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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.

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Wegener's Granulomatosis Responding to Antituberculous Drugs - )
From CHEST, 2/1/01 by Mikio Toyoshima

We present a ease of Wegener's granulomatosis (WG) that responded to antituberculous drugs. A 44-year-old woman with multiple nodules on chest radiograph received a diagnosis of pulmonary tuberculosis because open-lung biopsy specimens showed caseous granulomas. Her chest shadows underwent repeated resolution after the start of antituberculous treatment, and relapse after the cessation of the drugs. Antineutrophil cytoplasmic antibody was positive (14 enzyme-linked immunosorbent assay units), and the second lung biopsy specimens showed necrotizing granulomas and vasculitis without pathogenic organisms. Thus, the patient received a diagnosis of WG and was successfully treated with trimethoprim/sulfamethoxazole 10 years after her initial evaluation. Antituberculous drugs were effective in this case of WG.

(CHEST 2001; 119:643-645)

Key words: antituberculous drug; trimethoprim/sulfamethoxazole; Wegener's granulomatosis

Abbreviations: ELISA = enzyme-linked immunosorbent assay; T/S = trimethoprim/sulfamethoxazole; WG = Wegener's granulomatosis

Wegener's granulomatosis (WG) is a chronic systemic necrotizing vasculitis that classically affects the upper respiratory tract, lungs, and kidneys. Although the drugs used to treat this disease usually are cyclophosphamide and glucocorticoids, DeRemee et al[1] reported cases of WG successfully treated with trimethoprim/sulfamethoxazole (T/S). T/S is used alone or in combination with immunosuppressive agents in the treatment of WG and to prevent a relapse of WG.[1,2] Although the mechanisms by which T/S exerts its effects in WG have not been clarified as yet, the possible involvement of its antimicrobial effect has been suggested.[2,3] Here, we describe a case of WG that responded to antituberculous drugs.

CASE REPORT

A 44-year-old woman presented with abnormal shadows on a chest radiograph taken during a medical checkup in August 1987, and was admitted to our hospital on September 13, 1987, for further evaluation. She presented no symptoms, and findings on physical examination were unremarkable. The chest radiograph revealed bilateral multiple nodular shadows (Fig 1, top, left). The leukocyte count was 4,800 cells/[micro]L with a normal differential count, and the erythrocyte sedimentation rate was 13 mm/h. Urinalysis showed no abnormality. Skin reaction to purified protein derivative was positive. She had not received a bacille Calmette-Guerin vaccine, nor did she come from an area with a high prevalence of tuberculosis. The patient underwent right open-lung biopsy. Specimens obtained from two nodules showed granulomas with central necrosis; no vasculitis was observed. Although special stains and tissue culture for acid-fast bacilli, fungi, and bacteria yielded normal results, she received a diagnosis of pulmonary tuberculosis, and was started on a regimen of isoniazid, 400 mg; rifampicin, 450 mg; and ethambutol, 750 mg. This regimen was continued for 6 months and was followed by a regimen of isoniazid and rifampicin for 6 months. At the end of treatment, the multiple nodular shadows had almost disappeared (Fig 1, top, right). However, a follow-up chest radiograph taken on September 12, 1995, revealed bilateral multiple nodular shadows (Fig 1, middle, left). Treatment was reinitiated with the same regimen as the first time, and the shadows disappeared (Fig 1, middle, right). In November 1996, she began to complain of productive cough, nasal discharge, and arthralgia. A chest radiograph taken on December 6, 1997, disclosed bilateral multiple nodular shadows and infiltrates with or without cavities (Fig 1, bottom, left). Sputum and nasal cultures for acid-fast bacilli and bacteria were normal. Treatment with isoniazid and rifampicin was started, and the shadows began to resolve (Fig 1, bottom, right). However, antineutrophil cytoplasmic antibody directed against proteinase 3 measured by enzyme-linked immunosorbent assay (ELISA) was 14 ELISA units (normal range, [is less than] 10 ELISA units). Since examination of a nasal biopsy specimen produced unremarkable findings, she was subjected to open-lung biopsy again. Specimens obtained from the right middle lobe showed necrotizing granulomas with vasculitis that were consistent with WG (Fig 2). Tissue cultures for acid-fast bacilli, fungi, and bacteria proved normal. Polymerase chain reaction for DNA of mycobacteria found in lung tissue was also normal. After the diagnosis of WG was established, T/S (400-mg tablet/80-mg tablet, four tablets daily) was substituted for antituberculous drugs. Her symptoms and the shadows observed on the chest radiograph resolved within a few weeks.

[Figures 1-2 ILLUSTRATION OMITTED]

DISCUSSION

In the present case, although the patient initially received a diagnosis of pulmonary TB during the course of the disease, based on her clinical and radiologic characteristics and the results of cultures of lung tissue specimens, it was established that she had WG. Surprisingly, the multiple nodular shadows observed on the chest radiograph began to disappear just after each time she was started on antituberculous drugs. Although the possibility of a coincidental spontaneous resolution at the time of treatment cannot be excluded, we believe that antituberculous drugs were effective against WG in our case, since the response was reproducible three times.

It is not clear which antituberculous drug was effective, or the mechanisms of its effects. However, as in case of T/S, it can be speculated that antituberculous drugs eliminate causal or precipitating agents in WG. Stegeman et al[3] reported the association between nasal carriage of Staphylococcus aureus and an increased risk of relapse of WG.[3] They postulated that T/S treatment reduced the frequency of relapses by eliminating or reducing S aureus in the upper airways.[2,3] van Putten et al[4] also described two cases of WG associated with S aureus infection in the lower respiratory tract.[4] From these reports, although S aureus was not detected in nasal and tissue cultures in our case, we speculated that elimination of S aureus by rifampicin might induce resolution of WG. Although we cannot provide direct evidence supporting this hypothesis, it is possible that microorganisms sensitive to antituberculous drugs are causal or precipitating agents of WG.

REFERENCES

[1] DeRemee RA, McDonald TJ, Weiland LH. Wegener's granulomatosis: observation on treatment with antimicrobial agents. Mayo Clin Proc 1985; 60:27-32

[2] Stegeman CA, Tervaert JWC, de Jong PE, et al. Trimethoprim-sulfamethoxazole (co-trimoxazole) for the prevention of relapses of Wegener's granulomatosis. N Engl J Med 1996; 335:16-20

[3] Stegeman CA, Tervaert JWC, Sluiter WJ, et al. Association of chronic nasal carriage of Staphylococcus aureus and higher relapse rates in Wegener granulomatosis. Ann Intern Med 1994; 120:12-17

[4] van Putten JWG, van Haren EHJ, Lammers JWJ. Association between Wegener's granulomatosis and Staphylococcus aureus infection? Eur Respir J 1996; 9:1955-1957

(*) From the Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan. Manuscript received November 30, 1999; revision accepted July 12, 2000.

Correspondence to: Mikio Toyoshima, MD, Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 3600 Handacho, Hamamatsu 431-3192, Japan

COPYRIGHT 2001 American College of Chest Physicians
COPYRIGHT 2001 Gale Group

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