418. Fungal infections in Rheumatic diseases-Infection vs flare?- the dangerous mimics!
Creators: name: Sahana Baliga1
Publisher: name: Zenodo
Year: 2022
Subjects: Vasculitis
ANCA
Abstracts
Giant Cell Arthritis
MPA
GPA
EGPA
Takayasu
IgA vasculitis

description: <strong>Case 1:</strong> 30 years old lady developed fever, progressive dyspnoea and painful ulcers Her investigatory report were Hb 10.9 mg/dl, WBC count -10500 platelets- 4.51 lakh cells/cu mm, ESR:112mm/hr, P ANCA: positive, urinalysis: haematuria and proteinuria and 24 hour urine protein: 800 mg. HRCT showed pulmonary infiltrates. Skin biopsy was suggestive of necrotizing vasculitis. A diagnosis of ANCA vasculitis was made and she was initiated on steroids and cyclophosphamide. Patient was steroid dependent for next 9 years. After 9 years into the disease, she developed left sided headache. Over the next 6 months, she developed acute diplopia and mild weakness of the right arm. MRI brain was done which showed right eye pseudo tumour. Repeat ANCA was positive and ESR was 60mm/hr. She was diagnosed as right eye Inflammatory pseudotumor and the dose of steroids was increased. Within a week she was admitted elsewhere with complete right sided hemiplegia with aphasia. With optimal therapy she regained speech but she complained of severe left retro-orbital pain. A diagnosis of vasculitis flare was made and she was treated with pulse methylprednisolone and cyclophosphamide. On examination, weakness status was the same but pain in the left eye persisted. Repeat MRI brain confirmed a soft tissue mass in the left sphenoid sinus with a defect in the lateral wall. Trans-sphenoidal debridement was done and, microbiological examination and histopathology of the debrided tissue confirmed Aspergillus Fumigatus. She improved with voriconazole and repeated trans-sphenoidal debridements <strong>Case 2</strong>: 14 years old girl, developed high grade fever (103 degree F) with maculopapular rashes on bilateral upper and lower limbs, painless redness of both eye with no visual impairment, rhinorrhoea with mild epistaxis, intermittent severe abdominal pain, polyarthritis of small joints of right hand , left knee and ankle, dry cough and bluish discolouration of toes all of 2 months duration. She also had unintentional weight loss of 2 kgs. Lab investigations revealed Hb 11 gm%, WBC count 13800cells/cumm, platelets 3.29lakh/cumm, ESR 116 mm, Creatinine 0.8mg/dl. Urine had active sediments and the urine spot protein /creatinine ratio was 0.5. (Up to 0.2). On further evaluation C ANCA was positive. CT thorax showed nodular lesions in the right middle and lower lobe with cavitary lesion in the right posterior lower lobe (Figure 1). CT Paranasal sinus showed bilateral frontal and maxillary sinusitis. Arterial doppler of bilateral lower limb showed occlusion of left distal anterior tibial artery and dorsalis pedis artery on both sides. She underwent biopsy from the nasal cartilage which showed chronic granulomatous inflammation consistent with Granulomatosis Polyangitis. She was initially treated with pulse methylprednisolone and fortnightly intravenous pulse cyclophosphamide therapy and later maintained on azathioprine.After 7 months of therapy, she developed productive cough and dyspnoea with hoarseness of voice. Direct laryngoscopy showed nodular lesions in the vocal cords with erythema. HRCT chest showed right middle lobe consolidation and multiple variable sized nodules at right upper and lower lobe. Blood culture was sterile. Bronchoalveolar fluid showed gram positive cocci with KOH mount showing branched septate mycelial filaments. Ziehl Nielson stain was negative. BAL culture grew Aspergillus species. Serum aspergillus galactomannan was negative. <strong>Discussion:</strong> Here we present 2 cases of ANCA vasculitis who were initially treated with immunosuppressive therapy but later developed fungal infections. Fungal infections closely mimic vasculitis. They tend to occur in resistant vasculitis increasing morbidity and mortality. These situations require detailed history and appropriate investigations especially tissue diagnosis. In conclusion in patients with ANCA vasculitis who are adequately immunosuppressed, if there is a purported