Consultation and Reference Center for Vasculitis Diagnosis

The Vasculitis Consultation and Reference Center, chaired by Dr. Konstanze Holl-Ulrich, MD is the histopathology referral center of the national German Vasculitis Registry (GeVas).

The Consultation and Reference Center for histopathology vasculitis diagnostics is open to pathologists, rheumatologists, and other medical disciplines for the interpretation of tissue samples in vasculitis or suspected vasculitis cases.

Our focus is on ANCA-associated vasculitis, e.g. granulomatosis with polyangiitis (GPA). Tissue samples from any organ can be evaluated concerning possible vasculitis. In addition to conventional histopathological techniques and immunohistochemistry, molecular pathology methods are available in co-operation with our partners.

What is "Vasculitis"?

The term "vasculitis" is defined as an inflammation of blood vessels which can occur in any organ causing a multitude of various symptoms. Although vasculitis is a rare disease, multiple different types of vasculitis exist that may show rather similar clinical and histopathological features which makes the differential diagnosis often challenging. In finding typical changes associated with the various forms of the disease histopathology remains as gold standard. Only the pathologist can confirm the diagnosis and specify the type. The most frequent types of vasculitis are giant cell arteritis (temporal arteritis), ANCA-associated vasculitides, e.g. granulomatosis with polyangiitis (GPA) or cutaneous small vessel vasculitis (leukocytoclastic vasculitis).

For whom does the Consultation and Reference Center for Vasculitis Diagnosis work?

The Consultation and Reference Center for Vasculitis is not only available to pathologists, rheumatologists and other medical disciplines but also to patients seeking advice on the interpretation of vasculitis or suspected vasculitis in tissue samples.

In addition to the current specimen it might be helpful to re-evaluate tissue obtained during previous procedures in the patient’s medical history (e.g. former operations, endoscopies etc.) in order to make a confident diagnosis.

If a second opinion on previous tissue samples is required, the clinician should contact and advice the primary histopathology department to send the material directly to the Consultation and Reference Center for Vasculitis Diagnosis.

How to ship the tissue?     

pdf Vasculitis request form

Fresh tissue specimens should be routinely fixed in 4% buffered formaldehyde and sent by mail.

Pots and laboratory forms are available here .

In cases which need direct immunofluorescence (DIF), e.g. suspected IgA-vasculitis, please contact our department upfront (Tel. 0049 40 53805-751/752). These techniques require a separate biopsy to be sent directly to our co-operation partners as fresh material (in buffer only). Short transport time is mandatory for optimal tissue preservation (Please do not biopsy or mail samples short before weekends!).

For second opinion, please advise the primary histopathology department to send slides together with the paraffin blocks.

Please provide also information on the clinical aspect, the laboratory data and the patient’s medical history (e.g. a copy of the last medical report). For typing the vasculitis correlation of the histopathological appearances with the clinical findings might be essential.

What is important when taking tissue samples?

Generally, vasculitis can occur in any part of the body. However, typical changes are often distributed only focally in the organ.  Therefore, the tissue sample should include a viable and affected vessel. In severe cases, vasculitis leads to necrosis. One has to bear in mind that in the vicinity of necrosis, non-specific secondary vessel changes are regularly found preventing an accurate diagnosis of vasculitis.

Helpful guidelines:

  • ENT: several biopsies (minimum of 0.3 cm) from an endoscopically active area.

  • Skin:

    • Palpable purpura/suspected small vessel vasculitis: Punch biopsy (4-5 mm) or excisional biopsy from a fresh efflorescence (< 48h duration).

    • If IgA-vasculitis is suspected, a separate tissue sample (in buffer only) is required for direct immunofluorescence (DIF). Please contact us upfront (Tel. 0049 40 53805-751/752). A separate biopsy has to be sent directly to our co-operation partners. Please note, in order to keep transportation times short do not ship samples short before the weekend.

    • Subcutaneous nodules: Excisional biopsy including subcutis reaching the fascia. In order to get sufficient subcutaneous tissue, the fusiform biopsy should be en bloc (skin and subcutaneous tissue of the same shape and extension).

    • Skin ulcer: slender excisional biopsy of skin and subcutaneous tissue, 0.5 cm wide, laterally extending at least 2.0 cm beyond the edge of the ulcer. To get appropriate subcutaneous tissue, the biopsy should not be wedge-shaped but should have the same extension in the subcutaneous tissues as on the skin surface.

  • Lung, nodular change: ideally the complete nodule as well as surrounding lung tissue

  • Gastrointestinal tract: In case of ischaemic changes, please take several biopsies of the transition zone to viable mucosa/submucosa. In surgical resection specimens attached mesentery/mesocolic fat parallel to the gut including blood vessels is required.

  • Muscle and neural tissues: Tissue must be sent to a department of neuropathology first in order to exclude primary muscle or nerve diseases. Please follow the advice of the neuropathologist regarding handling and shipping of the specimen. Should the suspicion of vasculitis persist, the neuropathologist should be asked to transfer the material for second opinion to the Consultation and Reference Center for Vasculitis Diagnosis.

Please note, the neuropathologist might not be able to conduct a full neuropathological examination of tissue which has been already routinely processed in formaldehyde and paraffine embedded elsewhere. Therefore, please inform the neuropathologist first and ask for advice.

  • Kidney: The Consultation and Reference Center for Vasculitis Diagnosis does not deal with kidney biopsies. These are exclusively processed and diagnosed by nephropathologists.

 

Contact

Dr. med. Konstanze Holl-Ulrich

  • Tel. 040 53805-170
  • Fax 040 53805-750

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Contact

  • 0049 40 53805-751/752
  • 0049 40 53805-750

Opening hours

Mon-Fri 7:00 am-5:00 pm
Sat-Sun closed