Introduction:
This case report underscores the significance of maintaining a broad list of differential diagnosis, including secondary adrenal insufficiency when evaluating a patient who present with hypotension in AA amyloidosis.
Methods:
This is a prospective case of AA amyloidosis in young female who presents with nephrotic syndrome for 6 months. On clinical evaluation she had pedal edema. She had nephrotic range proteinuria (24-hour protein was 11.2 gm/day), her serum albumin was 1.2gm/dl and her serum creatinine was 1.3 mg/dl. Her immunological marker C3/C4 were within normal range. Her ANA and other autoimmune panel were negative. Her viral markers were negative. Her renal size on ultrasound were enlarge i.e. 11 cm. She underwent renal biopsy. Microscopy revealed deposition of amyloid. Immunofluorescence reveal negative deposits. Stain (IHC) for SAA is positive along areas of amyloid deposition.
With secondary amyloidosis as a diagnosis, panel of investigations were done. She had no joint pain and no early morning stiffness. Only positive history she had was of low to high grade fever for 2 years usually at night with on and off sweating. She used to take antipyretics and sometimes antibiotics but never got investigated for same. Her Digital X-ray chest PA s/o blunting of cardiopherenic angle and hilar prominence. Her routine ultrasound abdomen suggestive of ascites and few retroperitoneal lymph node. Her blood culture, urine culture were normal. She had normocytic normochromic anemia. Her ESR was raised to 50 at 1 hour (after correction). CECT Abdomen and Chest was done for PUO which revealed mild circumferential bowel wall thickening of ascending colon, hepatic flexure, transverse colon and distal ileum with mild mucosal fold thickening with evidence of stranding of adjacent fat planes with moderate to gross ascites with mesenteric and retroperitoneal lymph node. With this gastroenterologist opinion was taken and advice for tuberculin test, as India is a high burden country for tuberculosis. Her tuberculin test was strongly positive (>20mm in 48 hours). She was denied for colonoscopy as she has amyloidosis and risk of bleeding would be more. With this her empirical treatment for tuberculosis was started after dosage modification.
Results:
The challenge which author faced during her treatment was her substantially low blood pressure. She had persistent vomiting and fatigue. She underwent 2D echocardiography which was perfectly normal. For autonomic neuropathy, she had no fall in blood pressure in lying and after standing for 3 min. With this we started her on midodrine tablets at higher doses, despite this her blood pressure did not pick up. Her CECT abdomen had commented normal adrenals. Her morning serum cortisol came out to be low normal. Endocrinology opinion was taken and advice for ACTH stimulation test. After injecting ACTH 250microgram, her serum cortisol was still at lower side of normal hence she was started on tab prednisone 7.5mg daily in divided doses. Her blood pressure responded very well within one week of treatment. Her fatigue and vomiting episode recovered. After 1 month of antitubercular medicine her investigations improved. Her repeat ultrasound revealed no mesenteric and retroperitoneal lymph node.
Conclusions:
Secondary adrenal insufficiency typically characterized by weight loss, fatigue and recurrent vomiting. Restrictive cardiomyopathy and autonomic neuropathy are not always the cause of hypotension in secondary amyloidosis. Tuberculosis can involve adrenal gland in less than 5 % of cases. Tuberculosis leading to secondary amyloidosis is rare. Having two rare combination is rarest of rare. Hence keeping a broad list of differential diagnosis, including adrenal gland insufficiency when evaluating a patient who present with hypotension in AA amyloidosis is a must.
I have no potential conflict of interest to disclose.
I did not use generative AI and AI-assisted technologies in the writing process.