Presentation
Symptoms depend on type of reaction due to autoimmunity and its site. The common symptoms are fatigue, fever, malaise, joint pain, and rash. Anaphylaxis is characterized by dizziness, rash itching, breathless, tachycardia and lip swelling. Autoimmunity against blood components is characterized by fatigue, yellow urine, and fever. Involvement of lung is characterized by cough, fever and dyspnea [6].
Entire Body System
- Anemia
The authors describe a girl who developed a severe systemic autoimmune disease with severe autoimmune hemolytic anemia, mild autoimmune thrombopenia, antineutrophil antibodies, and fatal autoimmune hepatitis after EBV infection. [ncbi.nlm.nih.gov]
Some Autoimmune Disorders Disorder Main Tissues Affected Consequences Autoimmune hemolytic anemia Red blood cells Anemia (decreased number of red blood cells) develops, causing fatigue, weakness, and light-headedness. The spleen may enlarge. [merckmanuals.com]
Examples of autoimmune diseases include systemic lupus erythematosus, Sjogren syndrome, Hashimoto thyroiditis, rheumatoid arthritis, juvenile (type 1) diabetes, polymyositis, scleroderma, Addison disease, vitiligo, pernicious anemia, glomerulonephritis [rxlist.com]
Since vitamin B12 plays an important role in making red blood cells, a deficiency leads to anemia. [verywellhealth.com]
- Relapsing Polychondritis
Relapsing polychondritis (RP) is an uncommon inflammatory disease manifested by recurrent attacks of auricular chondritis. [ncbi.nlm.nih.gov]
polychondritis Scleroderma (systemic sclerosis) Psoriatic arthritis Sjogren's syndrome Vasculitis Antiphospholipid syndrome Treating Rheumatologic Autoimmune Diseases Treatments for rheumatologic autoimmune diseases depend on the specific conditions [utswmed.org]
A diagnosis of relapsing polychondritis was made. [nejm.org]
Some autoimmune disorders that can affect the ear include Cogan’s syndrome, relapsing polychondritis, polyarteritis nodosa, Wegener’s granulomatosis, systemic lupus erythematosus, ulcerative colitis, Sjogren’s syndrome, and rheumatoid arthritis. [vestibular.org]
- Hodgkin Lymphoma
Complete clinical examination, thoracoabdominal-pelvic computed tomography, and whole-body fludeoxyglucose F 18-positron emission tomography failed to show any underlying tumor, including Hodgkin lymphoma. [ncbi.nlm.nih.gov]
Unlike cancer, which is an umbrella category for a range of diseases (leukemia, breast cancer, prostate cancer, non-Hodgkin’s lymphoma, et al.), autoimmunity has yet to be embraced by the medical community (and the public) as a category of disease. [aarda.org]
- Reiter Syndrome
Keratoconjunctivitis sicca Ankylosing spondylitis Uveitis Reiter's syndrome Conjunctivitis, uveitis, keratitis Enteropathic arthritis Uveitis, episcleritis, peripheral ulcerative keratitis Psoriatic arthritis Uveitis, conjunctivitis, keratitis Systemic [aafp.org]
[…] occur in conjunction with other immune-related disorders Pure red cell aplasia Rasmussen's encephalitis anti-NR2A antibodies Raynaud phenomenon Suspected Can occur in conjunction with other immune-related disorders Relapsing polychondritis Accepted[61] Reiter's [hi.wikipedia.org]
Arthritis usually coupled with other normal symptoms; this is called Reiter’s syndrome, Reiter’s disease or Reiter’s arthritis. Hashimoto's disease Hashimoto's disease is a chronic disorder of autoimmune disease. [autoimmunediseases.imedpub.com]
Respiratoric
- Hemoptysis
We report a 73-year-old male who presented with cutaneous blistering, upper gastrointestinal bleeding, and hemoptysis. He later developed right flank pain secondary to a retroperitoneal hematoma. [ncbi.nlm.nih.gov]
Musculoskeletal
- Arthralgia
Most developed fever and arthralgias/arthritis. Other findings included serositis manifested by tachycardia, dyspnea and pleural effusions, headaches, and hair loss. [ncbi.nlm.nih.gov]
Patients presenting with DILE may experience fever, arthralgia, arthritis, myalgia, or serositis. [pharmacytimes.com]
Skin
- Ulcer
Potential application areas: Crohn’s disease, Ulcerative Colitis, Rheumatoid Arthritis (RA), Psoriasis and Systemic Lupus Erythematosus (SLE), etc. [myriadrbm.com]
Paracentral ulcerative keratitis: destructive ulceration of the clear central or paracentral cornea; associated with pain and photophobia Peripheral ulcerative keratitis: a peripheral corneal ulceration associated with a crescent-shaped destructive inflammation [aafp.org]
Two patients were treated with immunotherapy for metastatic malignant melanoma (MM) despite suffering from systemic autoimmune disease, i.e., ulcerative colitis (UC) and Behcets disease (BD), respectively. [ncbi.nlm.nih.gov]
For example, regarding the high prevalence of inflammatory bowel disease (IBD; Crohn’s disease and ulcerative colitis) in PSC, the authors speculate that PSC-IBD is a unique disease that shares some genetic factors with ulcerative colitis but distinct [ard.bmj.com]
In the group of 80 ulcerative colitis patients, coexistence of celiac disease basing on serological histopatological investigation was found in 4 patients (5%).” [glutenfreesociety.org]
