A case of membranous nephropathy associated with Sjögren syndrome, polymyositis and autoimmune hepatitis

Stefanidis, Ioannis; Giannopoulou, Myrto; Liakopoulos, V.; Dovas, Spiros; Karasavvidou, F; Zachou, Kalliopi; Koukoulis, George Κ.; Dalekos, George Ν.

doi:10.5414/cnp70245

Cited by 30 publications

(22 citation statements)

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“…Among the two types of AIH, type 1 is more often associated with other autoimmune diseases, but only exceptionally with inflammatory myopathies. Indeed, among the 11 reported cases of autoimmune hepatitis associated with inflammatory myositis, AIH type 1 was more common, patients exhibiting more often ASMA and ANA antibodies [10][11][12][13][14][15][16][17][18][19] (Table I). Moreover, among inflammatory myopathies, PM is more commonly associated with AIH [17].…”

Section: Discussionmentioning

confidence: 99%

Primary Biliary Cirrhosis – Autoimmune Hepatitis Overlap Syndrome Associated with Dermatomyositis, Autoimmune Thyroiditis and Antiphospholipid Syndrome

Pamfil

Candrea

Berki³

et al. 2015

JGLD

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Autoimmune liver diseases may be associated with extrahepatic autoimmune pathology. We report the case of a 52-year old woman who initially presented to the gastroenterology department for extreme fatigue, pale stools, dark urine and pruritus. Laboratory tests showed significant cholestasis and elevation of aminotransferase levels. Immunological tests revealed positive antinuclear (ANA=1:320) and antimitochondrial antibodies (AMA=1:40) with negative anti-smooth muscle and liver kidney microsomal type 1 antibodies. The biopsy was compatible with overlap syndrome type 1. The patient was commenced on immunosuppressive therapy according to standard of care (azathioprine 50mg, ursodeoxycholic acid and prednisone 0.5mg/kg), with moderate biochemical improvement. She subsequently developed proximal symmetrical weakness and cutaneous involvement and was diagnosed with biopsy-proven dermatomyositis. The immunosuppressive regimen was intensified to 150 mg azathioprine. At the three-month follow-up, her symptoms subsided and aminotransferases and muscle enzymes normalized. Upon further investigation the patient was diagnosed with autoimmune thyroiditis and antiphospholipid syndrome. To our knowledge, this is the first case of primary biliary cirrhosis - autoimmune hepatitis overlap syndrome associated with dermatomyositis, autoimmune thyroiditis and antiphospholipid syndrome.

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Section: Discussionmentioning

confidence: 99%

Primary Biliary Cirrhosis – Autoimmune Hepatitis Overlap Syndrome Associated with Dermatomyositis, Autoimmune Thyroiditis and Antiphospholipid Syndrome

Pamfil

Candrea

Berki³

et al. 2015

JGLD

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“…Polymyositis is a rare and gradually progressive autoimmune disease of skeletal muscle 1. The two main renal manifestations of polymyositis are known as acute kidney injury (AKI) secondary to rhabdomyolysis2,4,5 and polymyositis-associated glomerulonephritis 2,6–8. However, overflow proteinuria with rhabdomyolysis has been rarely described.…”

Section: Discussionmentioning

confidence: 99%

“…Rhabdomyolysis rarely develops in patients with polymyositis,10 and about 6% of patients have CPK levels higher than 3,000 IU/L,11 as was found in the present case. In patients with polymyositis, proteinuria is related to various types of glomerulonephritis,2,6–8 or myoglobinuria 2. As the patient had hypoalbuminemia and a moderate amount of proteinuria, we initially expected the proteinuria was of a glomerular origin.…”

Section: Discussionmentioning

confidence: 99%

Overflow proteinuria as a manifestation of unrecognized polymyositis

Kim¹,

Kim²,

Kim³

et al. 2014

IMCRJ

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Polymyositis is a rare and gradually progressive autoimmune disease of skeletal muscle. Two main types of renal involvement have been described: acute tubular necrosis related to rhabdomyolysis and glomerulonephritis. However, cases of overflow proteinuria related to polymyositis have rarely been reported. Herein, we report a case of a 41-year-old male who presented with edema of both lower extremities. Laboratory studies revealed elevated creatine phosphokinase level, hypoalbuminemia, and a moderate amount of proteinuria, although albuminuria was not dominant. Urine electrophoresis showed an abnormally restricted zone in the β-fraction, which suggested overflow proteinuria of non-glomerular origin. Despite intravenous hydration, his serum creatine phosphokinase level did not decrease and his symptoms did not improve. Electromyography showed myopathy, and muscle biopsy revealed findings consistent with polymyositis. After corticosteroid therapy, his creatine phosphokinase level and proteinuria decreased and his clinical symptoms improved. This case demonstrates an atypical presentation of polymyositis manifested by overflow proteinuria.

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“…AIH has frequently been reported to associate with collagen vascular diseases (CVD) such as rheumatoid arthritis and Sjögren’s syndrome 1. However, coincidences of AIH and PM/DM are rarely reported 2–5. Here, the authors report a rare case of coincidence of adult PM and AIH simultaneously.…”

Section: Introductionmentioning

confidence: 86%