CASE REPORT

Limited Wegener’s granulomatosis—is it limited?

Svetlana Lisitsin & Raymond Farah & Moshe Shay

Received: 8 March 2007 /Revised: 15 March 2007 /Accepted: 17 March 2007 / Published online: 31 March 2007# Clinical Rheumatology 2007

Abstract Complete heart block associated with Wegener’sgranulomatosis (WG) is rare especially in the limited formof the disease. We describe a case of a 43-year-old womanwith a limited form of WG who developed a complete heartblock. Prompt treatment with steroids and cyclophospha-mide led to temporary regression of complete heart block.Further involvement of lung was treated successfully bytumor necrosis factor-alpha inhibitor infliximab. Cardiacrhythm abnormalities should always be kept in mind bothin diagnosis and follow-up of WG.

Keywords Complete heart block . Limited .

Wegener’s granulomatosis

Case report

A 43-year-old woman was in our care because of a limitedform of Wegener’s granulomatosis (WG) with sinusinvolvement, nasal septum destruction, proven by biopsyand c-ANCA positive. She had been treated by trimetho-prim sulphamethoxasol and prednisone followed by pred-nisone and methotrexate, and her disease was under control.This patient was admitted to our hospital with complaints ofweakness, slow heart rate, epistaxis, and headache.

On physical examination, the patient’s blood pressurewas 140/80 mm/Hg, pulse rate 42 beats/min, regular. Thenasal mucosa was inflamed with crust formation. Thecardio-respiratory examination was clinically unremarkable,and general physical examination was normal.

The laboratory tests including complete blood count,electrolytes, glucose, blood urea nitrogen (BUN), creatinine,

and liver enzymes were within normal limits; erythrocytesedimentation rate was 70 mm/h. Urinalysis was normal. Thechest X-ray was normal. Electrocardiograph showed completeheart block with ventricular rate of 42 beats/min and narrowQRS complexes. Echocardiography showed normal leftventricle contractility and moderate mitral regurgitation.

Diagnosis of complete atrio-ventricular block was made,temporary pacemaker was inserted, and the patient receiveda pulse of cyclophosphamide 1 g intravenous and methyl-prednisolone 1 g/day for 3 days followed by oralprednisone. The patient’s condition was stabilized, andafter the second pulse of methylprednisone, complete atrio-ventricular block resolved; the rhythm returned to sinuswith first-degree atrio-ventricular block.

During the next 6 months, the patient was admitted tohospital twice more for complete heart block and success-fully treated with cyclophosphamide and methylprednisone,followed by intravenous immunoglobuline; the rhythmreturned to sinus rhythm.

When the patient returned with recurrence of the samesymptoms for the third time, a permanent cardiac pace-maker was inserted.

Despite the treatment with cyclophosphamide andprednisone, nasal septum and sinus destruction continued,and involvement of lung appeared with cavitation noticedon computerized tomography (CT) scan.

Thus, treatment with tumor necrosis factor-α inhibitor,infliximab, began.

The treatment showed good results: Lung cavitationdisappeared, and further destruction of nasal septum andsinuses came to a halt.

Discussion

The classical form of WG is necrotizing granulomatousangiitis that involves the upper and lower respiratory tract

Clin Rheumatol (2007) 26:1999–2000DOI 10.1007/s10067-007-0612-3

S. Lisitsin : R. Farah (*) :M. ShayDepartment of Internal Medicine F, Western Galilee Hospital,P.O.B 21, Nahariya 22100, Israele-mail: Raymond.Farah@naharia.health.gov.il

and kidneys. A limited form of WG exists when only oneor two organ systems are involved, and without renalinvolvement [1]. There have been reported cases of limitedforms of WG with respiratory tract, skin, oral, palatal, ear,and salivary gland involvement [2–4].

We describe a patient with a limited form ofWGwith upperrespiratory tract involvement and cardiac complications.

Although traditionally, cardiac involvement in WG isthought to be rare, it is not as uncommon as has beenbelieved. Different articles report a vastly varying in-cidence of cardiac involvement ranging from 8 to 44%[5–7]; these variations may be due to the varying history ofthe disease, the ethnic origin of patients, the medicalspecialty of the group investigating the patients, theinfluence of cyclophosphamide on the natural course ofthe disease, and the ability of techniques such as echocar-diography and Holter monitoring to diagnose subclinicalinvolvement.

