Rheumatoid vasculitisM06.99

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Rheumatism and vasculitis, vasculitis for rheumatism; rheumatoid vasculitis; SRV; systemic rheumatoid vasculitis; Vasculitis and rheumatism; Vasculitis in rheumatoid arthritis; vasculitis rheumatoid

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DefinitionThis section has been translated automatically.

Rheumatoid (necrotizing) vasculitis is a rare, often severe and lethal extra-articular complication (systemic vasculitis) of a usually long-term (10-15 years) rheumatoid arthritis (RA). Rheumatoid vasculitis usually affects medium-sized arteries, more rarely venous vessels in various organs and the skin.

Occurrence/EpidemiologyThis section has been translated automatically.

m>w (for rheumatoid arthritis: w:m=2/3:1)

EtiopathogenesisThis section has been translated automatically.

The cause of rheumatoid vasculitis is as little known as the underlying disease itself.

ManifestationThis section has been translated automatically.

The mean age of onset of the disease was about 70 years in a somewhat larger collective (n=18). The incidence of rheumatoid vasculitis has apparently been reduced in recent years by adequate therapy of the underlying disease.

Clinical featuresThis section has been translated automatically.

Rheumatoid vasculitis is a systemic vasculitis that occurs intermittently with severe general symptoms such as fatigue, fever, weight loss and arthritis, and is an extra-articular, complicative symptom accompanying rheumatoid arthritis.

The following organ manifestations may occur:

  • Kidneys (rare): proteinuria and microhaematuria, often also leukocyturia. Arterial hypertension often develops.
  • Cardiopulmonary: pleuritis, pericarditis, diffuse alveolitis and COPD, pulmonary hypertension.
  • Coronary arteries: pectanginous complaints.
  • Neurological complications (frequent): this is vasculitis of the vasa nervorum, usually of the distal nerves of the limbs, with consecutive sensory or mixed sensory-motor neuropathy.
  • Digital vessels: Vasculitic occlusion of digital vessels with digital necroses.
  • Skin: the dermatological spectrum covers a wide range of vasculitic diseases. Immunocomplex vasculitides are described under the clinical picture of acute (non-IgA) leukocytoclastic small vessel vasculitis (purpura). Furthermore, chronic recurrent vasculitis of medium-sized skin vessels with papulo-necrotic skin alterations. Also ulcerated nodes or necroses. Frequently the nail folds are also affected.
  • Nails: in acute attacks, possibly reddish crescents in the nail bed due to subungual bleeding.

HistologyThis section has been translated automatically.

IgG/IgM and sometimes IgA-positive leukocytoclastic vasculitis of the postcapillary venules Rarely is the infestation of medium-sized vessels at the croium/subcutaneous junction detectable.

TherapyThis section has been translated automatically.

This polyorganic systemic vasculitis must be treated depending on the organ involvement and the extent of the overall constellation.

For uncomplicated dermatologically accentuated clinical pictures an immunosuppressive therapy with glucocorticoids (prednisolone 30-50mg p.o. /day in combination with methotrexate 15-20mg s.c./week) may be sufficient.

The use of cyclophosphamide, rituximab or IVIG is reserved for severe courses of the disease.

Hydroxychloroquine and low-dose aspirin may play a protective role in the aftermath of the acute situation.

Progression/forecastThis section has been translated automatically.

Rheumatoid vasculitis is clinically severe. According to the literature, the 1-year mortality rate after diagnosis is 12%, the 5-year mortality rate 60%.

Case report(s)This section has been translated automatically.

Medical history: The 73-year-old patient has been suffering from a known rheumatoid arthritis (RA) for 12 years with typical morning stiffness, symmetrical involvement of the small joints.

Findings: Metacarpophalangeal (MCPs), proximal interphalangeal (PIPs) and metatarsophalangeal joints (MTPs) with pain and swelling in movement. Clear painful handshake (so-called Gaensslen's sign). The distal interphalangeal joints were uninvolved. For 1 year, inflammatory skin lesions on both lower legs and the back of the foot occurred intermittently under a considerable feeling of illness with fever, recurrent pericarditis and myocarditis with infark-like ECG changes and increased arthritic activity.
Dermatological findings: on both calves in an area of 20 x 10 cm size spontaneous and pressure painful, blurred surface smooth erythema, plaques and subcutaneous clots were found. In the centre of the lesion a 2.5 x 3.0 cm crusty covered ulcer was found.
Laboratory: BSG: 60/90mm/h (<20/<30); uric acid 9.2 mg/dl (<7.0) significantly elevated, CRP: 10.6 mg/dl (<5), rheumatoid factor IgA: 39 IU/ml (<14) IgG: 12 IU/ml (<7); citrulline AK 290 U/ml (<7). ANA, DNA-AK, SSA, SSB, C-ANCA, p-ANCA neg. troponin t-hs 10 pg/ml (<14.0) Urinary status: erythrocyturia, proteinuria. Glucose fasting: 90mg/dl (<100). Liver values without
histology: Regular orthokeratotic epidermis. In the middle corium conspicuous medium sized, partially obliterated vessels with thickened fibrinoid walls, focal wall necrosis and intra- and extravascular infiltrates of lymphocytes, macrophages, eosinophils and neutrophils. Sometimes foamy macrophages and extravasations of erythrocytes. Clear panniculitic accompanying reaction.
Therapy:initial 150 mg prednisolone i.v./day combined with 20 mg methotrexate s.c./week. Including sudden improvement of general symptoms of receding skin changes (ulcer healed completely within 3 weeks). Permanent therapy with 5 mg prednisolon/day and 20 mg methotrexate/week.

LiteratureThis section has been translated automatically.

  1. Cojocaru M et al (2015) New insight into the rheumatoid vasculitis. Rome J Internal Med 53:128-q32.
  2. Gutiérrez-González LA (2016) Biological Therapy-Induced Systemic Vasculitis. Curr Rheumatol Rep 18:39.
  3. Lori TM et al (1999) Cutaneous necrotizing vasculitis. Relation to systemic disease. Adv.Exp Med Biol 455: 115-125
  4. Makol A et al (2015) Rheumatoid vasculitis: an update. Curr Opin Rheumatol 27:63-70.
  5. Mansour R et al. (2016) Secondary vasculitis in rheumatoid arthritis. Act Dermatol 42: 273-275
  6. Ntatsaki E et al (2014) Systemic rheumatoid vasculitis in the era of modern immunosuppressive therapy. Rheumatology (Oxford) 53:145-152.
  7. Radic M et al (2013) Overview of vasculitis and vasculopathy in rheumatoid arthritis--something to think about. Clin Rheumatol 32:937-942.
  8. Xue Y et al. (2016) Skin Signs of Rheumatoid Arthritis and its Therapy-Induced Cutaneous Side Effects. At J Clin Dermatol 17:147-162.

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Last updated on: 29.10.2020