a 78-year old male with past medical history significant for chronic shortness of breath ( for the last two years ) , chronic systolic heart failure secondary to non - ischemic dilated cardiomyopathy , pulmonary hypertension , essential hypertension , coal miner s pneumoconiosis , and chronic obstructive lung disease was found to have bilateral pulmonary nodules on pre - operative chest x - ray . the work - up for the pulmonary nodules was initially planned on an outpatient basis . however , two weeks later the patient was admitted to the hospital with progressive shortness of breath . upon admission , vital signs included temperature 35.8c , pulse 56 beats / min , respiratory rate 22 breaths / min , and blood pressure 110/54 mmhg . on review of systems he reported decreased appetite for the past three months and a 20 pound weight loss . he denied hematuria , rhinorrhea , rashes , sinus pain , muscle aches , ear ache , hearing loss or hemoptysis . blood urea nitrogen was 18 mg / dl , creatinine 1.2 mg / dl , glomerular filtration rate 59 ml / min . chest x - ray revealed bilateral hilar and right upper lobe areas of infiltrate increased from the prior imaging examination . computed tomography ( ct ) scan revealed multiple cavitary lesions , the largest on the right , with a large air fluid level suspicious for infection . the day after admission the patient underwent ct guided fine needle aspiration of the right lung . all blood cultures were the negative and so were cultures of the pleural fluid and the surgical specimen . however , the antibiotics were soon discontinued secondary to low suspicion for the presence of active infection . at that point the diagnosis of vasculitis was entertained and a vasculitis panel was significant for elevated sedimentation rate at 84 mm / h . anti - glomerular basement membrane antibody level was negative , but proteinase 3-anti - neutrophil cytoplasmic antibody ( pr3-anca ) level was elevated at 3.8 units . stool studies were positive for clostridium difficile for which the patient was started on flagyl . repeat urinalysis showed brown colored urine with ph 6 , specific gravity 1.023 , moderate occult blood , bile negative , small leukocyte esterase , nitrate negative , 9 - 10 red blood cells ( rbc)/high power field ( hpf ) , 9 - 10 white blood cells / hpf , rare squamous epithelial cells , rare hyaline casts , 30a protein . kidney biopsy was inadequate for diagnosis . during the course of his treatment he also completed 5 cycles of plasmapheresis . wegener s granulomatosis ( wg ) is anca associated vasculitis of small and medium sized vessels . the disease has variable presentation and diagnosis is made on the basis of clinical , laboratory , pathologic and imaging studies . usually patients present with upper respiratory tract symptoms such as nasal ulcer , nasal discharge , rhinorrhea and sinus pain . kidney involvement manifests as acute renal failure with red cells , red cell casts and proteinuria . although wg is mainly characterized by respiratory disease and nephritis , it can also affect the nervous system , heart , eyes , skin , joints and spleen . the diagnostic criteria published by the american college of rheumatology include nasal or oral inflammation with ulcers or purulent bloody discharge ; chest radiograph showing nodules , cavities or infiltrates ; urinary sediment with red cell casts or microscopic hematuria ; granulomatous inflammation on lung biopsy . infection could serve as a trigger for disease expression and is an established factor for disease relapse . under the effect of unknown antigen , neutrophils express cytoplasmic pr3 , which leads to the production of anti - pr3 antibodies . laboratory studies include leukocytosis , elevated erythrocyte sedimentation rate , normocytic anemia , positive anca and rheumatoid factor . potential biopsy sites include affected areas such as nasal sinus , muscle , temporal artery , kidney , lung . kidney biopsies are done in patients with urinary sediment abnormalities , proteinuria or change in renal function . on histologic examination wg is characterized by vasculitis , inflammatory infiltration of medium and small vessels , and necrotizing granuloma formation . the limited form of wegener s usually does not affect the function of vital organs . the systemic form of wg is more common and only 25% of patients will have limited wg . some authors state that the limited form may evolve into systemic disease , implying that the two forms are different stages of progression of the same disease . they further state that the lack of progression in some of the cases is due to improved treatment modalities . c - anca is present in 50% of patients with the limited form and 80% of patients with the systemic form of the disease . initially our patient was found to have bilateral pulmonary nodules on preoperative chest x - ray . differential diagnosis at that time included more common conditions such as lung metastasis , tuberculosis , sarcoidosis , rheumatoid nodules , lung infection ( bacterial or fungal ) . the presence of vasculitic process was investigated after the above mentioned conditions were ruled out . the patient did not present with upper respiratory findings or findings indicative of kidney involvement . due to the absence of such findings he later developed diarrhea and was diagnosed with clostridium difficile infection . at that time the patient developed systemic manifestations of wg including renal failure , vasculitic rash and hemoptysis . the degree of kidney involvement could not be assessed secondary to failure to obtain satisfactory biopsy specimen . the infection could have triggered the evolvement of his limited wg into systemic disease , supporting the theory that those two forms are different stages of the same disease entity . the patient s condition rapidly transformed from a limited form to a systemic form of wg in the course of two weeks . this highlights the importance of timely treatment in patients with limited wg in order to prevent disease progression . when left untreated , wg is usually fatal . wg that does not involve the kidneys or other vital organs can be treated with less toxic regimens such as methotrexate or azathioprine . limited wg may progress to involve kidneys and other organs and hence should be treated and closely monitored . however , patients do not always present with the triad and a high index of clinical suspicion is needed in order to make a prompt diagnosis . when patients present with limited disease , without renal involvement , diagnosis is delayed and immunosuppressive treatment is not recommended . there are no clear guidelines on how limited wg should be approached , followed up , or treated . our case demonstrates that limited wg can evolve into systemic disease secondary to environmental triggers such as infection . this supports the notion that the two forms represent different stages of the same disease .
wegener s granulomatosis is a granulomatous vasculitis that can present with a wide spectrum of clinical manifestations . 
 this disease entity predominantly affects the respiratory tract and the kidneys . 
 two forms of wegener s granulomatosis have been recognized : systemic and limited . 
 it has not been established if the two forms represent separate disease entities or different stages of the same condition . in the limited form of wegener s granulomatosis 
 there is no immediate threat to the function of vital organs and there is no evidence of glomerulonephritis . 
 environmental factors that could serve as triggers for the activation of wegener s granulomatosis have not been clearly defined . 
 we report a case of a 78-year old male who was found to have bilateral pulmonary nodules on pre - operative chest x - ray and was diagnosed with the limited form of wegener s granulomatosis . 
 the patient developed clostridium difficile infection , and shortly after that active glomerulonephritis , a manifestation of systemic wegener s granulomatosis .