Table 1.
Major Diseases Linked to Autoimmunity to Basement Membrane Components.
| Human Diseases | Major Organ System | Key Clinical Features | BM Target |
|---|---|---|---|
| Anti-GBM GN & GP disease | Kidney & Lung & lung hemorrhage | GN with renal failure α5(IV)NC1 collagen | α3(IV)NC1 collagen |
| Bronchiolitis obliterans (post lung transplant) | Lung | Fibro-obliterative | Collagen V pulmonary failure |
| Rheumatoid arthritis | Synovial Joints | Destructive arthritis | Collagen II (native & citrullinated) |
| Autoimmune Dermatoses | |||
| Bullous pemphigoid | Skin | Epidermal-dermal blisters | Collagen XVII/BP180 |
| Epidermolysis bullosa acquisita | Skin | Epidermal-dermal blisters | Collagen VII |
| Anti-laminin γ1 pemphigoid | Skin | Epidermal-dermal blisters | Laminin γ1 chain |
| Anti-laminin-332 mucous membrane pemphigoid | Mucous membranes | Epidermal-dermal blisters | Laminin-332 (laminin-5) |
Abbreviations: BM, basement membrane; GBM, glomerular basement membrane; GN, glomerulonephritis; GP, Goodpasture’s; NCq, noncollagenous domain1.