Cryopyrin-Associated Periodic Syndromes and Treatment Options

Cryopyrin-associated periodic syndromes (CAPSs) are a growing family of autoinflammatory diseases, also known as periodic fever syndromes. There are three forms of CAPS: (1) Familial Cold autoinflammatory syndrome or familial cold urticaria, (2) Muckle-wells syndrome, and (3) neonatal-onset multisystem inflammatory disease or chronic infantile neurological cutaneous articular syndrome. Genetic mutations in the NLRP3 gene were found to be present in most patients. The foremost common findings between all the CAPS disorders are rash, fever which is sometimes present at birth or in early childhood, joint problems, and conjunctivitis. More extreme forms of CAPS include more persistent inflammation that can cause hearing loss and meningitis and can lead to mental and developmental delays. Drugs for CAPS target the source of inflammation – which is the over-production of interleukin 1ß by modified cryopyrin inflammasomes. Three drugs are used to treat CAPS: Rilonacept, canakinumab, and anakirna. With these drugs, the prognosis is greatly improved, with most patients having less frequent episodes, decreased buildup of amyloid in the body, and extended life of severe cases up to adulthood

Adverse Reactions to Intravenous Immunoglobulins - Our Experience

BACKGROUND: Adverse reactions to intravenous immunoglobulins (IVIG) are divided by organ system involved, or by timing of onset–immediate which occur during infusion usually rate-related, true IgE-mediated anaphylaxis and delayed reaction which occur hours to days after the infusion. AIM: To describe the adverse events of patients given IVIG infusions. METHODS: Total number of patients receiving IVIG was 41 with 25 males (60.97%) and 16 females (39.02%), age 2 months-35 years. A total number of infusions was 1350. RESULTS: Total number of adverse reactions 15, 14 patients with immediate-type and 1 with delayed type. Total percentage of adverse reactions in a given sample was 1.1% of all IVIG infusions. Fever was the most common immediate type of reaction occurring in 11 patients (78.57%) followed by acrocyanosis 10 patients (71.42%), skin rash 9 patients (64.28%) and headache 8 patients (57.14). Delayed-type of reactions (like fever, headache and vomiting) was present in one patient. Majority of the adverse effects occurred at the infusion rate higher than 1, 5 ml/kg/hour, which is still within recommended speed. CONCLUSION: About 1.1% of IVG infusions where with adverse events. Most common manifestations where: fever, acrocyanosis, skin rash and headache, which occurred 1-6 hours from the beginning of the infusion. The occurrence of adverse reactions to IVIG was related to the infusion rates in a fashion that faster infusion rate gives more reactions. Adverse reactions were managed by reduction of the infusion rate and administration of medications such as paracetamol, antihistamines and steroids

INTRAVENOUS IMMUNOGLOBULIN THERAPY IN MEDICAL PRAXIS

ABSTRACT Intravenous immunoglobulin (IVIG) is a blood product prepared from the serum of of a thousand or more blood donors. The IVIG that is available contains complete immunoglobulin G( IgG )molecules which has intact Fc-dependent effector functions. The IgG subclasses match those in normal human serum. It is the main treatment for patients with antibody deficiencies. High dose IVIG (hdIVIG), is used as an immunomodulatory agent in an increasing number of immune and inflammatory disorders. The clinical specialities using the largest amounts of IVIG are neurology, haematology, immunology, nephrology, rheumatology and dermatology. In this paper, we review recent developments in the understanding of mechanisms of action of IVIG and the major clinical areas of use

INTRAVENOUS IMMUNOGLOBULIN THERAPY IN MEDICAL PRAXIS

Intravenous immunoglobulin (IVIG) is a blood product prepared from the serum of of a thousand or more blood donors. The IVIG that is available contains complete immunoglobulin G( IgG )molecules which has intact Fc-dependent effector functions. The IgG subclasses match those in normal human serum. It is the main treatment for patients with antibody deficiencies. High dose IVIG (hdIVIG), is used as an immunomodulatory agent in an increasing number of immune and inflammatory disorders. The clinical specialities using the largest amounts of IVIG are neurology, haematology, immunology, nephrology, rheumatology and dermatology. In this paper, we review recent developments in the understanding of mechanisms of action of IVIG and the major clinical areas of use

VALUES OF LYMPHOCYTE SUBPOPULATIONS IN HEALTHY MACEDONIAN CHILDREN UNDER THE AGE OF FIVE

ABSTRACT Background: The effects of demographic factors on a wide range of immunological variables demonstrate the importance of having normative data representative of particular patient population. There was no lymphocyte subpopulation data for Macedonian children and the purpose of this study was to establish such a data. Subject and methods: The study population consists of 87 healthy children. Subjects were grouped into four age categories as follows: group 1 age range 5d-10d (n=15); group 2 age range 1 mo-1 yr (n=18); group 3 age 1yr-2 yr(n=20) and group 4 age 2yr-5 yr(n=34 Monoclonal antibodies labeled with fluorochromes and immunofluorescent microscopy were used to detect cells bearing specific cell markers

JOINT DISEASE IN CHILDREN WITH X-LINKED AGAMMAGLOBULINEMIA

Patients with X-linked agammaglobulinemia (XLA) are prone to recurrent bacterial infections due to low levels of immunoglobulins. Clinical symptoms include recurrent bacterial otitis media, bronchitis, pneumonia, meningitis, skin infection and arthritis.In the majority of cases arthritis can be shown to be caused by infection, but also aseptic arthritis and autoimmune diseases may be present. Monoarthritis and oligoarthritis is usual pattern, although polyarthritis may occur. This paper presents diagnostic and therapeutic problems in our patients with agammaglobulinemia and arthritis