Clinical manifestations of chronic graft-versus-host disease (GVHD).
| 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. Topical steroid ointment may be used if it gives symptomatic relief to localized areas. |
| Nails | Vertical ridging, fragile. damage. | Clinical. | Nail polish may help to decrease further |
| Sweat glands | Destruction leading to risk of hyperthermia. | Avoid excessive heat. | |
| Hair | Scalp and body hair is thin and fragile, can be partially or completely lost. | Clinical. | |
| Eyes | Dryness, photophobia, and burning. Progression to corneal abrasion. | Regular ophthalmologic evaluation including Schirmer’s 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 Avoid foods which are not tolerated. Regular in the cheeks and tongue identical to lichen planus. Erythema and dental care preceded by appropriate endocardi-painful ulcerations, mucosal scleroderma with decreased sensitivity to temperature can also happen. | Regular dental evaluation (with appropriate endocarditis prophylaxis). Viral and fungal cultures at diagnosis and at any worsening. tis prophylaxis. Topical steroid rinses followed by an antifungal agent for symptomatic relief. | |
| Respiratory tract | Bronchiolitis Obliterans can manifest as dyspnea, wheezing, cough with normal CT scan and marked obstruction at pulmonary function tests. Chronic sinopulmonary symptoms and/or infections are also common.With abnormal chest CT, must rule out infections. Lung biopsy if clinically indicated. | Pulmonary function tests including FEV1, FVC, DLCO, helium lung volumes. CT scan in symptomatic patients. | Investigational therapy. |
| Gastrointestinal | Abnormal motility and strictures. Weight loss. | Swallowing studies, endoscopy if clinically indicated. Nutritional evaluation. intervention. | Systemic treatment of GVHD; endoscopical/ surgical treatment of strictures. Nutritional |
| 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 the range of motion. Bone density evaluation especially in patients using steroids. | Aggressive physical therapy program. |
| Immune system Variable IgG levels. | Profound immunodeficiency. Functional asplenia. High risk of pneumococcal sepsis, PCP, and invasive fungal infections. GVHD has resolved. | Assume all patients as severely immuno-compromised and asplenic to 6 months after vaccinations. | PCP prophylaxis (until 6 months after no GVHD) and Pneumococcal prophylaxis (lifetime). Delay |
| Hematopoietic system | Cytopenias. Occasional eosinophilia. | Counts. Bone marrow aspirate and biopsy, anti-neutrophil and anti-platelet antibodies when indicated. | Systemic treatment of GVHD. |
| Others | Virtually all autoimmune disease manifestations 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. Topical steroid ointment may be used if it gives symptomatic relief to localized areas. |
| Nails | Vertical ridging, fragile. damage. | Clinical. | Nail polish may help to decrease further |
| Sweat glands | Destruction leading to risk of hyperthermia. | Avoid excessive heat. | |
| Hair | Scalp and body hair is thin and fragile, can be partially or completely lost. | Clinical. | |
| Eyes | Dryness, photophobia, and burning. Progression to corneal abrasion. | Regular ophthalmologic evaluation including Schirmer’s 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 Avoid foods which are not tolerated. Regular in the cheeks and tongue identical to lichen planus. Erythema and dental care preceded by appropriate endocardi-painful ulcerations, mucosal scleroderma with decreased sensitivity to temperature can also happen. | Regular dental evaluation (with appropriate endocarditis prophylaxis). Viral and fungal cultures at diagnosis and at any worsening. tis prophylaxis. Topical steroid rinses followed by an antifungal agent for symptomatic relief. | |
| Respiratory tract | Bronchiolitis Obliterans can manifest as dyspnea, wheezing, cough with normal CT scan and marked obstruction at pulmonary function tests. Chronic sinopulmonary symptoms and/or infections are also common.With abnormal chest CT, must rule out infections. Lung biopsy if clinically indicated. | Pulmonary function tests including FEV1, FVC, DLCO, helium lung volumes. CT scan in symptomatic patients. | Investigational therapy. |
| Gastrointestinal | Abnormal motility and strictures. Weight loss. | Swallowing studies, endoscopy if clinically indicated. Nutritional evaluation. intervention. | Systemic treatment of GVHD; endoscopical/ surgical treatment of strictures. Nutritional |
| 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 the range of motion. Bone density evaluation especially in patients using steroids. | Aggressive physical therapy program. |
| Immune system Variable IgG levels. | Profound immunodeficiency. Functional asplenia. High risk of pneumococcal sepsis, PCP, and invasive fungal infections. GVHD has resolved. | Assume all patients as severely immuno-compromised and asplenic to 6 months after vaccinations. | PCP prophylaxis (until 6 months after no GVHD) and Pneumococcal prophylaxis (lifetime). Delay |
| Hematopoietic system | Cytopenias. Occasional eosinophilia. | Counts. Bone marrow aspirate and biopsy, anti-neutrophil and anti-platelet antibodies when indicated. | Systemic treatment of GVHD. |
| Others | Virtually all autoimmune disease manifestations have been described in association with chronic GVHD. | As clinically indicated. |