- Alopecia
Development of diffuse BFHs has been associated with autoimmune disease and with the development of diffuse alopecia. Two women with autoimmune diseases had diffuse alopecia develop. [ncbi.nlm.nih.gov]
Types of autoimmune diseases include: Alopecia areata, Antiphospholipid antibody syndrome (aPL), Autoimmune hepatitis, Celiac disease, Diabetes type 1, Graves disease, Guillain-Barre syndrome, Hashimotos disease, Hemolytic anemia, Idiopathic thrombocytopenic [scitechnol.com]
[…] history of autoimmune disease People who have certain environmental exposures that could act as the trigger People of a particular race or ethnicity Many types of arthritis are considered autoimmune diseases, including: Other autoimmune diseases include alopecia [verywellhealth.com]
- Eczema
[…] that resulted in complete remission of his eczema. [ncbi.nlm.nih.gov]
Common Autoimmune Disease Symptoms Some of the most common autoimmune disease symptoms you should look out for include: Headaches Anxiety Brain fog Attention deficit problems Body rashes, red bumps on facial skin and red flaking skin Acne Rosacea Eczema [draxe.com]
Eczema Eczema, also known as atopic dermatitis, is generally a mild skin disease and is the most common skin disease in primary immunodeficiency diseases. [primaryimmune.org]
- Petechiae
They may develop a pinpoint red rash caused by small hemorrhages called petechiae. They may notice nosebleeds that are more frequent and difficult to resolve. The gums may bleed easily. The urine may have an orange, pink or red color. [primaryimmune.org]
Neurologic
- Headache
Vasculitis — Rash, night sweats, headache, fever, weight loss, weakness or numbness, aches and pains, and fatigue result from this disease that inflames blood vessels. [templehealth.org]
Other findings included serositis manifested by tachycardia, dyspnea and pleural effusions, headaches, and hair loss. All had a positive antinuclear antibody (ANA), and the majority had double stranded (ds) DNA antibodies. [ncbi.nlm.nih.gov]
[…] gain If left un-repaired, it can lead to more severe health issues like inflammatory bowel disease, IBS, arthritis, eczema, psoriasis, depression, anxiety, migraine headaches, muscle pain and chronic fatigue. [draxe.com]
Ten individuals experienced general clinical symptoms, which included abdominal pain, diarrhea, and headache. [omicsonline.org]
Astrovirus infections are marked by symptoms such as diarrhea, vomiting, abdominal cramping, headache, fever, and mild dehydration. [contagionlive.com]
- Guillain-Barré Syndrome
Autoimmune with no specific causal infectious agent identified: Sarcoidosis Guillain-Barré Syndrome / Acute Inflammatory Demyelinating Polyneuropathy (AIDP) Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) Vasculitis Polyarteritis Nodosa (PAN) [foundationforpn.org]
Wegener's granulomatosis Goodpasture's syndrome Accepted[1] II Anti-Basement Membrane Collagen Type IV Protein Graves' disease Accepted[1] II thyroid autoantibodies (TSHR-Ab) that activate the TSH-receptor (TSHR) Guillain-Barré syndrome (GBS) Accepted [hi.wikipedia.org]
This causes the body to go into overdrive, leading to symptoms including: A racing heart Anxiety Weight loss Feeling hot Sweating more than usual Guillain-Barré Syndrome Guillain-Barré syndrome (GBS) results from an immune system attack on the nervous [verywellhealth.com]
Balo’s Disease Graves’ Disease Guillain-Barré Syndrome Fact Sheet Autoimmune hepatitis Department of Dermatology: Autoimmune Diseases What is Lupus Endometriosis Systemic lupus erythematosus (SLE or lupus) Antiphospholipid Syndrome Antinuclear Antibodies [blog.delimmune.com]
- Vertigo
Vestibular symptoms can include disequilibrium, ataxia, motion intolerance, positional vertigo, and episodic vertigo. [emedicine.medscape.com]
| N Engl J Med 2018; 379:2152-2159 A 23-year-old woman presented with vertigo and vision loss affecting the right eye. Hearing loss and tinnitus of the right ear developed. [nejm.org]
Symptoms may include weakness, abnormal sensations, vertigo, problems with vision, muscle spasms, and incontinence. Symptoms vary over time and may come and go. The prognosis varies. [merckmanuals.com]
[…] swollen nymph nodes/glands night sweats numbness and tingling in hands and feet low blood pressure tremors and seizures dry eyes weight loss hair loss dry mouth shortness of breath heart palpitations abdominal pain, cramping and tenderness dizziness and vertigo [evenbetterhealth.com]
- Polyradiculoneuropathy
Chronic active hepatitis (CAH) Primary Biliary Cirrhosis (PBC) Primary sclerosing cholangitis (PSC) Celiac disease Pernicious anemia Inflammatory bowel disease Neuromuscular system disorders affect nerves and muscles Chronic inflammatory demyelinating Polyradiculoneuropathy [autoimmunediseaselist.com]
Workup
The diagnosis can be done by physical exam and laboratory investigation. Important laboratory tests are antinuclear antibody tests, autoantibody tests, complete blood cound (CBC), comprehensive metabolic panel, C-reactive protein (CRP), Erythrocyte sedimentation rate (ESR) and urinalysis.
