Rapidly Progressive Glomerulonephritis Clinical Presentation: History, Physical Examination (original) (raw)

Presentation

History

The most common prodrome of antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis is flulike illness characterized by malaise, fever, arthralgias, myalgias, anorexia, and weight loss. This occurs in more than 90% of patients and can occur within days to months of the onset of nephritis or other manifestations of vasculitis.

Following the prodrome, the most common complaints are abdominal pain, painful cutaneous nodules or ulcerations, and a migratory polyarthropathy. When pulmonary or upper airway involvement is present, patients complain of sinusitis symptoms, cough, and hemoptysis.

Physical Examination

Hypertension can be present but is not common. Unless specific findings are present, such as those listed below, the physical examination results are usually normal. Organs or systems affected by ANCA-associated disease are listed below.

Skin findings are as follows:

• Leukocytoclastic vasculitis is common (40-60%) and usually affects the lower extremities
• Necrotizing arteritis can result in painful erythematous nodules, focal necrosis, ulceration, and livedo reticularis
• Patients with granulomatosis with polyangiitis or eosinophilic granulomatosis with polyangiitis (EGPA) can also have granulomatous cutaneous nodules
• Nailfold infarcts can be present

Nervous system findings are as follows:

• Mononeuritis multiplex is the most common nervous system manifestation of ANCA-associated disease; this condition is caused by inflammation of the epineural arteries and arterioles, which results in ischemia of the nerve tissue
• Central nervous system disease can result from involvement of meningeal vessels and manifest as generalized seizures
• Nervous system involvement is present in 30% of patients with microscopic polyangiitis and 70% of patients with EGPA

Musculoskeletal findings are as follows:

• Pain and elevation in tissue enzyme levels can result from inflammation in the arteries of skeletal muscle
• Musculoskeletal involvement is present in 60% of patients with ANCA-associated disease
• Arthritis is a very common symptom; it is usually symmetrical and migratory and usually involves the small joints
• Arthralgias are also common, but this is not considered a marker of active vasculitis

Gastrointestinal (GI) findings are as follows:

• Arteritis can result in ischemic ulceration in the GI tract, causing pain and bleeding, which is usually occult
• The most serious complications of GI ischemia are intussusception and pancreatitis
• GI involvement occurs in 50% of patients with ANCA

Kidney findings are as follows:

• The diagnostic biopsy finding is proliferative necrotizing crescentic glomerulonephritis
• If overt kidney disease is not present upon presentation, then the most common finding is microscopic hematuria
• The prevalence rate of kidney disease is 90% for those with microscopic polyangiitis, 80% for those with granulomatosis with polyangiitis, and 45% for those with EGPA

Respiratory findings are as follows:

• Pulmonary manifestations range from fleeting focal infiltrates to hemorrhagic alveolar capillaritis resulting in massive pulmonary hemorrhage and hemoptysis; this is the most deadly complication of ANCA disease
• The prevalence rate of pulmonary findings is 50% in those with microscopic polyangiitis, 90% in those with granulomatosis with polyangiitis, and 80% in those with EGPA
• Upper respiratory manifestations include sinusitis, otitis media, ulcers in the nasal mucosa, and subglottic stenosis

Ocular findings are as follows:

• Iritis, uveitis, and conjunctivitis are the most common ocular manifestations of ANCA-associated disease
• Involvement occurs in approximately 2% of patients with ANCA-associated disease

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Author

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

George R Aronoff, MD Director, Professor, Departments of Internal Medicine and Pharmacology, Section of Nephrology, Kidney Disease Program, University of Louisville School of Medicine

George R Aronoff, MD is a member of the following medical societies: American Federation for Medical Research, American Society of Nephrology, Kentucky Medical Association, National Kidney Foundation

Disclosure: Nothing to disclose.

Chief Editor

Vecihi Batuman, MD, FASN Professor of Medicine, Section of Nephrology-Hypertension, Deming Department of Medicine, Tulane University School of Medicine

Vecihi Batuman, MD, FASN is a member of the following medical societies: American College of Physicians, American Society of Hypertension, American Society of Nephrology, Southern Society for Clinical Investigation

Disclosure: Nothing to disclose.

Additional Contributors

F John Gennari, MD Associate Chair for Academic Affairs, Robert F and Genevieve B Patrick Professor, Department of Medicine, University of Vermont College of Medicine

F John Gennari, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, American Federation for Medical Research, American Heart Association, American Physiological Society, American Society for Clinical Investigation, American Society of Nephrology, International Society of Nephrology

Disclosure: Nothing to disclose.

Acknowledgements

Kerry C Owens, MD Consulting Staff, Department of Internal Medicine, Section of Nephrology, Integris Baptist Medical Center of Oklahoma City

Kerry C Owens, MD is a member of the following medical societies: American College of Physicians, American Medical Association, American Society of Nephrology, International Society of Nephrology, Oklahoma State Medical Association, and Sigma Xi

Disclosure: Nothing to disclose.