Rheumatoid vasculitis M06.99

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.

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

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

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.

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.

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.

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.

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

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.

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