Chest X-ray and pulmonary function test may be required in case of lung involvement. Similarly, bone marrow biopsy or kidney biopsy may be done if required.
Kidney function test and renal function test may be required in advanced cases or the cases taking treatment for long period or for the cases like graft rejection [7].
HLA Type
- HLA-A2
Bjorkman PJ, Saper MA, Samraoui B, et al: Structure of the human class I histocompatibility antigen, HLA-A2. Nature 1987;329:506–512. Cuvelier C, Barbatis C, Mielants H, et al: Histopathology of intestinal inflammation related to reactive arthritis. [karger.com]
Treatment
Symptomatic control, control of autoimmune reaction and maintainance of general immunity are the objectives of treatment. Mainstay of treatment is control of immune reaction by immunosuppressant drugs either corticosteroids like prednisone or non-steroids like tacrolimus, cyclophosphamide, sirolimus, mycophenolate or azathioprine. Further treatment depends on type of disease like hormones, hematinincs, blood transfusion or physiotherapy. Anaphylaxis is an emergency and requires prompt emergency services including oxygen, advanced life support measures, drugs like adrenaline [8].
Prognosis
Autoimmune diseases are chronic, characterized by remission and relapse or flare up. But clinical symptoms are well controlled by treatment [5].
Etiology
The reason that body fails to recognize between ‘self’ and ‘non-self ’may be immunologic, genetic or microbial. Mixed etiology may be possible. There is polyclonal activation of B-cells along with generation of self reacting B-Cells. This may be augmented by increased T helper cell activity. Genetic factors include increased expression of Class II HLA antigens on affected tissues. Infection with virus like Epstein–Barr virus (EBV) or bacteria like streptococcus and mycoplasma may trigger immune reaction against host tissue [2].
Epidemiology
In the United States autoimmune conditions are among the leading causes of death among women of young and middle-age. Incidence rates differ according to disease. Less than one case of systemic sclerosis is diagnosed per 100,000 person years whereas more than 20 cases of adult-onset rheumatoid arthritis are diagnosed per 100,000 person-years. Diseases like type I diabetes primarily occur in childhood and adolescence whereas myasthenia gravis or multiple sclerosis occurs in the mid-adult years. Rheumatoid arthritis, primary systemic vasculitis are diagnosed among older adults [3].
Pathophysiology
Once autoantibodies are formed against own tissue there may be one of four actions, i.e. anaphylactic, cytotoxic, immune complex or cell mediated. Anaphylaxis is characterized by releasing histamine locally or generally. Examples are asthma, hay fever and serum sickness. Examples of cytotoxic reaction are autoimmune hemolytic anemia, transfusion reaction, erthrobalstosis foetalis, idiopathic thrombocytopenic purpura (ITP) and myasthenia gravis. Examples of immune complex reaction are farmer’s lung, glomerulonephritis, collagen disease, Goodpasture syndrome and arthritis. Cell mediate reactions are leprosy and graft rejection [4].
Prevention
Since etiology of autoimmunity is immune-genetic prevention of these diseases is not possible. Only conditions precipitated by bacterial or viral infections are preventable by control of these infections [9].
Summary
Autoimmunity is a condition in which immune system of the body fails to recognize between ‘self’ and ‘non-self ’ and reacts by formation of antibodies against own tissues.
The condition maybe limited to one of the organ like in Goodpasture syndrome or may be may involve specific tissues of different organs like in systemic lupus erythematosus. Clinical symptoms depend on the type of tissue involved. Diagnosis is always done by the detection of autoantibodies against specific tissue and histopathology. Immunosuppressant drugs are important treating agent in these conditions [1].
