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Wegener's granulomatosis

In medicine (rheumatology), Wegener's granulomatosis is a form of vasculitis that affects the lungs, kidneys and other organs. Due to its end-organ damage, it can be a serious disease that requires long-term immune suppression. more...

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It is part of a larger group of vasculitic syndromes that all feature positivity for ANCAs (antineutrophil cytoplasmic antibodies) and affect small and medium-sized blood vessels. Apart from Wegener's, it includes Churg-Strauss syndrome and microscopic polyangiitis.

Signs and symptoms

Initial signs are protean, and diagnosis can be severely delayed due to the non-specific nature of the symptoms. The rhinitis is generally the first sign in most patients.

  • Upper airway, eye and ear disease:
    • Nose: pain, stuffiness, nosebleeds, rhinitis, crusting, saddle-nose deformity
    • Ears: conductive hearing loss due to Eustachian tube dysfunction, sensorineural hearing loss (unclear mechanism)
    • Eyes: pseudotumours, scleritis, conjunctivitis, uveitis, episcleritis
  • Airways:
    • Trachea: subglottal stenosis
    • Lungs: pulmonary nodules, infiltrates (often interpreted as pneumonia), cavitary lesions, pulmonary haemorrhage causing hemoptysis), and rarely bronchial stenosis.
  • Kidney: rapidly progressive segmental necrotising glomerulonephritis (75%), leading to chronic renal failure
  • Arthritis: Pain or swelling (60%), often initially diagnosed as rheumatoid arthritis
  • Skin: nodules on the elbow, purpura, various others (see cutaneous vasculitis)
  • Nervous system: occasionally sensory neuropathy (10%) and rarely mononeuritis multiplex
  • Heart, gastrointestinal tract, brain other organs: rarely affected.

Diagnosis

Vasculitis such as Wegener's granulomatosis is usually only suspected when a patient has had unexplained symptoms for a longer period of time. Determination of ANCAs can aid in the diagnosis, but positivity is not conclusive, and neither are negative ANCAs enough to reject the diagnosis. Cytoplasmic staining ANCAs that react with proteinase 3 (cANCA) are associated with Wegener's.

If the patient has renal failure or cutaneous vasculitis, these are the most logical organs to obtain a biopsy from. Rarely, thoracoscopic lung biopsy is required. On histopathological examination, a biopsy will show leukocytoclastic vasculitis with necrotic changes and granulomatous inflammation. The latter is the main reason for the appellation of "Wegener's granulomatosis", although it is not an essential feature. Unfortunately, many biopsies can be aspecific and 50% provide too little information for the diagnosis of Wegener's.

Differential diagnosis can be extensive. ANCAs can be positive after the use of certain drugs, and other forms of vasculitis can present with very similar symptoms. The saddle-nose deformity is also seen in cocaine abuse.

Read more at Wikipedia.org


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Case report: Wegener's granulomatosis presents as pulmonary infection
From American Family Physician, 3/15/05 by Matthew Falagas

TO THE EDITOR: A 35-year-old man with relapsing infections of the upper respiratory tract and nasal bleeding for five months was admitted to our hospital. The patient had been discharged from another hospital after showing no clinical improvement from one month of antibacterial and antituberculous treatment, and refusing to undergo lung operation for the postulated diagnosis of pulmonary abscess.

Physical examination on admission to our hospital showed fever as high as 38.8[degrees] C (101.9[degrees] F), pulse of 80 beats per minute, and blood pressure of 120/70 mm Hg. The only abnormal findings on physical examination were small blood clots in the nose and ronchi in the upper right lung field. Laboratory tests revealed the following: white blood cell count of 13,230 per [mm.sup.3] (13.2 x [10.sup.9] per L [77.9 percent neutrophils, 12.9 percent lymphocytes]); hematocrit level, 36.3 percent; hemoglobulin level, 11.4 g per dL (114 g per L); platelet count, 588,000 per [mm.sup.3] (5,880 x [10.sup.9] per L); C-reactive protein 21.74 mg per dL (normal values less than 0.50 mg per dL); and erythrocyte sedimentation rate (ESR), 105 mm per first hour (normal values zero to 20 mm per hour). Laboratory tests for renal function, including urinalysis, were normal. Chest radiography and computed tomography (CT) of the thorax showed a large infiltrate with a central cavitation in the right upper pulmonary field (see accompanying figure). Craniofacial CT revealed ethmoid sinusitis.

