Pathogenesis (disease development)
In lupus erythematosus, antigen-specific T and B lymphocytes cause pathologic (abnormal) immune responses, which in turn lead to the formation of autoantibodies (antibodies that bind an endogenous antigen). A possible trigger of systemic lupus erythematosus could be Enterococcus gallinarum. This is a motile bacterium from the genus Enterococcus. The intestinal bacterium has been detected in the liver, where it promotes the formation of proteins that could trigger the autoimmune disease, Note: Not a human study; Model: Mouse
Etiology (Causes)
Biographic causes
- Genetic burden
- In juvenile systemic lupus erythematosus, an index case (first documented case of disease) is found in approximately 15% of cases and a familial burden of autoimmune disease in 40%
- Genes/SNPs (single nucleotide polymorphism; English : single nucleotide polymorphism):
- Genes: HLA-DQ1, IRF5, STAT4
- SNP: rs7574865 in the gene STAT4
- Allele constellation: GT (1.55-fold).
- Allele constellation: TT (2.4-fold)
- SNP: rs2187668 in the gene HLA-DQ1
- Allele constellation: AG (2.3-fold).
- Allele constellation: AA (2.3-fold)
- SNP: rs2004640 in the gene IRF5
- Allele constellation: GT (1.4-fold).
- Allele constellation: TT (1.4-fold)
- Allele constellation: GG (0.9-fold)
- SNP: rs13192841 in an intergenic region.
- Allele constellation: AG (0.7-fold).
- Allele constellation: AA (0.5-fold)
- Genetic factors, unspecified (systemic lupus erythematosus).
The following trigger factors (possible triggers) are known:
Behavioral trigger factors
- Diet
- Micronutrient deficiency (vital substances) – see Prevention with micronutrients.
- Consumption of stimulants
- Tobacco (smoking)
Disease-related trigger factors
Infectious and parasitic diseases (A00-B99).
- Viral infections, unspecified
Neoplasms – tumor diseases (C00-D48).
- Paraneoplastic – subacute cutaneous lupus erythematosus may occur in the setting of cancer, v.a. gastric carcinoma, liver carcinoma, mammary carcinoma (breast cancer), prostate carcinoma (prostate cancer), uterine carcinoma (uterine cancer), Hodgkin’s lymphoma, squamous cell carcinoma (skin cancer, of the head and neck).
Pregnancy, childbirth and puerperium (O00-O99).
- Worsening of findings during pregnancy is possible.
Other causes
- Irritative stimuli
- UV light – sun exposure, artificial light sources (solarium).
Medications that are said to have an association with lupus erythematosus:
- ACE inhibitors (antihypertensive) – captopril, enalapril, lisinopril, cilazapril.
- Antiestrogens such as anatrozole or tamoxifen.
- Beta blockers (antihypertensive) – acebutol, oxprenolol, practolol
- Biopharmaceuticals (synonyms: biopharmaceuticals, biologicals, biologics or biologics) – etanercept, efalizumab, adalimumab, infliximab.
- Bupropion (nicotine cessation medication).
- Calcium channel blockers (antihypertensive) – diltiazem, verapamil, nifedipine, nitrendipine.
- Carbamazepine (anti-epileptic).
- Quinidine
- Chloropromazine (neuroleptic)
- Docetaxel (chemotherapeutic agent)
- D-Penicillamine
- Fluorouracil (chemotherapeutic agent)
- Griseofulvin (mycotic)
- Hepatitis B vaccination – reported to be able to trigger cutaneous lupus erythematosus.
- HMG-CoA reductase inhibitors (statins) – simvastatin, pravastatin
- Hydantoin (antiepileptic) – no longer in use today.
- Hydralazine (antihypertensive)
- Hydrochlorothiazide (HCT)
- Interferon
- Isoniazid (tuberculostat)
- Live vaccines should not be used in cutaneous lupus erythematosus with immunosuppressive therapy
- Leflunomide (immunosuppressant).
- Leuprorelin – hormonal drug, which is used mainly in prostate cancer.
- Naproxen (analgesic)
- Estrogens
- Penicillamine
- Phenytoin (antiepileptic)
- Piroxicam (non-steroidal anti-inflammatory drug, NSAID).
- Procainamide (local anesthetic)
- Proton pump inhibitors – lansoprazole, pantoprazole, omeprazole.
- Reserpine (antihypertensive).
- Rifampicin (tuberculostatic drug)
- Spironolactone (potassium-sparing diuretic).
- Sulfasalazine
- Terbinafine (antifungal) → subacute cutaneous lupus erythematosus (SCLE).
- Ticlopidine hydrochloride (ADP-antagonizing platelet function inhibitor).
Köbner phenomenon
In Köbner’s phenomenon, a non-specific skin irritation triggers the skin symptoms that already exist due to a skin disease in another part of the body. Köbner’s phenomenon can be triggered by the following skin irritations:
- Argon laser treatment
- DNCB (dinitrochlorobenzene) sensitization
- Performance of electromyography – registration of the electrical activity of the muscles can lead to the triggering of the Köbner phenomenon in lupus erythematosus profundus.
- Scratching
- Cryotherapy (cold treatment)
- Moxibustion – method from traditional Chinese medicine.
- Nickel contact dermatitis
- Surgical procedures
- Smallpox vaccination
- Radiatio (radiotherapy)
- Tattoo
- UVA emission of a photocopier
- Burns
- Wounds, bite injuries