Patient Information
- Definition: Autoimmunity is a condition in which immune system of the body fails to recognize between ‘self’ and ‘non-self ’ and reacts by formation of antibodies against own tissues. This condition may be limited to one of organ like in Goodpasture syndrome or maybe may involve specific tissues of different organs like in systemic lupus erythematosus. Clinical symptoms depend on type of tissue involved. Diagnosis is always done by detection of autoantibodies against specific tissue and histopathology. Immunosuppressant drugs are important treating agents in these conditions. Examples of the diseases are asthma, hay fever, serum sickness autoimmune hemolytic anemia and transfusion reaction, erthroblastosis fetalis, ITP, myasthenia gravis, farmer’s lung, glomerulonephritis, collagen disease, Goodpasture syndrome, arthritis, leprosy and graft rejection. Diseases like type I diabetes primarily occur in childhood and adolescence whereas myasthenia gravis, multiple sclerosis occurs in the mid-adult years. Rheumatoid arthritis and primary systemic vasculitis are diagnosed among older adults.
- Symptoms: Symptoms depend on type of reaction due to autoimmunity and its site. The common symptoms are fatigue, fever, malaise, joint pain, and rash. Anaphylaxis is characterized by dizziness, rash, itching, breathless, increased heart rate, lip swelling. Autoimmunity against blood component is characterized by fatigue, yellow urine, and fever. Involvement of lung is characterized by cough, fever, breathlessness.
- Diagnosis: The diagnosis can be done by physical exam and laboratory investigation including antinuclear antibody tests, autoantibody tests, complete blood count (CBC), C-reactive protein (CRP), erythrocyte sedimentation rate (ESR) and urinalysis. Chest X-ray and pulmonary function test may be required in case of lung involvement. Similarly, bone marrow biopsy or kidney biopsy may be done if required. Kidney function test and renal function test may be required in advanced cases or cases taking treatment for long period or for a cases like graft rejection.
- Treatment: Symptomatic control, control of autoimmune reaction and maintainance general immunity are the objectives of treatment. Mainstay of treatment is control of immune reaction by immunosuppressant drugs either corticosteroids like prednisone or non-steroids like tacrolimus, cyclophosphamide, sirolimus, mycophenolate or azathioprine. Further treatment depends on type of disease like hormones, hematinincs, blood transfusion, physiotherapy. Anaphylaxis is an emergency and requires prompt emergency services including oxygen, advanced life support measures, drugs like adrenaline. Since causes of autoimmunity are immune-genetic prevention of these diseases is not possible. Only conditions precipitated by bacterial or viral infections are preventable by control of these infections. Autoimmune diseases are chronic characterized by period of no symptom and flare up. But clinical symptoms are well controlled by treatment [10].
References
- Rose NR. Predictors of autoimmune disease: autoantibodies and beyond. Autoimmunity 2008; 41:419.
- Drachman DB. How to recognize an antibody-mediated autoimmune disease: Criteria. In: Immunologic Mechanisms in Neurologic and Psychiatric Disease, Waksman BH (Ed), Raven, New York 1990. p.183.
- Hartmann RC, Conley CL. Studies on the initiation of blood coagulation, III. The clotting properties of canine platelet-free plasma. J Clin Invest 1952; 31:685.
- Swanborg RH. Experimental autoimmune encephalomyelitis in rodents as a model for human demyelinating disease. Clin Immunol Immunopathol 1995; 77:4.
- Horak I, Löhler J, Ma A, Smith KA. Interleukin-2 deficient mice: a new model to study autoimmunity and self-tolerance. Immunol Rev 1995; 148:35.
- Mackay IR, Leskovsek NV, Rose NR. Cell damage and autoimmunity: a critical appraisal. J Autoimmun 2008; 30:5.
- Pendergraft WF 3rd, Preston GA, Shah RR, et al. Autoimmunity is triggered by cPR-3(105-201), a protein complementary to human autoantigen proteinase-3. Nat Med 2004; 10:72.
- Lee YH, Rho YH, Choi SJ, et al. The PTPN22 C1858T functional polymorphism and autoimmune diseases--a meta-analysis. Rheumatology (Oxford) 2007; 46:49.
- Emmi L, Romagnani S. Role of Th1 and Th2 cells in autoimmunity. In: The Autoimmune Diseases, Rose NR, Mackay IR (Eds), Academic Press, San Diego 2006. p.83.
- Oppermann M, Fritzsche J, Weber-Schoendorfer C, et al. A(H1N1)v2009: a controlled observational prospective cohort study on vaccine safety in pregnancy. Vaccine 2012; 30:4445.