<prompt> Ormonds disease is -Idiopathic lymphadenopathyRetractile testisIdiopathic retroperitonial fibrosisIdiopathic mediastinitis  <response>: Uncommon inflammatory condition characterized by the proliferation of fibrous tissue in the retroperitoneum The fibrosis is usually confined to the central and paravertebral spaces between the renal arteries and sacrum and tends to encase the aorta, IVC and ureters. The process usually begins at the level of the aortic bifurcation and spreads cephalad upto renal artery generally.Etiology Around 70 cases are primary or idiopathic Ormonds diseaseCauses of Secondary 30Q Retroperitoneal Fibrosis Inflammatory conditions: CATH Chronic pancreatitis, Actinomycosis, Tuberculosis, Histoplasmosis Drugs: Methylsergide Most important, methyldopa, hydralazine, entacapone, beta-blockers, bromocriptine, phenacetin, amphetamines MAHE-BP Malignancies: CA prostate, NHL, CA stomach, sarcoma and carcinoid tumor Autoimmune disorders: SLE, PAN and ankylosing spondylitis RadiationClinical Features More common in males of 40-60 years. Early symptoms are vague and non-specific abdominal or flank pain, weight loss, malaise, and hypertension Obstructive uropathy dysuria, frequency, fever due to secondary infection of hydroneprotic kidney is the earliest and MC specific symptom. Ureters are MC involved, MC site is lower third of ureter. Partial or complete obstruction occurs in 75 patientsDiagnosis: In absence of uremia, diagnosis is made by IVP. IVP or RGP: Hydronephrosis with dilated tortuous upper ureter, Medial pulling of ureters or pipestem ureters Extrinsic ureteral compression CT scan is IOC for retroperitoneal fibrosis. MRI is IOC in cases of compromised renal function, because contrast cannot be given.Treatment Primary, idiopathic retroperitoneal fibrosis: Ureteral stenting and immunosuppression TAPS: Tamoxifen, Azathioprine, Penicillamine, Steroids Secondary retroperitoneal fibrosis: Midline transperitoneal ureterolysis with wrapping the ureter with omental flap or lateral retroperitoneal ureteral transposition.TAPS: Tamoxifen, Azathioprine, Penicillamine, Steroids are used in primary retroperitoneal fibrosis. 