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P31 Fungal infections in rheumatic diseases: experience with 4 patients
Abstracts of papers presented in IRACON-2010 P29 Gastrointestinal involvement in systemic sclerosis—a cross sectional study in Indian population
Poster presentations
S21
Conclusion: IFI in immunocompromised patients has high morbidity and mortality. The diagnosis requires a high degree of suspicion and treatment includes appropriate antifungal therapy and surgical intervention when necessary.
Rahul Chaudhary, Uma Kumar, A Saraya, S Sharma, A Seith, Sreenivas Department of Medicine, AIIMS, Delhi Background: Gastrointestinal involvement (GI) is a serious complication of Systemic Sclerosis (SSc). Methods: We carried out a cross sectional study in 35 patients (32 males and 3 females) of SSc to find out the prevalence of GI and hepatobilliary involvement and to determine the predictors of GI involvement. A detailed history and physical examination was recorded on a preformed Performa. All patients had undergone Barium swallow, Upper GI endoscopy and H2 Breath test. Those with symptoms of small bowel involvement underwent D-Xylose test and Barium meal follow through while those with symptoms of large bowel involvement underwent colonoscopy and Barium enema. Results: GI involvement was observed in 80% of the patients. Upper GI involvement was found in 74.3% patients, small bowel involvement in 37.1% while 5.7% of the patients had hepatobilliary involvement in the form of biopsy proven autoimmune hepatitis. Dysphagia was the commonest symptoms and 69.7% of the patients had an abnormal Barium swallow. The median age of onset and duration of disease was 25 years and 8 years respectively. 74.29% of the patients had limited cutaneous involvement while 25.71% had diffuse cutaneous SSc. 35.3% of the patients had a positive H2 Breath test suggesting small intestinal bacterial overgrowth (SIBO). GI involvement was more common in patients with limited SSc. But there was no significant correlation of GI involvement with the age of onset, duration of disease and limited versus diffuse SSc. Conclusion: GI involvement is common in patients with SSc, with upper GI symptoms being more common than lower GI symptoms. GI involvement is more common in patients with limited SSc. A significant number of these patients have evidence of SIBO which may account for malabsorption in these patients.
P30 Plasma dyscrasias and inflammatory arthritis: not so uncommon association Vishnu Sharma, S Nagaraj, Rohini Samant Department of Rheumatology, PD Hinduja National Hospital and MRC, Mumbai Background: Plasma dyscrasias can occasionally manifest with joint disease. There are few case reports describing articular manifestations of plasma dyscrasias. In most of these cases plasma dyscrasias were diagnosed prior to the articular symptoms. We report six cases of inflammatory arthritis which presented to us with articular symptoms and were later diagnosed as plasma dyscrasias. Material & Methods: Retrospective analysis of the case-files of patients with inflammatory arthritis and plasma dyscrasias seen in the past year was done. 6 patients were identified. The demographic data of the patients and the modality of treatment were recorded. The diagnosis of monoclonal gammapathy was based on protein electrophoresis, immunoelectrophoresis and bone marrow biopsy. The outcomes of the treatment were analyzed. Results: Four patients had MGUS and two patients had multiple myeloma. Mean age of the patients was 64.16 years (59–73). Three patients presented with oligoarticular arthritis, two with symmetrical polyarticular joint pains and one with fleeting soft tissue pain. Wrist and shoulder were the most common involved joints. Three patients had carpal tunnel syndrome. Mean ESR was 89.83 mm/hr (67–120). Arthritis improved with chemotherapy in patients with multiple myeloma. Conclusion: Occurrence of inflammatory arthritis with plasma dyscrasias is more than a chance association. A plasma dyscrasia should be searched for in elderly patients presenting with atypical arthritis with disproportionatly high ESR, high creatinine and hyperglobulinemia.
P31 Fungal infections in rheumatic diseases: experience with 4 patients S Nagaraj, V Sharma, C Balakrishnan, R Soman PD Hinduja National Hospital and MRC, Veer Savarkar Marg, Mahim, Mumbai Background: Invasive fungal infections are uncommon, but cause significant problems when encountered in the setting of rheumatic diseases. We present 4 such patients and review the relevant literature. Methods: Four patients with rheumatolgoical disorders and invasive fungal infections (IFI) were retrospectively studied. The demographic data of patients were recorded. Clinical and laboratory data, including fungal species, site/s of infection, corticosteroid and other immunosuppressant medications used, treatment given were noted and analyzed. Results: Three patients had ANCA-positive vasculitis (2 patients with Wegener’s granulomatosis and one with Microscopic polyangiitis) and one patient had SLE. Except for one patient with Wegener’s granulomatosis (WG) all were females. The age of the patients ranged from 14 to 54 years (average 34.3 years). All patients had infection with Aspergillus fumigatus. One patient (with WG) had mixed infection with A. fumigatus and mucor spp. Major organs involved were respiratory tract in two patients (both with WG), heart in one patient (patient with SLE) and fronto-parietal region of brain with sphenoid sinus in another (patient with MPA). All patients received appropriate antifungal therapy and supportive care. Two patients needed surgical intervention. One patient expired and 2 of the 3 patients who survived had severe neurological deficits.
