Urology

• Renal involvement in systemic disease

  Glomerular injury may be the prominent feature in the effect of multisystemic disease
• Causes of renal injury in systemic disease

  • Immune
  • Metabolic
  • Neoplastic
  • Paraproteinemia
• Immune-mediated renal involvement (SLE/Lupus nephritis)

  Renal involvement is common clinically and almost universal histopathologically
• Clinical presentation of lupus nephritis

  • Proteinuria
  • Microalbuminuria
  • Nephrotic syndrome
  • Hematuria (mainly microscopic)
  • Hypertension (20%)
  • Varying degree of renal failure
• Immunological reaction in SLE

  Affects the vascular system and causes systemic disease, including lupus nephritis and lupus pneumonitis
• Histopathological classification of lupus nephritis

  • Class I - Minimal mesangial
  • Class II - Mesangial proliferative
  • Class III - Focal nephritis
  • Class IV - Diffuse nephritis
  • Class V - Membranous nephritis
  • Class VI - Sclerotic nephritis

  
• Lupus nephritis

  • Resembles minimal change nephropathy
  • Basement membrane or dense deposits
  • Kidney shrinkage due to fibrosis and sclerosis

  
• Importance of lupus nephritis classification

  • Defines clinical and pathological correlation
  • Provides valuable prognostic information
  • Basis for modern treatment recommendations

  
• Diagnosis of lupus nephritis

  1. Clinical evaluation

  2. Histopathological examination

  3. Serological evaluation (Anti-dsDNA, ANA, C3, C4)
• Symptoms correlate with histopathological findings, e.g., sclerotic type requires aggressive renal replacement therapy
• Immunosuppressive therapy is useful in lupus nephritis as it is an immune-mediated disease
• Anti-dsDNA is specific but not sensitive, while ANA is sensitive but not specific; both are important in choosing appropriate treatment and prognosis
• In glomerular diseases, we find reduced C3 or C4, except in SLE where both are reduced
• Lupus nephritis treatment

  • Oral steroids
  • Parenteral steroids (pulse therapy)
  • Azathioprine
  • Cyclophosphamide
  • Cyclosporin
  • MMF (Cellcept)
  • Rituximab
• Lupus nephritis prognosis

  • Good prognosis: Minimal, focal, mesangial
  • Bad prognosis: Proliferative, membranous, sclerosing
• Parenteral steroids (methylprednisolone) are used in cases of gastric ulcer or malabsorption disease like celiac
• MMF is the new group of azathioprine, and if it causes gastric ulcer, EC-MPA (Myfortic) can be used
• Rituximab is used in active, aggressive diseases and is a powerful medication
• Anti-GBM antibody diseases

  Immune insult with anti-GBM antibody directed against lungs and kidneys
• Predisposing factors for anti-GBM antibody diseases

  • Smoking
  • Inhaled toxins
  • Solvent
  • Upper respiratory tract infections
• Histological findings in anti-GBM antibody diseases

  • Focal or segmental necrosis
  • Formation of crescents
  • Interstitial and tubular nephritis

  
• ANCA is positive in 15% of anti-GBM antibody diseases
• Poor prognosis in anti-GBM antibody diseases

  • More than 50% of crescents
  • Advanced fibrosis
  • Serum creatinine >5
  • Oliguria
  • Need for acute dialysis
• Treatment of anti-GBM antibody diseases

  1. High-dose steroids

  2. Plasmapheresis

  3. Kidney transplant (after at least six months)
• Rituximab is possible in anti-GBM antibody diseases, but it is recommended to wait for six months until serum antibodies become undetected
• Systemic vasculitis

  Affects arterioles, capillaries, small arteries, and even larger arteries; associated with glomerulonephritis
• A 32-year-old male patient presented with symptoms of nasal blockage, blood-mixed nasal discharge, redness of eyes, and was provisionally diagnosed with allergic rhinosinusitis with polyposis
• Further evaluation revealed mild anemia, leukocytosis with neutrophilia, markedly elevated ESR, cough, occasional dyspnea, chest discomfort, microscopic hematuria, and proteinuria

  
• Management of granulomatosis with polyangiitis

  1. Immunosuppressive medications (e.g., corticosteroids, cyclophosphamide) to control inflammation

  2. Supportive care for symptoms (anemia management, renal involvement treatment, respiratory symptom management)

  
• Wegener's granulomatosis

  A multisystem disorder of unknown cause characterized by necrotizing granulomatous inflammation and vasculitis of small and medium vessels

  
• Clinical features of Wegener's granulomatosis

  • Fever
  • Purulent rhinorrhea
  • Cough
  • Hemoptysis
  • Microscopic hematuria
  • Proteinuria
  • Polyarthralgia
  • Skin manifestations
  • Mononeuritis multiplex

  
• Wegener's granulomatosis can present without renal involvement (limited Wegener's)
• Wegener's granulomatosis is common in patients with alpha-1 antitrypsin deficiency

  
• Diagnosis of Wegener's granulomatosis

  1. Chest X-ray (infiltration and cavities)

  2. Renal biopsy (non-caseating granuloma without immune deposit)

  3. ANCA positive

  
• Treatment of Wegener's granulomatosis

  • Steroids
  • Plasmapheresis
  • Cyclophosphamide
• Wegener's granulomatosis should be differentiated from conditions like tuberculosis, rheumatoid arthritis, and various forms of glomerular lesions

  
• Membranoproliferative glomerulonephritis and mixed type of MPGN and MCGN are common secondary causes of membranous nephropathy

  
• Essential cryoglobulinemia


  Circulating cold-precipitable immunoglobulins (IgG, IgM) manifested by fever, malaise, necrotizing skin lesions, purpura, and low serum complement levels

  
• Causes of essential cryoglobulinemia

  • Hepatitis B and C virus infection
  • Fungal infection
  • Other viral infections
  • Chronic infection
• Treatment of essential cryoglobulinemia

  1. Eradicate infection

  2. Plasma exchange

  3. Steroids

  4. Cytotoxic agents (e.g., cyclophosphamide)