The pathologic involvement of the heart in WG includes(as percentage of all cases with cardiac involvement):coronary arteritis, 50% cases; myocarditis, 25%; valvulitisor endocarditis, 21%; conduction system granulomata,17%; arteritis of the blood supply to the atrio-ventricularnode, 13%; myocardial infarction, 11%; epicarditis, 8% [8].

The likely explanation for the conduction disturbances inour patient is arteritis of atrio-ventricular nodal artery ordirect granulomatosis involvement of conduction system. Arapid resolution with pulse of methylprednisone favors anassociated coronary arteritis.

Complete heart block caused by WG has been reportedin only several other cases [8–12]. Our patient is only thesecond report of atrio-ventricular block in patient withlimited form of WG [12].

Conclusion

We describe a patient with limited form of WG with furtherinvolvement of the heart and lungs. It is unclear if thelimited form of WG represents a separate entity or an earlyand modified state of the systemic disease.

There is a need to emphasize that conventional treatmentof this disease by cyclophosphamide and steroids had noeffect. Based on latest reports of successful treatment ofWG with infliximab [13–15], we attempted this treatment,with good results.

The present case shows that awareness of the rarecardiac conduction defect possible in WG is important forearly diagnosis and appropriate treatment.

It remains to be seen whether more widespread use oftelemetry and Holter monitoring will find an increase inasymptomatic heart block in WG patients.

References

1. Luqmani RA, Bacon PA, Beaman M et al (1994) Classical versusnon-renal Wegener’s granulomatosis. Q J Med 87(3):161–167

2. Ahmad I, Lee WC, Nagendran V, Wilson F, Shortridge RT (2000)Localised Wegener’s granulomatosis in otolaryngology: a reviewof six cases. ORL J Otorhinolaryngol Relat Spec 62(3):149–155

3. Hansen LS, Silverman S Jr, Pons VG et al (1985) LimitedWegener’s granulomatosis. Report of case with oral, renal andskin involvement. Oral Surg Oral Med Oral Pathol 60(5):524–531

4. Rahilly G, Rahilly M (2000) A case of palatal Wegener’sgranulomatosis. Oral Dis 6(4):259–261

5. Hoffman GS, Kerr GS, Leavitt RY et al (1992) Wegener’sgranulomatosis: an analysis of 158 patients. Ann Intern Med116:488–498

6. Fauci AS, Haynes BF, Katz P, Wolff SM (1983) Wegener’sgranulomatosis: prospective clinical and therapeutic experiencewith 85 patients for 21 years. Ann Intern Med 98:76–85

7. Pinching AJ, Lockwood CM, Pussell BA et al (1983) Wegener’sgranulomatosis: observation on 18 patients with severe renaldisease. Q J Med 208:435–460

8. Forstot JZ, Overlie PA, Neufeld GK, Harmon CE, Forstot SL(1980) Cardiac complications of Wegener’s granulomatosis: acase report of complete heart block and review of literature. SeminArthritis Rheum 10:148–154

9. Handa R, Wali JP, Aggarwal P, Wig N, Biswas A, Kumar AK(1997) Wegener’s granulomatosis with complete heart block. ClinExp Rheumatol 15:97–99

10. Wilcke JT, Nielsen PK, Jacobsen TN (2003) Reversible completeheart block due to Wegener’s granulomatosis. Int J Cardiol89:297–298

11. Suleymenlar G, Sarikaya M, Sari R, Tuncer M, Sevinc A (2002)Complete heart block in a patient with Wegener’s granulomatosisin remission—a case report. Angiology 53(3):337–340

12. Ghaussy NO, Du Clos TW, Ashley PA (2004) Limited Wegener’sgranulomatosis presenting with complete heart block. Scand JRheumatol 33:115–118

13. Lamprecht P, Voswinkel J, Lilienthal B et al (2002) Effectivenessof TNF-alpha blockade with infliximab in refractory Wegener’sgranulomatosis. Rheumatology (Oxford) 41:1303–1307

14. Kleinert J, Lorenz M, Kostler W, Horl W, Sunder-Plassmann G,Soleiman A (2004) Refractory Wegener’s granulomatosisresponds to tumor necrosis factor blockade. Wien KlinWochenschr 116(9–10):334–338

15. Bartolucci P, Ramanoelina J, Cohen P et al (2002) Efficacy of theanti-TNF-alpha antibody infliximab against refractory systemicvasculitides: an open pilot study on 10 patients. Rheumatology(Oxford) 41(10):1126–1132

2000 Clin Rheumatol (2007) 26:1999–2000