P32 Subcutaneus zygomycosis masquerading as scleroderma like illness: a rare case report Jyoti Ranjan Parida, Ravi Mallick, Bidyut K Das Division Of Rheumatology, Department Of Medicine, S. C. B. Medical College, Cuttack Background: Infection mimicks rheumatological problems. This is a rare case report of subcutaneous zygomycosis masquerading as scleroderma like illness. Methods: A 7 yr old male child diagnosed as subcutaneous zygomycosis on basis of clinical features, histopathological study & treatment response and followed up for a period of 2 years. Observations: A 7 yr male child from rural area of orissa presented with gradual onset progressive tightening of skin involving the lower extremities and trunk. On examination, skin was thick and firmly adherent to underlying subcutaneous tissue with brawny nonpitting subcutaneous edema over both legs, gluteal region and lower trunk. Histopathological study from skin biopsy showed multiple non caeseasting granulomas with splendore-hoeppli phenomenon and few fungal hyphae in eosinophilic background. A diagnosis of subcutaneous zygomycosis made. The patient received oral potassium iodide and had a complete clinical improvement within 2 months of treatment. The patient followed up in OPD basis for next 2 years and did not show any relapse of symptoms. Results: A 7 year child who presented with progressive skin tightening and swelling (mimicking scleroderma like illness), diagnosed as subcutaneous zygomycosis and successfully treated with oral potassium iodide. Conclusions: Subcutaneous zygomycosis is a treatable condition that can mimick scleroderma like illness. It should be included in the differential diagnosis of scleroderma like illness, particularly in a tropical country like India.
P33 Type 2 lepra reaction as initial presentation of Hansen’s disease and mimicking rheumatoid arthritis Praveen Kumar Department of Medicine, Trivandrum Medical College, Trivandrum Introduction: Hansen’s disease is at the verge of elimination in India. However cases are still reported from various parts in our country. Type 2 Lepra reaction is an immune mediated type III hypersensitivity reaction (immune complex) which can mimic autoimmune diseases with even antibody positivity like RA factor in high titre. If it is the presenting manifestation it can exactly mimic any autoimmune diseases and can pose great diagnostic dilemma. Here we present a patient with history suggestive of an autoimmune disease (Rheumatoid Arthritis) which turned out to be Hansen’s disease- LL type in type 2 lepra reaction. Case Report: A 58 year old gentleman presented with multiple painful nodules all over his body predominantly over trunk and base with reddish discoloration around the lesions associated with low grade intermittent fever. The lesions were evanescent and arising in crops lasting for 24–48 hrs. He also had asymmetric inflammatory polyarthritis involving both large and small joints with significant morning stiffness for past 3 months. He had 1 episode of inflammatory polyarthritis 1 year back which lasted for 1 month with raised ESR and positive RA factor and was on NSAIDS for few months, thereafter became asymptomatic. This time he was having RAfactor positivity in high titrre (128 U/l) and raised ESR. AntiCitrullinated peptide antibody was marginally elevated. ANA was borderline positive with normal ANA profile. The skin lesion was diagnosed as erythema nodosum and investigations to rule out all causes of erythema nodosum were carried out. As these lesions were on atypical sites Hansen’s disease was considered and a slit skin smear was performed. Surprisingly we could demonstrate acid fast bacilli suggestive of M. leprae with bacteriological index of 4 + (globi). There were no other clinical features of Hansen’s disease like hypopigmented anesthetic skin patches, thickened peripheral nerves etc. Biopsy of the lesions showed lepra bacilli with no evidence of septal panniculitis. Thus a diagnosis of Hansen’s disease with Lepra reaction Type2 was made. He was put on MDT therapy with steroids to tackle the reaction. His nodules slowly subsided and became asymptomatic. Steriod was withdrawn. He is still continuing the MDT regimen for Lepromatous Leprosy. Conclusion: Hansen’s disease and its reactions can be great mimickers and should be kept in mind even in this era. In patients with Constitutional symptoms, arthritis, atypical skin lesions with Positive autoimmune markers Lepra reaction should be kept in the list of differential diagnosis.
