A patient with bronchiectasis develops nephrotic syndrome. The most probable diagnosis is?
ANSWER: (C) AmyloidosisREF: Interstitial Lung Disease by Marvin L Schwarz, Talmadge E. King Jr., SchwTarz, Talmadge E. King, Jr. M.D Page 1095, Harrison's 18th ed, Robbins 8th edIn addition to respiratory tract involvement, nephrotic syndrome is seen in 33% of patients with primary amyloidosis, myocardiopathy in 25%, neuropathy in 17%, and gastrointestinal involvement in 8% of cases. Furthermore, amyloidosis is a complication of long-standing bronchiectasis, which can also result in nephrotic syndrome.Bronchiectasis and nephritic syndrome are seen in following conditions:SLECystic fibrosis (Rare)Amyloidosis (amyloidosis is a complication of long-standing bronchiectasis, wThich can also result in nephrotic syndrome)ImmunodeficiencyOption (A): In connective tissue disorders like SLE, the systemic manifestations generally precede the pulmonary manifestations. Also Pleuritis with or without effusion is the most common pulmonary manifestationOption (B): Cystic fibrosis causes about half of all cases of bronchiectasis. Renal disease is not usually considered a major problem in CF. This is surprising since the cystic fibrosis transmembrane conductance regulator (CFTR) protein is expressed in all nephron segments of the kidney (Morales etal, 2000)Option (D): Most common pulmonary manifestation of HIV is pneumonia not bronchiectasis, however chronic HIV-associated lung disease including bronchiectasis. But nephropathy usually comes first as it is a direct manifestation of HIV virus itself
SLE
Cystic fibrosis
Amyloidosis
HIV
Pneumonia, Bronchiectasis, and Lung Abscess