Autoimmune and Autoinflammatory Diseases with Inflammatory Arthritis (AiArthritis Diseases)

Sarcoidosis is an inflammatory disease characterized by the formation of sarcoid granulomas—tiny clumps of inflammatory cells—in one or more organs of the body. When the immune system goes into overdrive and too many of these clumps form, they can interfere with an organ’s structure and function. When left unchecked, chronic inflammation can lead to fibrosis, which is the permanent scarring of organ tissue. This disorder affects the lungs or lymph nodes in approximately 90% of cases, but it can affect almost any organ in the body. 

Relapsing Polychondritis (RP) is a rare autoimmune disease that causes inflammation of cartilage in the body. It most commonly affects the ears, nose, airways, and joints but can also involve the eyes, heart, and brain.

Behcet’s disease is a rare condition that causes inflammation of the blood vessels (vasculitis). It can affect various parts of the body, including the mouth, eyes, skin, and internal organs.

Familial Mediterranean Fever (FMF) is a genetic disorder that causes repeated episodes of pain and inflammation in the abdomen, chest, joints, and skin, along with fever. It mostly affects people from Mediterranean backgrounds, such as Arab, Armenian, Turkish, and Sephardic Jewish populations. FMF comes in two types:

• FMF Type 1: Causes recurring inflammation and symptoms like fever, abdominal pain, chest pain, and joint pain. If not treated, it can lead to kidney problems due to a buildup of a protein called amyloidosis.
• FMF Type 2: The main symptom is amyloidosis, even if other FMF symptoms aren’t present.

Hyper IgD Syndrome (HIDS), also called mevalonate kinase deficiency (MKD), is a rare autoinflammatory disease caused by a genetic mutation. This mutation affects the mevalonate kinase enzyme, leading to inflammation in the body. Symptoms usually begin in early childhood and happen in episodes, meaning they come and go with periods of little to no symptoms in between.

Chronic Nonbacterial Osteomyelitis (CNO) also called Chronic Recurrent Multifocal Osteomyelitis (CRMO) is a rare autoinflammatory disease where the bones become inflamed without infection. It causes long-term bone pain and swelling, often affecting the long bones, pelvis, shoulder, and spine. The disease mostly occurs in children.

Schnitzler syndrome is a rare autoinflammatory disease. People with this condition usually have non-itchy hives (urticaria) and abnormal levels of certain proteins in their blood, especially a protein called monoclonal IgM gammopathy.

Cryopyrin-Associated Periodic Syndromes (CAPS) are a group of rare genetic disorders characterized by recurrent episodes of inflammation and related symptoms. CAPS is caused by mutations in the NLRP3 gene, which provides instructions for making a protein called cryopyrin. Cryopyrin is involved in the regulation of the immune system, specifically in the process of inflammation.

There are three main conditions within the CAPS spectrum, which vary in terms of severity:

• Familial Cold Autoinflammatory Syndrome (FCAS): This is the mildest form of CAPS. Symptoms are triggered or worsened by exposure to cold temperatures. Patients experience rash, fever, joint pain, and other signs of inflammation during these episodes.
• Muckle-Wells Syndrome (MWS): MWS is a moderate form of CAPS. Symptoms are more persistent and may not always be triggered by cold exposure. Patients with MWS experience recurrent fever, rash, joint pain, and eye redness. Progressive hearing loss can also occur.
• Chronic Infantile Neurological, Cutaneous, and Articular Syndrome (CINCA) or Neonatal-Onset Multisystem Inflammatory Disease (NOMID): CINCA/NOMID is the most severe form of CAPS. Symptoms begin in infancy and include rash, joint swelling, chronic meningitis, and skeletal abnormalities. Neurological symptoms are also common, leading to developmental delays.

Fibromyalgia is a chronic condition characterized by widespread pain, fatigue, and tenderness in muscles, ligaments, and tendons. It frequently coexists with autoimmune and autoinflammatory arthritis, causing additional pain and fatigue, and is often misunderstood to be an autoimmune disease

Heart disease encompasses a range of conditions affecting the heart, including coronary artery disease, heart attacks, and heart failure. Autoimmune and autoinflammatory arthritis can increase the risk of heart disease due to chronic inflammation.

Interstitial lung disease includes a group of lung disorders that cause scarring of lung tissue, leading to breathing difficulties and reduced oxygen intake. Inflammatory arthritis can cause lung inflammation and scarring, contributing to these lung issues.

Dysautonomia is a disorder of the autonomic nervous system, affecting heart rate, blood pressure, digestion, and temperature regulation. It often occurs in patients with autoimmune diseases, causing symptoms like lightheadedness and fatigue.

Raynaud's phenomenon is a condition where blood flow to fingers and toes is restricted, leading to cold, numb, and discolored extremities. It often occurs with autoimmune diseases like scleroderma and lupus, as inflammation affects blood vessels.

Eye inflammation includes conditions such as uveitis, iritis, scleritis, and ocular inflammation, causing pain, redness, and vision problems. It can be a complication of autoimmune and autoinflammatory arthritis.

Potential dental issues include gum inflammation, periodontal disease, and tooth decay. Inflammation from autoimmune diseases can affect oral health, leading to increased dental problems.

Vasculitis is the inflammation of blood vessels, causing reduced blood flow and potential damage to organs and tissues. It can occur with autoimmune diseases, leading to symptoms like rashes, fatigue, and organ damage.

Celiac disease is an autoimmune disorder where ingestion of gluten leads to damage in the small intestine. It frequently coexists with other autoimmune diseases, causing digestive issues and nutrient absorption problems.

Lupus nephritis is an inflammation of the kidneys caused by systemic lupus erythematosus (SLE), leading to kidney damage. It is a severe complication of lupus, affecting kidney function and health.