P34 Withdrawn P35 Long-term continuous use of biologic agents for axial spondyloarthritis in Indian patients Ramesh Jois Rheumatologist, Wockhardt Hospitals, Bangaluru
Poster presentations
S21
Conclusion: IFI in immunocompromised patients has high morbidity and mortality. The diagnosis requires a high degree of suspicion and treatment includes appropriate antifungal therapy and surgical intervention when necessary.
Rahul Chaudhary, Uma Kumar, A Saraya, S Sharma, A Seith, Sreenivas Department of Medicine, AIIMS, Delhi Background: Gastrointestinal involvement (GI) is a serious complication of Systemic Sclerosis (SSc). Methods: We carried out a cross sectional study in 35 patients (32 males and 3 females) of SSc to find out the prevalence of GI and hepatobilliary involvement and to determine the predictors of GI involvement. A detailed history and physical examination was recorded on a preformed Performa. All patients had undergone Barium swallow, Upper GI endoscopy and H2 Breath test. Those with symptoms of small bowel involvement underwent D-Xylose test and Barium meal follow through while those with symptoms of large bowel involvement underwent colonoscopy and Barium enema. Results: GI involvement was observed in 80% of the patients. Upper GI involvement was found in 74.3% patients, small bowel involvement in 37.1% while 5.7% of the patients had hepatobilliary involvement in the form of biopsy proven autoimmune hepatitis. Dysphagia was the commonest symptoms and 69.7% of the patients had an abnormal Barium swallow. The median age of onset and duration of disease was 25 years and 8 years respectively. 74.29% of the patients had limited cutaneous involvement while 25.71% had diffuse cutaneous SSc. 35.3% of the patients had a positive H2 Breath test suggesting small intestinal bacterial overgrowth (SIBO). GI involvement was more common in patients with limited SSc. But there was no significant correlation of GI involvement with the age of onset, duration of disease and limited versus diffuse SSc. Conclusion: GI involvement is common in patients with SSc, with upper GI symptoms being more common than lower GI symptoms. GI involvement is more common in patients with limited SSc. A significant number of these patients have evidence of SIBO which may account for malabsorption in these patients.
P30 Plasma dyscrasias and inflammatory arthritis: not so uncommon association Vishnu Sharma, S Nagaraj, Rohini Samant Department of Rheumatology, PD Hinduja National Hospital and MRC, Mumbai Background: Plasma dyscrasias can occasionally manifest with joint disease. There are few case reports describing articular manifestations of plasma dyscrasias. In most of these cases plasma dyscrasias were diagnosed prior to the articular symptoms. We report six cases of inflammatory arthritis which presented to us with articular symptoms and were later diagnosed as plasma dyscrasias. Material & Methods: Retrospective analysis of the case-files of patients with inflammatory arthritis and plasma dyscrasias seen in the past year was done. 6 patients were identified. The demographic data of the patients and the modality of treatment were recorded. The diagnosis of monoclonal gammapathy was based on protein electrophoresis, immunoelectrophoresis and bone marrow biopsy. The outcomes of the treatment were analyzed. Results: Four patients had MGUS and two patients had multiple myeloma. Mean age of the patients was 64.16 years (59–73). Three patients presented with oligoarticular arthritis, two with symmetrical polyarticular joint pains and one with fleeting soft tissue pain. Wrist and shoulder were the most common involved joints. Three patients had carpal tunnel syndrome. Mean ESR was 89.83 mm/hr (67–120). Arthritis improved with chemotherapy in patients with multiple myeloma. Conclusion: Occurrence of inflammatory arthritis with plasma dyscrasias is more than a chance association. A plasma dyscrasia should be searched for in elderly patients presenting with atypical arthritis with disproportionatly high ESR, high creatinine and hyperglobulinemia.
P31 Fungal infections in rheumatic diseases: experience with 4 patients S Nagaraj, V Sharma, C Balakrishnan, R Soman PD Hinduja National Hospital and MRC, Veer Savarkar Marg, Mahim, Mumbai Background: Invasive fungal infections are uncommon, but cause significant problems when encountered in the setting of rheumatic diseases. We present 4 such patients and review the relevant literature. Methods: Four patients with rheumatolgoical disorders and invasive fungal infections (IFI) were retrospectively studied. The demographic data of patients were recorded. Clinical and laboratory data, including fungal species, site/s of infection, corticosteroid and other immunosuppressant medications used, treatment given were noted and analyzed. Results: Three patients had ANCA-positive vasculitis (2 patients with Wegener’s granulomatosis and one with Microscopic polyangiitis) and one patient had SLE. Except for one patient with Wegener’s granulomatosis (WG) all were females. The age of the patients ranged from 14 to 54 years (average 34.3 years). All patients had infection with Aspergillus fumigatus. One patient (with WG) had mixed infection with A. fumigatus and mucor spp. Major organs involved were respiratory tract in two patients (both with WG), heart in one patient (patient with SLE) and fronto-parietal region of brain with sphenoid sinus in another (patient with MPA). All patients received appropriate antifungal therapy and supportive care. Two patients needed surgical intervention. One patient expired and 2 of the 3 patients who survived had severe neurological deficits.