[FIGURE OMITTED]

The diagnosis of Wegener's granulomatosis was suggested by a significantly increased titer of the cytoplasmic-type antineutrophil cytoplasmic antibody (c-ANCA) of 65.3 u (normal: up to 30 u), and was confirmed by histopathologic examination of excised nasal mucosal specimens. The patient received treatment with cyclophosphamide, prednisolone, and cotrimoxazole, which improved symptoms and laboratory test abnormalities.

When fever persists in a patient with a significantly high ESR value, and radiographic and CT findings reveal sinusitis or a large pulmonary infiltrate with a central cavitation, Wegener's granulomatosis must be considered. (1) Differential diagnosis of a pulmonary abscess-like lesion in an imaging test should include bronchial neoplasms (either as necrotizing carcinoma or as the cause of poststenotic cavernous pneumonia that led to an abscess), infectious diseases (like tuberculosis, aspergillosis and other fungal infections, Pneumonocystis carinii pneumonia, and actinomycosis), pulmonary embolism, or vasculitis (primarily Wegener's granulomatosis). (2,3) Surgical lung biopsy may establish the final diagnosis; however, when Wegener's granulomatosis is suspected, the upper respiratory tract may be involved, making diagnosis less invasively established from histopathologic examination of biopsied nasal specimens. (4-6)

The diagnostic clue from our patient's clinical history was the upper respiratory tract symptoms for five months with progressive deterioration and nasal bleeding. Otorhinolaryngologists and other physicians should always have a high index of suspicion because Wegener's granulomatosis often presents at early stages as a localized form with disease activity limited to the upper respiratory tract. (6) In most cases, Wegener's granulomatosis is diagnosed histologically on biopsy specimens taken from the ear, nose, and throat region. Initially, the disease acute-phase proteins and c-ANCA have a normal value. (5) Early diagnosis can lead to early institution of the appropriate treatment and can prevent the irreversible destructive lesions of the disease.

MATTHEW FALAGAS, M.D., M.SC.

Adjunct Assistant Professor of Medicine

Tufts University School of Medicine Boston, Massachusetts

Alfa HealthCare Infectious Diseases Clinic

"Henry Dunant" Hospital

9 Neapoleos St. Marousi, Athens, Greece 15132

REFERENCES

(1.) Kariv R, Sidi Y, Gur H. Systemic vasculitis presenting as a tumorlike lesion. Four case reports and an analysis of 79 reported cases. Medicine 2000;79:349-59.

(2.) Allewelt M, Lode H. Diagnosis and therapy of abscess forming pneumonia [in German]. Ther Umsch 2001;58:599-603.

(3.) Sando Y, Sugita Y, Kaneko K, Ubukata M, Motegi M, Takayanagi N. Three cases of pulmonary actinomycosis [in Japanese]. Nihon Kyobu Shikkan Gakkai Zasshi 1992;30:1869-73.

(4.) Dechambre S, Driesschaert P, Goncette L, Pringot J. Unilateral peripheral pulmonary nodules: initial symptoms of a limited form of Wegener's disease [in French]. J Belge Radiol 1996;79:162-4.

(5.) Paulsen JI, Rudert H. Manifestations of primary vasculitis in the ENT region [in German]. Z Rheumatol 2001;60:219-25.

(6.) Rasmussen N. Management of the ear, nose, and throat manifestations of Wegener granulomatosis: an otorhinolaryngologist's perspective. Curr Opin Rheumatol 2001;13:3-11.

COPYRIGHT 2005 American Academy of Family Physicians
COPYRIGHT 2005 Gale Group

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