A Masquerader Vasculitis as Usual: Time Is Tissue

M. Hari Meyyappan 1, Senthil Kumar1, S. Kandasamy1, T. Joseph1, Ravichandran1, R. Varun Prasanna1, Senthivel Murugan1, Dheenadhayalan1, S. Thilagavathy1, Vinodhini2, Kavitha2, Shantha Ravishankar2, Subbaiah Ramanaathan1,*

1Kauvery Hospital, Trichy, India

2Neuberg Ehrlich laboratory, Chennai, India

*Correspondence: drrms5@yahoo.in

Background

Wegener’s granulmatosis is a rare idiopathic inflammatory disorder affecting blood vessels of upper respiratory tract sinuses, lungs and kidneys. Histopathologic evidence of necrotizing granulomatous inflammation and pauci-immune small-vessel vasculitis though non-pathognomonic, is essential for a diagnosis [1]. Serologic evidence of antineutrophil cytoplasmic antibodies also supports a diagnosis. Non-specific clinical features and a frequent misdiagnosis makes it a challenge to clinch a diagnosis. Diagnosis is based usually on patient’s clinical presentation and supporting laboratory and histopathologic findings [2]. Immunosuppressive therapy with corticosteroids and Cyclophosphamide still remains as an effective, recognized and cost-effective therapeutic modality. Untreated disease carries a high mortality.

Case Presentation

A 58-year-old female came with right hemifacial pain associated with swelling since a week. She had angle of mouth deviated to the left, incomplete closure of right eyelid. She also reported occasional bleeding through the right nostril. She reported no fever, bleeding elsewhere. She had no significant weight or appetite loss. No dysphagia, hearing disturbances, seizures, visual disturbances, cough or dyspnea were present. She also complained of intermittent right ankle swelling associated with pain since last three years. She is a known hypothyroid on replacement therapy, optimally controlled. She sought neurology OPD services for her symptoms. In view of associated mastoiditis and sinusitis, Facial palsy was considered secondary to an ENT pathology and hence she was transferred under ENT services.

Physical examination then revealed a right facial LMN type palsy with swelling involving right side of face. Angle of mouth was deviated to the left, no obvious oral pathology was identified, no significant lymphadenopathy, organomegaly or other neurologic deficits were present. Chest examination and abdomen examination were normal. Nasal endoscopic examination revealed sinusitis, she was managed with antimicrobials, facial physiotherapy after which she improved initially. However, she came with progression of symptoms after two weeks. She looked sick with increased facial edema. Endoscopic examination revealed necrotic lesions and in view of COVID pandemic and recent surge of Mucormycosis, it was considered as a possible etiology. MRI of orbit, PNS and brain were done. It revealed sinusitis involving frontal, ethmoid and maxillary sinuses, however there was no evidence of bone involvement, intraorbital or intracranial extension. Chest CT showed no obvious pulmonary pathology. Endoscopic sinus surgery was planned as a diagnostic and therapeutic strategy. Presurgical laboratory evaluation revealed a normocytic anemia, neutrophil predominant differential count, reactive thrombocytosis, hypoproteinemia, hypoalbuminemia, normal serum creatinine, normal prothrombin and activated partial thromboplastin times. HBsAg, HCV and HIV serologies were negative. Endoscopic medial maxillectomy was done, necrotizing mucosal areas were identified and sent for histopathlogical examination. Scraping for KOH preparation and fungal culture showed no evidence of the same.

The next day in the ward she developed few palpable purpurae over her legs, infarcts were seen over soles of feet. Small non-tender vesicular lesions were picked up on the dorsum of skin over interphalangeal joints. Serum creatinine showed a rising trend, urine routine examination revealed microscopic hematuria and few pus cells. Urine culture and blood culture were sterile. Serum procalcitonin and LDH were not raised but CRP and d-dimer were raised. Echocardiogram done revealed a normal study and no vegetations ruling out an infective endocarditis. Ultrasound abdomen revealed only a fatty liver, neither an obstructive uropathy nor contracted kidneys. Small vessel vasculitis was considered in view of skin, renal and upper respiratory tract pathology. Serology sent for ANA, c-ANCA were reported positive by indirect immunoflorescence. Meanwhile histopathology of sinonasal tissue revealed a necrotizing granulomatous inflammation, however there were no fungal elements evident. Hence, considering above clinical presentation of a sinusitis like picture with LMN type facial palsy (mononeuritis), necrotizing granulomatous inflammation on histopathology of sinonasal mucosa, acute kidney injury with microscopic hematuria, c-ANCA positivity, negative infective etiology, Wegener’s granulomatosis was considered as the diagnosis. Patient was also worked up by internal medicine, rheumatology, nephrology, cardiology and clinical haematology teams with diagnostic support from radiology, microbiology and pathology. Family was counselled about the disorder, possibility of waxing and waning course, need for intense immunosuppressive therapy and possible side effects associated with immunosuppression. Need for urgent therapy was also explained in view of a possible rapid progressive renal failure (RPRF) due to a probable crescentic glomerulonephritis. After an informed consent she was started on pulse corticosteroid with Inj. Methylprednisolone after which her symptoms improved. She needs to kept on regular follow up.

Discussion

Granulomatosis with polyangiitis or Wegener’s granulomatosis though rare needs to be suspected whenever a small vasculitis disorder involving kidneys, lungs or upper respiratory tract tissues are involved. Rarely it can also involve musculoskeletal, skin, cardiac, neural, endocrine, salivary gland or orofacial tissues [3]. Constitutional symptoms in the form of weight loss, fever or malaise may also present. Precise etiology is unknown, it affects both genders equally. Typically, it involves adults, though younger age group and children may also be affected rarely [4]. Disorder may mask as infective diseases (endocarditis, syphilis, retroviral disease, tuberculosis or histoplasmosis), connective tissue disorders (rheumatoid arthritis, systemic lupus erythematosus with lupus nephritis or other small vessel vasculitides), other granulomatous disorder like sarcoidosis, glomerulonephritis (post streptococcal, anti GBM disease) or drug abuse such as cocaine. Wegener’s granulomatosis in view of non-pathognomonic features it usually masks as other above listed disorders [3]. High index of suspicion and prompt evaluation is indicated especially when the patient is not responding to initial therapy of a suspected disorder. Lung and kidney involvement carry a high mortality and early therapy is warranted. An interdisciplinary care approach is shown to improve outcomes [5].

References

  1. Almouhawis HA, Leao JC, Fedele S et al. Wegener’s granulomatosis: a review of clinical features and an update in diagnosis and treatment. J Oral Pathol Med. 2013;42(7):507-16.
  2. Lutalo PM, D’Cruz DP. Diagnosis and classification of granulomatosis with polyangiitis (aka Wegener’s granulomatosis). J Autoimmun. 2014;48:94-8.
  3. Tarabishy AB, Schulte M, Papaliodis GN, Hoffman GS. Wegener’s granulomatosis: clinical manifestations, differential diagnosis, and management of ocular and systemic disease. Survey Ophthalmol. 2010;55(5):429-44.
  4. Akikusa JD, Schneider R, Harvey EA et al. Clinical features and outcome of pediatric Wegener’s granulomatosis. Arthritis Rheum. 2007;57(5):837-44.
  5. Reinhold-Keller E, Beuge N, Latza U et al. An interdisciplinary approach to the care of patients with Wegener’s granulomatosis: long-term outcome in 155 patients. Arthritis Rheum. 2000;43(5):1021-32.

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