P32 Subcutaneus zygomycosis masquerading as scleroderma like illness: a rare case report Jyoti Ranjan Parida, Ravi Mallick, Bidyut K Das Division Of Rheumatology, Department Of Medicine, S. C. B. Medical College, Cuttack Background: Infection mimicks rheumatological problems. This is a rare case report of subcutaneous zygomycosis masquerading as scleroderma like illness. Methods: A 7 yr old male child diagnosed as subcutaneous zygomycosis on basis of clinical features, histopathological study & treatment response and followed up for a period of 2 years. Observations: A 7 yr male child from rural area of orissa presented with gradual onset progressive tightening of skin involving the lower extremities and trunk. On examination, skin was thick and firmly adherent to underlying subcutaneous tissue with brawny nonpitting subcutaneous edema over both legs, gluteal region and lower trunk. Histopathological study from skin biopsy showed multiple non caeseasting granulomas with splendore-hoeppli phenomenon and few fungal hyphae in eosinophilic background. A diagnosis of subcutaneous zygomycosis made. The patient received oral potassium iodide and had a complete clinical improvement within 2 months of treatment. The patient followed up in OPD basis for next 2 years and did not show any relapse of symptoms. Results: A 7 year child who presented with progressive skin tightening and swelling (mimicking scleroderma like illness), diagnosed as subcutaneous zygomycosis and successfully treated with oral potassium iodide. Conclusions: Subcutaneous zygomycosis is a treatable condition that can mimick scleroderma like illness. It should be included in the differential diagnosis of scleroderma like illness, particularly in a tropical country like India.
P33 Type 2 lepra reaction as initial presentation of Hansen’s disease and mimicking rheumatoid arthritis Praveen Kumar Department of Medicine, Trivandrum Medical College, Trivandrum Introduction: Hansen’s disease is at the verge of elimination in India. However cases are still reported from various parts in our country. Type 2 Lepra reaction is an immune mediated type III hypersensitivity reaction (immune complex) which can mimic autoimmune diseases with even antibody positivity like RA factor in high titre. If it is the presenting manifestation it can exactly mimic any autoimmune diseases and can pose great diagnostic dilemma. Here we present a patient with history suggestive of an autoimmune disease (Rheumatoid Arthritis) which turned out to be Hansen’s disease- LL type in type 2 lepra reaction. Case Report: A 58 year old gentleman presented with multiple painful nodules all over his body predominantly over trunk and base with reddish discoloration around the lesions associated with low grade intermittent fever. The lesions were evanescent and arising in crops lasting for 24–48 hrs. He also had asymmetric inflammatory polyarthritis involving both large and small joints with significant morning stiffness for past 3 months. He had 1 episode of inflammatory polyarthritis 1 year back which lasted for 1 month with raised ESR and positive RA factor and was on NSAIDS for few months, thereafter became asymptomatic. This time he was having RAfactor positivity in high titrre (128 U/l) and raised ESR. AntiCitrullinated peptide antibody was marginally elevated. ANA was borderline positive with normal ANA profile. The skin lesion was diagnosed as erythema nodosum and investigations to rule out all causes of erythema nodosum were carried out. As these lesions were on atypical sites Hansen’s disease was considered and a slit skin smear was performed. Surprisingly we could demonstrate acid fast bacilli suggestive of M. leprae with bacteriological index of 4 + (globi). There were no other clinical features of Hansen’s disease like hypopigmented anesthetic skin patches, thickened peripheral nerves etc. Biopsy of the lesions showed lepra bacilli with no evidence of septal panniculitis. Thus a diagnosis of Hansen’s disease with Lepra reaction Type2 was made. He was put on MDT therapy with steroids to tackle the reaction. His nodules slowly subsided and became asymptomatic. Steriod was withdrawn. He is still continuing the MDT regimen for Lepromatous Leprosy. Conclusion: Hansen’s disease and its reactions can be great mimickers and should be kept in mind even in this era. In patients with Constitutional symptoms, arthritis, atypical skin lesions with Positive autoimmune markers Lepra reaction should be kept in the list of differential diagnosis.
P34 Withdrawn P35 Long-term continuous use of biologic agents for axial spondyloarthritis in Indian patients Ramesh Jois Rheumatologist, Wockhardt Hospitals, Bangaluru