flare of the disease, care should be taken to rule out infections, especially fungal infections. <strong>Disclosures</strong>: None <strong>Case 1:</strong> 30 years old lady developed fever, progressive dyspnoea and painful ulcers Her investigatory report were Hb 10.9 mg/dl, WBC count -10500 platelets- 4.51 lakh cells/cu mm, ESR:112mm/hr, P ANCA: positive, urinalysis: haematuria and proteinuria and 24 hour urine protein: 800 mg. HRCT showed pulmonary infiltrates. Skin biopsy was suggestive of necrotizing vasculitis. A diagnosis of ANCA vasculitis was made and she was initiated on steroids and cyclophosphamide. Patient was steroid dependent for next 9 years. After 9 years into the disease, she developed left sided headache. Over the next 6 months, she developed acute diplopia and mild weakness of the right arm. MRI brain was done which showed right eye pseudo tumour. Repeat ANCA was positive and ESR was 60mm/hr. She was diagnosed as right eye Inflammatory pseudotumor and the dose of steroids was increased. Within a week she was admitted elsewhere with complete right sided hemiplegia with aphasia. With optimal therapy she regained speech but she complained of severe left retro-orbital pain. A diagnosis of vasculitis flare was made and she was treated with pulse methylprednisolone and cyclophosphamide. On examination, weakness status was the same but pain in the left eye persisted. Repeat MRI brain confirmed a soft tissue mass in the left sphenoid sinus with a defect in the lateral wall. Trans-sphenoidal debridement was done and, microbiological examination and histopathology of the debrided tissue confirmed Aspergillus Fumigatus. She improved with voriconazole and repeated trans-sphenoidal debridements <strong>Case 2</strong>: 14 years old girl, developed high grade fever (103 degree F) with maculopapular rashes on bilateral upper and lower limbs, painless redness of both eye with no visual impairment, rhinorrhoea with mild epistaxis, intermittent severe abdominal pain, polyarthritis of small joints of right hand , left knee and ankle, dry cough and bluish discolouration of toes all of 2 months duration. She also had unintentional weight loss of 2 kgs. Lab investigations revealed Hb 11 gm%, WBC count 13800cells/cumm, platelets 3.29lakh/cumm, ESR 116 mm, Creatinine 0.8mg/dl. Urine had active sediments and the urine spot protein /creatinine ratio was 0.5. (Up to 0.2). On further evaluation C ANCA was positive. CT thorax showed nodular lesions in the right middle and lower lobe with cavitary lesion in the right posterior lower lobe (Figure 1). CT Paranasal sinus showed bilateral frontal and maxillary sinusitis. Arterial doppler of bilateral lower limb showed occlusion of left distal anterior tibial artery and dorsalis pedis artery on both sides. She underwent biopsy from the nasal cartilage which showed chronic granulomatous inflammation consistent with Granulomatosis Polyangitis. She was initially treated with pulse methylprednisolone and fortnightly intravenous pulse cyclophosphamide therapy and later maintained on azathioprine.After 7 months of therapy, she developed productive cough and dyspnoea with hoarseness of voice. Direct laryngoscopy showed nodular lesions in the vocal cords with erythema. HRCT chest showed right middle lobe consolidation and multiple variable sized nodules at right upper and lower lobe. Blood culture was sterile. Bronchoalveolar fluid showed gram positive cocci with KOH mount showing branched septate mycelial filaments. Ziehl Nielson stain was negative. BAL culture grew Aspergillus species. Serum aspergillus galactomannan was negative. <strong>Discussion:</strong> Here we present 2 cases of ANCA vasculitis who were initially treated with immunosuppressive therapy but later developed fungal infections. Fungal infections closely mimic vasculitis. They tend to occur in resistant vasculitis increasing morbidity and mortality. These situations require detailed history and appropriate investigations especially tissue diagnosis. In conclusion in patients with ANCA vasculitis who are adequately immunosuppressed, if there is a purported flare of the disease, care should be taken to rule out infections, especially fungal infections. <strong>Disclosures</strong>: None