American Thoracic Society 2012 San Franscisco
A Case Of Wegner's Granulomatosis With Pulmonary Tuberculosis: A Diagnostic Dilemma
A. Kansal, V. Chopra, A. Gupta, S. Kansal, S. Oberoi, G. Chawla, N. Bansal
Govt. Medical College and Hospital - Patiala/IN
INTRODUCTION
Wegner’s Granulomatosis is a disease of unknown etiology, which was described for the first time by Wegner in 1936. Wegner’s Granulomatosis usually present as a triad of airway necrotising granulomas, systemic vasculitis and focal necrotising granulomatosis. The lungs are affected in 90 percent of patients. In a country like India where tuberculosis is a very common disease, a case of Wegner’s granulomatosis with such presentation both clinically and radiologically is likely to be mistaken for tuberculosis which leads to delayed diagnosis and hence flaring up of the disease.
We report a unique case of dual pathology of pulmonary tuberculosis and wegner's granulomatosis in a 36 year old male in which the diagnosis of the later was delayed due to concurrent pulmonary tuberculosis.
CASE REPORT
A 36 year old male presented to us with one month history of fever and cough with blood streaked expectoration and worsening of chest xray while on Anti tubercular therapy (ATT) which was started on basis of bronchalveolar lavage (BAL) fluid positive for acid fast bacilli (AFB).
Patient also complained of rhinorrhoea, post nasal drip and joint pains. Physical examination and routine blood investigations were normal, Urine microscopy showed presence of 10-15 Rbc’s per HPF with traces of protein. RA factor was negative.
Figure 1 showing a cavity with air fluid level on left side and a small nodule on right side Figure 2 Section of CT chest showing a cavitatory lesion with thick irregular shaggy walls and air fluid level in left upper lobe and a well defined rounded nodule in posterior segment of right upper lobe
Pus was aspirated from the cavity under CT guidance, Cytology of pus showed necrotizing granulomatous pathology.
CT PNS suggested pansinusitis with thinning of nasal septum of bilateral middle and inferior turbinates.
Cosidering the above mentioned clinical, lab and radiological findings, possibility of wegner’s granulomatosis was made. Nasal biopsy was done and serum c-ANCA was sent.
Nasal biopsy revealed necrotizing granulomatous pathology and serum c-ANCA was strongly positive and hence conforming the diagnosis of wegner’s granulomatosis. Patient was started on cyclophosphamide and prednisolone along with ATT. He responded well and is on follow up.
DISCUSSION
Differential diagnosis of tuberculosis and Wegener's granulomatosis poses a great problem. In both cases, clinical and radiological findings may be identical. Even histopatologic finding can make confusion, since both diseases have granulomatous changes.In our case the diagnosis of Wegner's was delayed due to similar symptoms and radiological findings of wegner’s to pulmonary tuberculosis and also due to high incidence of tuberculosis in this part of world.
Wegner’s granulomatosis affects mainly the upper and lower respiratory tracts along with kidneys.
The disease responds well to immunosuppressive agents and steroids. Much literature is not available depicting the effect of other infectious disease like tuberculosis on the course of wegner’s granulomatosis.
Wegner’s Granulomatosis is a disease of unknown etiology, which was described for the first time by Wegner in 1936. Wegner’s Granulomatosis usually present as a triad of airway necrotising granulomas, systemic vasculitis and focal necrotising granulomatosis. The lungs are affected in 90 percent of patients. In a country like India where tuberculosis is a very common disease, a case of Wegner’s granulomatosis with such presentation both clinically and radiologically is likely to be mistaken for tuberculosis which leads to delayed diagnosis and hence flaring up of the disease.
We report a unique case of dual pathology of pulmonary tuberculosis and wegner's granulomatosis in a 36 year old male in which the diagnosis of the later was delayed due to concurrent pulmonary tuberculosis.
CASE REPORT
A 36 year old male presented to us with one month history of fever and cough with blood streaked expectoration and worsening of chest xray while on Anti tubercular therapy (ATT) which was started on basis of bronchalveolar lavage (BAL) fluid positive for acid fast bacilli (AFB).
Patient also complained of rhinorrhoea, post nasal drip and joint pains. Physical examination and routine blood investigations were normal, Urine microscopy showed presence of 10-15 Rbc’s per HPF with traces of protein. RA factor was negative.
Figure 1 showing a cavity with air fluid level on left side and a small nodule on right side Figure 2 Section of CT chest showing a cavitatory lesion with thick irregular shaggy walls and air fluid level in left upper lobe and a well defined rounded nodule in posterior segment of right upper lobe
Pus was aspirated from the cavity under CT guidance, Cytology of pus showed necrotizing granulomatous pathology.
CT PNS suggested pansinusitis with thinning of nasal septum of bilateral middle and inferior turbinates.
Cosidering the above mentioned clinical, lab and radiological findings, possibility of wegner’s granulomatosis was made. Nasal biopsy was done and serum c-ANCA was sent.
Nasal biopsy revealed necrotizing granulomatous pathology and serum c-ANCA was strongly positive and hence conforming the diagnosis of wegner’s granulomatosis. Patient was started on cyclophosphamide and prednisolone along with ATT. He responded well and is on follow up.
DISCUSSION
Differential diagnosis of tuberculosis and Wegener's granulomatosis poses a great problem. In both cases, clinical and radiological findings may be identical. Even histopatologic finding can make confusion, since both diseases have granulomatous changes.In our case the diagnosis of Wegner's was delayed due to similar symptoms and radiological findings of wegner’s to pulmonary tuberculosis and also due to high incidence of tuberculosis in this part of world.
Wegner’s granulomatosis affects mainly the upper and lower respiratory tracts along with kidneys.
The disease responds well to immunosuppressive agents and steroids. Much literature is not available depicting the effect of other infectious disease like tuberculosis on the course of wegner’s granulomatosis.