RESPIRATORY
WEGENER'S GRANULOMATOSIS (GRANULOMATOSIS WITH POLYANGIITIS/GPA)
PromptClinical features of Wegeners/GPA
ResponseRespiratory
Myalgia/arthralgia/arthiritis
Conjunctivitis/episcleritis/uveitis
Cranial and peripheral neuritis
Myo/pericarditis
- Pulmonary haemorrhage
- Sub-glottic stenosis
- Collapsing nasal bridge
- Upper airway granuloma
- Pulmonary fibrosis
- Pulmonary hypertension
Myalgia/arthralgia/arthiritis
Conjunctivitis/episcleritis/uveitis
Cranial and peripheral neuritis
Myo/pericarditis
PromptConfirmation investigations in Wegeners/GPA
ResponseANCA - 90% sensitivity for GPA
PromptManagement of Wegeners/GPA
ResponseImmunotherapy
Steroids
Plasma exchange
Immunosuppressants
Intubation for respiratory support
Steroids
Plasma exchange
Immunosuppressants
Intubation for respiratory support
PromptGOODPASTURE’S SYNDROME
ResponseGoodpasture syndrome (GPS), also known as anti–glomerular basement membrane disease, is a rare autoimmune disease in which antibodies attack the basement membrane in lungs and kidneys, leading to bleeding from the lungs, glomerulonephritis, and kidney failure
- Wiki
Prompt30 F with hx of goodpasture's - immunosuppressed
ResponseBilateral
Patchy alveolar opacities
Both ling fields widespread distribution
DDx
Pulmonary haemorrhage (goodpastures)
LRTI - staph, influenza
Opportunistic infection - PJP
Cryptogenic organising pneumonia/bronchiolitis obliterans
CCF
Malignancy with lymphatic dissemination/Lymphangitis carcinomatosis
Treatment Goodpastures
Plasma exchange
Immunosuppression
Patchy alveolar opacities
Both ling fields widespread distribution
DDx
Pulmonary haemorrhage (goodpastures)
LRTI - staph, influenza
Opportunistic infection - PJP
Cryptogenic organising pneumonia/bronchiolitis obliterans
CCF
Malignancy with lymphatic dissemination/Lymphangitis carcinomatosis
Treatment Goodpastures
Plasma exchange
Immunosuppression
Granulomatosis with polyangiitis (GPA), formerly known as Wegener granulomatosis, is a rare multisystem autoimmune disease of unknown etiology. GPA is one of the antineutrophil cytoplasmic antibody (ANCA)–associated vasculitic disorders, which can affect the ears, nose, throat, respiratory tract, skin, kidneys, and peripheral nervous system. [1] Its hallmark features include necrotizing granulomatous inflammation and pauci-immune vasculitis in small- and medium-sized blood vessels.
- Medscape