Clinical manifestations of chronic graft-versus-host disease
| Organ . | Clinical manifestation . | Evaluation . | Intervention . |
|---|---|---|---|
| Skin | Erythematous papular rash (lichenoid) or thickened, tight, fragile skin (sclerodermatous) | Clinical and biopsy to confirm the diagnosis of GVHD | Moisturize (petroleum jelly), treat local infections, protect from further trauma |
| Nails | Vertical ridging, fragile | Clinical | Nail polish may help to decrease further damage |
| Sweat glands | Destruction leading to risk for hyperthermia | Avoid excessive heat | |
| Hair | Scalp and body hair thin and fragile; can be partially or completely lost | Clinical | |
| Eyes | Dryness, photophobia, and burning Progression to corneal abrasion | Regular ophthalmological evaluation including Schirmer test | Preservative-free tears during the day and preservative-free ointment at night |
| Mouth | Dry, sensitivity to mint, spicy food, tomato Whitish lace-like plaques in the cheeks and tongue identical to lichen planus Erythema and painful ulcerations, mucosal scleroderma with decreased sensitivity to temperature possible | Regular dental evaluation (with appropriate endocarditis prophylaxis) Viral and fungal cultures at diagnosis and at any worsening | Avoid foods that are not tolerated Regular dental care, preceded by appropriate endocarditis prophylaxis |
| Respiratory tract | Bronchiolitis obliterans can manifest as dyspnea, wheezing, cough with normal CT imaging findings and marked obstruction at pulmonary function tests Chronic sinopulmonary symptoms, infections, or both also common | Pulmonary function tests including FEV1, FVC, DLCO, helium lung volumes Computed tomography imaging in symptomatic patients (rule out infections if findings are abnormal) Lung biopsy if clinically indicated | Investigational therapy |
| Gastrointestinal | Abnormal motility and strictures Weight loss | Swallowing studies, endoscopy if clinically indicated Nutritional evaluation | Systemic treatment of GVHD Endoscopic/surgical treatment of strictures Nutritional intervention |
| Liver | Cholestasis (increased bilirubin, alkaline phosphatase) Isolated liver involvement needs histologic confirmation | Liver function tests Liver biopsy if clinically indicated | No specific therapy is proven superior FK506 may concentrate in the liver |
| Musculoskeletal | Fasciitis Myositis is rare Osteoporosis may occur secondary to hormonal deficits, use of steroids, decreased activity | Periodical physical therapy evaluation to document range of motion Bone density evaluation, especially for patients using steroids | Aggressive physical therapy program |
| Immune system | Profound immunodeficiency Functional asplenia High-risk for pneumococcal sepsis, P carinii pneumonia, and invasive fungal infections Variable IgG levels | Assume all patients are severely immunocompromised and asplenic | P carinii pneumonia prophylaxis (until 6 months after no GVHD) and pneumococcus prophylaxis (lifetime) Delay vaccinations to 6 months after GVHD has resolved |
| Hematopoietic system | Cytopenias Occasional eosinophilia | Counts Bone marrow aspirate and biopsy, antineutrophil and antiplatelet antibodies when indicated | Systemic treatment of GVHD |
| Others | Virtually all manifestations of autoimmune disease have been described in association with chronic GVHD | As clinically indicated |
| Organ . | Clinical manifestation . | Evaluation . | Intervention . |
|---|---|---|---|
| Skin | Erythematous papular rash (lichenoid) or thickened, tight, fragile skin (sclerodermatous) | Clinical and biopsy to confirm the diagnosis of GVHD | Moisturize (petroleum jelly), treat local infections, protect from further trauma |
| Nails | Vertical ridging, fragile | Clinical | Nail polish may help to decrease further damage |
| Sweat glands | Destruction leading to risk for hyperthermia | Avoid excessive heat | |
| Hair | Scalp and body hair thin and fragile; can be partially or completely lost | Clinical | |
| Eyes | Dryness, photophobia, and burning Progression to corneal abrasion | Regular ophthalmological evaluation including Schirmer test | Preservative-free tears during the day and preservative-free ointment at night |
| Mouth | Dry, sensitivity to mint, spicy food, tomato Whitish lace-like plaques in the cheeks and tongue identical to lichen planus Erythema and painful ulcerations, mucosal scleroderma with decreased sensitivity to temperature possible | Regular dental evaluation (with appropriate endocarditis prophylaxis) Viral and fungal cultures at diagnosis and at any worsening | Avoid foods that are not tolerated Regular dental care, preceded by appropriate endocarditis prophylaxis |
| Respiratory tract | Bronchiolitis obliterans can manifest as dyspnea, wheezing, cough with normal CT imaging findings and marked obstruction at pulmonary function tests Chronic sinopulmonary symptoms, infections, or both also common | Pulmonary function tests including FEV1, FVC, DLCO, helium lung volumes Computed tomography imaging in symptomatic patients (rule out infections if findings are abnormal) Lung biopsy if clinically indicated | Investigational therapy |
| Gastrointestinal | Abnormal motility and strictures Weight loss | Swallowing studies, endoscopy if clinically indicated Nutritional evaluation | Systemic treatment of GVHD Endoscopic/surgical treatment of strictures Nutritional intervention |
| Liver | Cholestasis (increased bilirubin, alkaline phosphatase) Isolated liver involvement needs histologic confirmation | Liver function tests Liver biopsy if clinically indicated | No specific therapy is proven superior FK506 may concentrate in the liver |
| Musculoskeletal | Fasciitis Myositis is rare Osteoporosis may occur secondary to hormonal deficits, use of steroids, decreased activity | Periodical physical therapy evaluation to document range of motion Bone density evaluation, especially for patients using steroids | Aggressive physical therapy program |
| Immune system | Profound immunodeficiency Functional asplenia High-risk for pneumococcal sepsis, P carinii pneumonia, and invasive fungal infections Variable IgG levels | Assume all patients are severely immunocompromised and asplenic | P carinii pneumonia prophylaxis (until 6 months after no GVHD) and pneumococcus prophylaxis (lifetime) Delay vaccinations to 6 months after GVHD has resolved |
| Hematopoietic system | Cytopenias Occasional eosinophilia | Counts Bone marrow aspirate and biopsy, antineutrophil and antiplatelet antibodies when indicated | Systemic treatment of GVHD |
| Others | Virtually all manifestations of autoimmune disease have been described in association with chronic GVHD | As clinically indicated |
GVHD indicates graft-versus-host disease.