normal Limits ◽  
Cranial Mri ◽  
Spinal Mri ◽  
The Right ◽  
Loss Of Strength

Aim.Spontaneous cervical epidural hematoma (SCEH) is defined as an epidural hematoma that does not have an etiological explanation. The most common site for SCEH is cervicothoracic area. Early diagnosis and treatment are important for prognosis and good results. In this paper, we aimed to present a case who complains of sudden weakness on right extremities imitating cerebral stroke and that neuroimaging reveals spontaneous cervical epidural hematoma.Case.A 72-year-old woman was admitted to our hospital with acute neck pain and loss of strength on right extremities. On neurological examination, the patient had right hemiparesis. PT, aPTT, and INR results were 50.5, 42.8, and 4.8, respectively. Cranial MRI was in normal limits. Spinal MRI revealed a lesion that extends from C4 to C7 located on the right side and compatible with epidural hematoma. The patient was operated after normalization of INR values.Conclusion.Even though SCEH is a rare condition, it can cause severe morbidity and mortality. Early diagnosis and treatment are quiet important for prognosis. SCEH can easily be mistaken for stroke as with other pathologies and this diagnosis should come to mind especially in patients who have diathesis of bleeding.

scholarly journals Common Variable Immunodeficiency Presenting with Recurrent Ascending Cholangitis Treated with Oral Immunoglobulins

2020 ◽  
Vol 1 (1) ◽  
pp. 11-13
Author(s):  
Marija Rowane ◽  
Jaimin Patel ◽  
Tina Abraham ◽  
Jason Schend ◽  
Robert Hostoffer
Keyword(s):  
Common Variable Immunodeficiency ◽  
Primary Immune Deficiency ◽  
Normal Ranges ◽  
First Case ◽  
Serum Iga ◽  
Ascending Cholangitis ◽  
History Of ◽  
Irritable Bowel ◽  
Duct Stricture ◽  
Common Variable

Abstract Background: Common variable immunodeficiency (CVID) is a heterogeneous group of primary immune deficiency disorders that may be characterized by heightened susceptibility to gastrointestinal (GI) infection. GI conditions manifest in 20 to 50% of CVID patients but rarely include cholangitis. Methods/Results: This is a 61-year-old female, with a history of cholecystectomy and gastric bypass, who presented with recurrent ascending cholangitis for eight years. After a hepaticojejunostomy to correct a bile duct stricture complication from the cholecystectomy, ascending cholangitis was diagnosed by clinical presentation of fever and right upper quadrant pain and imaging revealing pneumobilia. CVID was diagnosed after no response to pneumococcal polysaccharide vaccine, as well as serum IgA and IgG measured below the normal ranges. The patient was prescribed a successful, weekly regimen of 15 g of oral intravenous immunoglobulin (IVIG) ten percent liquid (Gammaplex® 10%, 5 gm/50 mL). Conclusion: Oral human IVIG is a novel treatment and has been infrequently utilized for management of chronic rotavirus, necrotizing enterocolitis, diarrhea, HIV enteropathy, irritable bowel syndrome, and malnutrition. This is the first case of recurrent ascending cholangitis as the primary manifestation of CVID, as well as successful treatment of this condition with oral IVIG, in the literature.

scholarly journals Transforaminal Endoscopic Decompression for a Giant Epidural Gas-Containing Pseudocyst: A Case Report and Literature Review

2017 ◽  
Vol 3 (20;3) ◽  
pp. E445-E449
Author(s):  
Xiao Guang Liu
Keyword(s):  
Endoscopic Surgery ◽  
Tissue Injury ◽  
Soft Tissue Injury ◽  
Rare Condition ◽  
Radicular Pain ◽  
Recurrence Rates ◽  
Endoscopic Techniques ◽  
First Case ◽  
The Right ◽  
Epidural Gas

The isolated epidural gas-containing pseudocyst is an uncommon pathogenic factor for severe pain of the lower limb as a result of nerve root compression. After reviewing these rare cases reported in the literature, we found that the name, pathogenesis, and treatment strategy of this pathology remained controversial. The most common treatment is conservative treatment or percutaneous aspiration which might result in poor pain relief and high recurrence rates. Moreover, the patient who received open surgery had good clinical outcome; however, he or she might experience a significant soft tissue injury. In this study, we report the first case of a patient who had a giant epidural gas-containing pseudocyst and received percutaneous endoscopic surgery. This 57-year-old man had been complaining of severe radicular pain in his right ankle for one year. According to computed tomography (CT) and magnetic resonance imaging (MRI) prior to the surgery, the results showed an isolated epidural gas-containing pseudocyst was located in the right lateral recess of S1. At the last follow-up period, postoperative CT scan showed the gas-contained pseudocyst was completely resected and this patient was free from the pain. Due to the great advances in endoscopic techniques and equipment, it is easier to perform lumbar surgery through the endoscope. With this first case of percutaneous endoscopic treatment for the symptomatic epidural gas-containing pseudocyst reported in this study, we believe that this surgical method provides an option to treat this rare condition because it provides sufficient decompression, has a low recurrence rate, and is minimally invasive. Key words: Endoscopic surgery, pseudocyst, epidural gas, intraspinal gas, radiulopathy

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