Immune complex vasculitis

Classification according to ICD-10
M31	Other necrotizing vasculopathies
M31.0	Hypersensitivity angiitis, including Goodpasture syndrome
ICD-10 online (WHO version 2019)

The immune complex is a non-bacterial inflammation of the blood vessels ( "vasculitis" ), is adjacent to the Wegener's granulomatosis ( granulomatous vasculitis ) and ANCA-associated vasculitis to the subgroups of vascular inflammation . In immune complex vasculitis, the vessel walls are attacked by an immunological reaction. If the body's own antibodies , bacteria or drug components are responsible for the formation of immune complexes that are deposited in the vessel walls and damage them, a drop in complement values can be detected in the blood . The histology of an immune complex vasculitis is characterized by: endothelial swelling , diapedesis of granulocytes and lymphocyte accumulation around the blood vessels (perivascular).

Cutaneous immune complex vasculitis

The Cutaneous immune complex vasculitis , including allergic vasculitis , is a small vessel vasculitis. Episodic immune complex vasculitis often follows an infection ( streptococci , gastrointestinal infections, erysipelas , tonsillitis , viral infections, viral hepatitis ). If the disease is chronically recurrent , the main cause is hepatitis C , followed by collagenosis (especially with urticarial symptoms). Drugs such as antibiotics, foreign proteins and sulfonamides can, in addition to food additives, lead to necrotizing vasculitides. The development of skin changes is promoted by physical exertion.

A distinction is made between different types, transitional and mixed forms:

Hemorrhagic type : classic rheumatic purpura, anaphylactoid purpura, Henoch-Schönlein purpura .
Hemorrhagic-necrotic type : flat, reddish-blackish necroses of the skin.
Papulonecrotic type : Papules that become centrally necrotic and later heal with scarring. The extensor sides of the extremities are particularly affected, especially the knees and elbows.
Polymorph-nodular type : macular, papular, urticarial and nodular hemorrhagic phenomena next to each other. May be reminiscent of erythema multiforme.
Urticarial vasculitis: Special form of allergic vasculitis, which is characterized by the long persistence of the wheals and a hemorrhagic note.

Systemic immune complex vasculitis

Systemic immune complex vasculitis includes cryoglobulinemic vasculitis in connection with a hepatitis C virus infection (HCV). It affects the skin, kidneys and the central nervous system.

The Polyarteritis nodosa is a form of systemic immune complex, in which especially the small and medium-sized vessels ( arterioles ) are affected. There are inflammatory nodules arranged like a string of pearls.

Symptoms of illness

The main symptoms are livedo reticularis (blue-reddish, reticular skin changes) with kidney involvement (vascular nephropathy ) as well as gastrointestinal and neurological symptoms.

General unspecific symptoms are fatigue, muscle pain , heaviness in the legs, weight loss and night sweats. Neurological symptoms can be found in the classic form: in about 75% of the cases one sees nerve inflammation associated with paralysis ( mononeuritis simplex ); Headache and circulatory- related general brain disorders occur in around 20% of cases. Focal hemiplegia , epilepsy and cranial nerve failure are rare . Occasional involvement of the spinal cord is found . Changes in the skin are the livedo reticularis mentioned above , subcutaneous aneurysms can be felt as painful nodules. A heart involvement can manifest itself as angina pectoris up to a heart attack . Colic- like abdominal pain, bleeding and intestinal infarction occur on the part of the digestive tract . Kidney involvement manifests itself in microaneurysms of the kidney vessels; glomerulonephritis usually does not occur. An association with hepatitis B or C is rare.

forecast

Without therapy and with involvement of the central nervous system, the prognosis is poor.

therapy

Initially, steroids and cyclophosphamide are combined for therapy . The intermittent intravenous and continuous administration of cyclophosphamide are controversial. Antiviral substances and cautious immunosuppression are used in hepatitis B-associated cases.

literature

D. Hermann, T. Steiner, HC Diener: Vascular neurology: cerebral ischemia, hemorrhage, vascular malformations, vasculitis and vascular dementia . Thieme 2010.
MP Stoffel: Significance of the autoantibody findings for diagnosis and differential diagnosis of collagenoses and systemic vasculitides. Clinical and serological follow-up on 200 patients . Herbert Utz Verlag 1998.
H.-H. Peter, WJ Pichler, U. Müller-Ladner: Clinical Immunology . Urban & Fischer Verlag / Elsevier 2012.

Individual evidence

↑ K.-G. Hermann: Die rheumatoid arthritis: Pathologische Anatomie , p. 81. here online (accessed on September 17, 2012).
↑ O. Braun-Falco, HH Wolff u. a .: dermatology and venereology . Springer Medizin Verlag, 2005, ISBN 3-540-40525-9 , pp. 785-786.
^ HW Doerr, W. Gerlich Medical Virology: Basics, Diagnostics, Prevention and Therapy . Thieme 2002, ISBN 3-13-113962-5 , p. 258.
↑ M. Reiss specialist knowledge of ENT medicine: differentiated diagnostics and therapy . Springer Medizin Verlag Heidelberg, 2009. ISBN 3-540-89440-3 , p. 101.

[1] K.-G. Hermann: Die rheumatoid arthritis: Pathologische Anatomie , p. 81. here online (accessed on September 17, 2012).

[2] O. Braun-Falco, HH Wolff u. a .: dermatology and venereology . Springer Medizin Verlag, 2005, ISBN 3-540-40525-9 , pp. 785-786.

[3] HW Doerr, W. Gerlich Medical Virology: Basics, Diagnostics, Prevention and Therapy . Thieme 2002, ISBN 3-13-113962-5 , p. 258.

[4] M. Reiss specialist knowledge of ENT medicine: differentiated diagnostics and therapy . Springer Medizin Verlag Heidelberg, 2009. ISBN 3-540-89440-3 , p. 101.